Article

Angiofollicular lymph node hyperplasia: A comparison of unicentric, multicentric, hyaline vascular, and plasma cell types of disease by morphometric and clinical analysis

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Abstract

A morphometric and clinical analysis of 47 cases of angiofollicular lymph node hyperplasia (AFLH) demonstrated morphometric differences between plasma cell (28 cases) and hyaline vascular (19 cases) AFLH. Twenty-one AFLH cases were unicentric, and 26 cases were multicentric. Plasma cell types of AFLH, defined by standard morphologic and objective morphometric assessment, were associated with systemic clinical and laboratory abnormalities. Unicentric or hyaline vascular AFLH was amenable to surgical therapy, and multicentric or plasma cell AFLH without neuropathy was amenable to steroid treatment or chemotherapy with good prognoses. Multicentric or plasma cell AFLH with neuropathy was associated with resistance to steroids and chemotherapy and with a poor prognosis.

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... The mantle zones of these follicles are broad and in many cases, the mantle zones are composed of concentric rings of small lymphocytes (the socalled onion skin pattern). 4,5,14 At low power, these changes in the follicles impart a target-like appearance (see Fig. 3). The expanded follicles can compress or obliterate contiguous structures. ...
... Clusters of plasmacytoid dendritic cells are often (but not invariably) present in the interfollicular regions, usually as clusters with a starry sky pattern. 4,5,14 Scattered PCs, immunoblasts, and eosinophils can be seen in the interfollicular regions; sheets of PCs (as seen in PC-CD) are absent. Lymph node sinuses are characteristically absent in fully developed HV-CD and obliteration of the subcapsular sinuses is often an early morphologic change in HV-CD. ...
... In addition, dysplastic FDCs are uncommon. 4,10,19 The interfollicular areas and medulla are expanded by variable numbers of PCs that show a range of maturation, from mature to atypical, the latter associated with HHV8 infection. Vascular proliferation is also present in the interfollicular areas. ...
Article
Castleman disease (CD) is divided clinically into unicentric or multicentric type. Pathologically, CD is divided into hyaline-vascular and plasma cell variants. Unicentric CD is most common, about 75% of these cases are hyaline-vascular variant, and surgical excision is often curative. In contrast, there are a number of types of multicentric CD including HHV8-associated, idiopathic, and a subset of cases that arise in association with POEMS syndrome. Therapy is required for most patients with multicentric CD, but there is no consensus approach currently. As is evidence, the designation Castleman disease encompasses a heterogeneous group of diseases of varied pathogenesis and which require different therapies.
... Castleman's disease (CD), otherwise known as angiofollicular lymph node hyperplasia, giant lymph node hyperplasia as well as angiomatous lymphoid hamartoma, et al., is a rare chronic disease characterized by lymphadenopathy with unique histopathological features and incompletely unknown etiology [1][2][3]. It was first reported by Benjamin Castleman who delineated the clinical and pathological features of 13 patients with solitary mediastinal lymphadenopathy in 1954 [4]. ...
... It was first reported by Benjamin Castleman who delineated the clinical and pathological features of 13 patients with solitary mediastinal lymphadenopathy in 1954 [4]. Since then, the clinical and pathological reports about CD cases throughout the whole body have been increasingly reported in the literature [1][2][3]5]. ...
... Histologically, both UCD and MCD are characterized by lymph node enlargement with overall intact structure. The hyaline-vascular variant, the most common type of CD, is featured with abnormal follicles with regressed germinal centers and broad zone of IgM and IgD expressing lymphocytes organized in concentric rings, so-called Bonion-skin^a rrangement [1,2]. However, in the plasma-cell type of CD, there are more numerous and larger hyperplastic follicles with more expanded mantle zones as compared with the hyalinevascular variant. ...
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Objectives: The aim of the present study was to comprehensively summarize the epidemiological, clinicopathological characteristics, treatments as well as prognosis of Castleman's disease (CD) identified in the oral maxillofacial and neck region. Materials and methods: Patients with CD in the oral maxillofacial and neck were retrieved from disease registry at our institution from Jan. 1990 to Dec. 2015. Systematic reviews from both English and Chinese literature were performed to collect the detailed information about the oral maxillofacial and neck CD. The epidemiological, clinicopathological data and treatment outcomes were further statistically analyzed. Results: Four patients with the oral maxillofacial and neck CD were identified and histologically confirmed as hyaline-vascular type. They underwent surgical excision without recurrence during the follow-up. Systematic literature reviews identified 221 cases from 123 eligible articles which satisfied the inclusion criteria. In 225 patients, most patients were diagnosed as unicentric (207) or hyaline-vascular type (205) of CD and identified in the neck, and treated by surgical resection with good prognosis. In contrast, the minority of patients was multicentric or plasma-cell/mixed type and treated by chemotherapy with inferior outcomes. Kaplan-Meir analyses revealed that both clinical and pathological types were significantly associated with patients' overall survival. Conclusions: Although rare, most cases of the oral maxillofacial neck CD are found in adults and classified as unicentric and hyaline-vascular type of CD. Complete surgical excision is preferred with favorable prognosis for unicentric disease, whereas chemotherapy is usually exploited for multicentric disease with inferior outcomes. Clinical relevance: These data provide comprehensive information about the epidemiology, clinicopathological features, treatments, and outcomes of the oral maxillofacial and neck CD.
... Из лабораторных нарушений наиболее типичными являются анемия, часто в виде аутоиммунной гемолитичес кой, увеличение СОЭ, гипоальбуминемия, повышенный уровень СРБ и фибриногена и такие иммунологические отклонения, как поликлональная гипергаммаглобулинемия и наличие аутоантител (АНФ, антиДНК, антитела к тиреоглобулину, париетальным клеткам и клеткам надпочеч ников) [12,26,30]. БК также характеризуется рядом гемато логических и коагулопатических нарушений: рефрактерной СИСТЕМНАЯ КРАСНАЯ ВОЛЧАНКА ИЛИ БОЛЕЗНЬ КАСТЛЕМАНА? ...
... Мультицентрический вариант болезни может либо заканчиваться ремиссией, либо про текать с частыми рецидивами, либо носить стабильный, медленнопрогрессирующий характер, либо характеризо ваться быстропрогрессирующим течением с трансформа цией в злокачественную лимфому [27]. Четкой зависимос ти между наличием определенных клинико-лабораторных проявлений и исходом или течением мультицентрическо го варианта БК не отмечено, за исключением перифери ческой невропатии, которая ассоциируется с нечувствитель ностью к проводимой терапии и плохим прогнозом [26]. В отличие от локализованной формы БК мульти центрическая требует системной терапии кортикостерои дами и цитотоксическими препаратами [12,26,27]. ...
... Четкой зависимос ти между наличием определенных клинико-лабораторных проявлений и исходом или течением мультицентрическо го варианта БК не отмечено, за исключением перифери ческой невропатии, которая ассоциируется с нечувствитель ностью к проводимой терапии и плохим прогнозом [26]. В отличие от локализованной формы БК мульти центрическая требует системной терапии кортикостерои дами и цитотоксическими препаратами [12,26,27]. Леталь ность при данном варианте болезни достигает почти 50%, а средняя продолжительность жизни 26 мес [27]. ...
... Surgery is considered to be the most adequate therapy for unicentric resectable cases of CD, as it seems to be curative in almost all of the cases [4,6,[12][13][14][15][16][17][18][19][20]. Radiotherapy has also been described as a definitive treatment, however with a variable response rate [4,9,10,[21][22][23][24][25][26][27][28][29][30]. ...
... There is no consensus yet concerning the most adequate treatment for CD. Surgery is considered to be the most adequate therapy for unicentric cases of CD as it seems to be curative in most of the cases [4,6,[12][13][14][15][16][17][18][19][20]. Various strategies have been described in case of irresectable unicentric CD ranging from primary radiotherapy, incomplete resection [10] and chemotherapy [31]. ...
... The evidence in the current literature reveals that radical surgery results in excellent rates of cure [4,6,[12][13][14][15][16][17][18][19]. Also the literature reports excellent response rates with primary radiotherapy (table 1). ...
Article
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Castleman disease (CD) is a rare benign disorder characterised by hyperplasia of lymphoid tissue that may develop at a single site or throughout the body. The etiology of this disorder is unclear, although the histopathological presentation can be differentiated into a hyaline vascular variant, a plasma cell variant and a mixed variant. Clinically, it has been recorded that 3 manifestations of CD are characterized: a localized unicentric type, a generalized multicentric type and a mixed form. Surgery remains the main treatment for resectable unicentric CD, since removal of the large node is possible without further complications. No consensus has been reached concerning the most adequate treatment for irresectable unicentric CD. Case report of a 67 year old woman. This report, describes the case of a 67-year-old woman with unicentric Castleman disease located in the right lower abdomen. The patient had symptoms of fatigue, dyspnoea and pain in the right lower abdomen. Computed tomography (CT)- examination revealed a tumour, which had grown to form a close relationship with the common iliac vessels and the sacral bone. A Laparotomy procedure revealed that the tumour was an irresectable mass. Neo-adjuvant radiotherapy (40 Gy) was administered in order to downsize the tumour. Six weeks later a new CT-scan revealed a major reduction of the tumour, which enabled a successful radical resection of the tumour to be performed. Histopathological analysis of the tumour showed the hyaline vascular type of CD. Neo-adjuvant radiotherapy should be considered in case of an irresectable unicentric CD.
... 11,12 The optimal treatment of Castleman disease is unknown; however, surgery has long been considered standard therapy for the unicentric type, with a variety of case reports and retrospective series reporting excellent rates of cure. 7,10,[13][14][15][16][17][18][19] Keller et al. 2 reported in 1972 that all reported cases of unicentric Castleman disease had an indolent clinical course with elimination of all systemic symptoms after surgical excision. However, they also reported that radiotherapy in doses ranging from 1800 to 4300 centigrays (cGy) resulted in little shrinkage of lymphadenopathy. 2 As a result, the prevailing medical opinion became that radiotherapy was ineffective in the treatment of Castleman disease. ...
... 28,29 Surgery has long been considered the standard therapy for the management of unicentric Castleman disease, and cure rates approach 100%. 7,10,[13][14][15][16][17][18][19]28,29 Our data support the effectiveness of surgery; all of the patients in our series treated with complete or partial resection are alive and asymptomatic. Of interest are the two patients in our series treated only with partial resection. ...
Article
BACKGROUND Although surgery is considered standard therapy for unicentric Castleman disease, favorable responses to radiotherapy also have been documented. The authors undertook this study to analyze the clinical factors, treatment approaches, and outcomes of patients with unicentric or multicentric Castleman disease, and to report the outcomes of patients with unicentric Castleman disease treated with radiotherapy.METHODS The authors reviewed the medical records of 22 patients who had received a histologic diagnosis of Castleman disease at the University of Texas M. D. Anderson Cancer Center between 1988 and 1999. One patient with a concurrent histopathologic diagnosis of nonsecretory multiple myeloma was excluded from the study. In all patients, the diagnosis of Castleman disease was based on the results of lymph node biopsies. Disease was categorized as being either unicentric or multicentric and further subdivided into hyaline vascular, plasma cell, or mixed variant histologic types. Clinical variables and outcomes were analyzed according to treatment, which consisted of surgery, chemotherapy, or radiotherapy.RESULTSRecords from 21 patients were analyzed: 12 had unicentric disease, and 9 had multicentric disease. The mean follow-up time for the entire series was 51 months (median, 40 months). Four patients with unicentric disease were treated with radiotherapy alone: 2 remain alive and symptom free, 2 died of causes unrelated to Castleman disease and had no evidence of disease at last follow-up. Eight patients with unicentric disease were treated with complete or partial surgical resection, and all are alive and asymptomatic. All nine patients with multicentric disease were treated with combination chemotherapy: five are alive with no evidence of disease, and four are alive with progressive disease.CONCLUSIONS Surgery results in excellent rates of cure in patients with unicentric Castleman disease; radiotherapy can also achieve clinical response and cure in selected patients. Multicentric Castleman disease is a more aggressive clinical entity and is most effectively treated with combination chemotherapy, whereas the role of radiotherapy in its treatment remains unclear. Cancer 2001;92:670–6. © 2001 American Cancer Society.
... Within the context of POEMS syndrome, the absence of osteosclerotic lesions has been implicated as an adverse feature [24], but the issue in CD had not been addressed. We and others have speculated previously that among patients with CD peripheral neuropathy is associated with a worse outcome [35,36], and the current data strongly support that hypothesis. Most of the factors identified as risk factors on univariate analysis, including multicentric disease, plasma cell variant, peripheral neuropathy, organomegaly, low albumin, and an abnormal platelet count, cosegregated with POEMS syndrome, which supports the concept of four categories of CD: (1) unicentric CD; (2) multicentric CD associated with the osteosclerotic variant of POEMS syndrome; (3) multicentric CD without POEMS syndrome; and (4) multicentric CD with POEMS syndrome without osteosclerotic lesions. ...
... The observations that multicentric patients are older, more likely to have the plasma cell variant, and more likely to be symptomatic are not novel [5,7,8,[35][36][37][38][39][40]43,44]. For the 19 patients for whom there was discordance between disease extent and histology, the course of disease was dictated by extent rather than by histology, despite the fact that this was a population of patients who had limited imaging. ...
Article
Castleman's disease (CD) is a rare, poorly understood lymphoproliferative disease. The spectrum of symptoms and course of disease are broad, but there is no large study describing the natural history of this disease. Basic clinic and laboratory data from the records of 113 patients with CD evaluated at the Mayo Clinic and University of Nebraska were abstracted. The impact of these variables on overall survival (OS) from time of diagnosis was evaluated. Sixty patients had multicentric disease. Of the patients with multicentric CD, 32% had criteria sufficient for a diagnosis of POEMS syndrome. For all patients, 2, 5, and 10-year OS was 92%, 76%, 59%, respectively. Most of the factors identified as risk factors for death on univariate analysis cosegregated with diagnostic criteria for POEMS syndrome, which supported the concept of four categories of CD, which are (along with their 5-year OS): (1) unicentric CD (91%); (2) multicentric CD associated with the osteosclerotic variant of POEMS syndrome (90%); (3); multicentric CD without POEMS syndrome (65%); and (4) multicentric CD with POEMS syndrome without osteosclerotic lesions (27%). We have demonstrated that CD represents a spectrum of disease that can be differentiated by simple prognostic factors that provide a framework for further study. Am. J. Hematol. 2012. © 2012 Wiley Periodicals, Inc.
... The latter involves lymph nodes separately or in aggregations and often displays multicentricity with systemic symptoms including autoimmune phenomena and aggressive course. Small hyalinized and hypervascular germinal centers with hypervascular interfollicular stroma and sinus effacement are common features of the HVCD, whereas hyperplastic germinal centers with plasma cell aggregates in lymph node paracortex and partially spared sinuses are characteristic features of the PCCD [2]. A key event in the pathogenesis of CD has been recently suggested to be an abnormal production of a B cell growth factor, such as IL-6, leading to lymphoid cell proliferation and plasma cell differentiation [3,4]. ...
... The earliest description of Castleman's disease dates back to 1954 [1]. Histologically, three variants are recognized: hyaline vascular, plasma cell type, and mixed type [2]. Hyaline-vascular type is the commonest type and is usually localized to mediastinum or pulmonary hilum; while only 6% of all cases of CD present as primary cervical lymphadenopathy [7]. ...
Article
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Malignancy in a setting of hyaline vascular type of Castleman's disease (HVCD) is an exceptional occurrence. Herein, we report an extremely rare case of synchronous unicentric cervical HVCD and squamous cell carcinoma (SCC) of tongue mimicking stage IV disease. A 32-year-old gentleman presented with an ulcerated mass on the right tongue border and ipsilateral cervical nodal mass. As the clinical stage was IVB (T(1)N(3)M(0)), an anterior two-third glossectomy with bilateral modified neck dissection was performed. On gross examination, an ulcerated mass on the right lateral border was identified. In addition, an 8 cm large nodal mass at right level III-V was seen. Microscopy from the ulcerated growth in the tongue revealed an invasive well differentiated squamous cell carcinoma. However, the right cervical nodal mass yielded surprise histology of Castleman's disease, hyaline-vascular type. Final tumor pathological staging was revised to pT1N0M0 . This case reveals that HVCD can rarely be associated with an epithelial malignancy wherein it can clinically mimic nodal metastasis. Whether such a phenomenon occurs due to underlying immune aberrations or is a rare co-incidence remains unclear.
... The plasma-cell type accounts for 20% of UCD and 90% of MCD. 2,4 Mixed forms of CD exist with elements of hyalinevascular and plasma-cell types both present. In recent years, human herpes virus-8 (HHV-8)associated MCD, which is also known as plasmablastic MCD, has been distinguished from MCD. ...
... Approximately 50% of plasma-cell CD patients display systemic symptoms of fever, night sweats, malaise, splenomegaly, anemia, cytopenia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia and bone marrow plasmacytosis. 4 Plasma-cell CD is also considered to be a risk for POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome and lymphoma. 18 Histologically, continuous sheets of dense plasma-cell infiltration and less vascular interfollicular stroma surrounding the germinal centers were prominent features of plasma-cell CD. ...
Article
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Castleman's disease (CD) is a rare and complex disease that involves the head and neck region. In our hospital, only three cases of CD in the head and neck region have been encountered in the past 20 years. To establish a database of Castleman's disease in Taiwan and to compare it to published western studies, we collected case reports of CD in the head and neck region in Taiwan from 1992 to 2007. We used "Castleman's disease" and "Taiwan" as keywords to search medical journal databases. A total of 15 cases were incorporated in our study, including three from our own hospital. These data were integrated for studying the disease characteristics in Taiwan. Submandibular area (level I) was the most commonly involved site (5 cases, 33.3%). The ratio of unicentric to multicentric CD and the proportion of histological classifications were similar to those in the western studies included in our review. All of the unicentric CD treated by complete resection showed no evidence of recurrence. Multicentric CD was present in two patients, although one died following a heart attack. The other multicentric CD patient was treated by medication and survived for several years. The features of head and neck CD in Taiwan are similar to those reported in western studies. Although CD is not a common disease, it should be considered as a differential diagnosis in patients with a neck mass, especially when the patient is suffering from long-term inflammation. Multicentric CD is also likely to be initially misdiagnosed as unicentric CD. It is important to remember that all patients diagnosed with CD should receive a systemic survey to exclude the possibility of ignored lesions.
... Multicentric CD may be associated with HIV and POEM's syndrome. 9 Elevated erythrocyte sedimentation rate, anemia, thrombocytopenia, polyclonal hypergammaglobulinemia, and elevated serum levels of IL-6, LDH and C-reactive protein are commonly observed in MC castleman disease. Patients with multicentric CD are treated with chemotherapy and steroids. ...
... 5,6 They typically present with fever, fatigue, excessive seating, weight loss and skin rash. 7 It can be either idiopathic (iMCD) or HHV-8 associated MCD. 8 Based on clinical features, iMCD is further subdivided into iMCD-Thrombocytopenia, anasarca, fever/elevated C-reactive protein (CRP), renal dysfunction/reticulin myelofibrosis, organomegaly (TAFRO) and iMCD-Not otherwise specified (NOS). 9 Pathophysiology of Human herpes virus 8 (HHV-8) associated multicentric CD (MCD) is postulated to be excess production of interleukin-6 (IL-6) in germinal centers and at increased risk of developing Kaposi sarcoma. ...
Article
Full-text available
p class="abstract"> Background: Castleman’s disease (CD) is a rare lymphoproliferative disorder. It can involve single (unicentric CD) or multiple (multicentric CD) lymph nodal regions. It occurs predominantly in mediastinum, and treatment options include surgery, radiotherapy, chemotherapy, and monoclonal antibodies. Methods: Here, we describe two cases of CD which presented with stridor. The first case was a 38-year-old male, a recurrent multicentric CD in retropharyngeal and cervical lymph nodal regions, treated with radiotherapy and rituximab. The second case was a 25-year-old male, a multicentric CD in lower cervical lymph nodal region, treated with steroids and radiotherapy. He subsequently developed Hodgkin’s lymphoma and was treated for the same with chemotherapy and involved-field radiation therapy (IFRT). Results: Post-treatment, both the patients were asymptomatic and progression-free at 15 months and 42 months follow-up, respectively. Conclusions: Combined modality of treatment with radiotherapy and chemotherapy or monoclonal antibodies offers good local control in multicentric CD. </p
... Multicentric CD may be associated with HIV and POEM's syndrome. 9 Elevated erythrocyte sedimentation rate, anemia, thrombocytopenia, polyclonal hypergammaglobulinemia, and elevated serum levels of IL-6, LDH and C-reactive protein are commonly observed in MC castleman disease. Patients with multicentric CD are treated with chemotherapy and steroids. ...
... Multicentric Castleman disease with and without peripheral neuropathy tend to be different; it has even been proposed that the presence or absence of peripheral neuropathy should be part of the multicentric Castleman disease classification system.136 Those patients with peripheral neuropathy are more likely to have edema and impaired peripheral circulation,133,[137][138][139][140][141][142] and they are also more likely to have a monoclonal lambda protein in their serum and/or urine.143 Between 11% and 30% of POEMS patients who have a documented clonal plasma cell disorder also have documented Castleman ...
Article
Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. Diagnosis: The diagnosis of POEMS syndrome is made with 3 of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy RISK-ADAPTED THERAPY: For those patients with a dominant sclerotic plasmacytoma, first-line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3-6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low-dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti-VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
... Multicentric Castleman disease can be encountered with or without peripheral neuropathy [29]. Castleman with peripheral neuropathy is more likely to also demonstrate edema and a monoclonal protein [30]. In various series, the percentage of POEMS with Castleman ranges from 11 to 25% [5-7, 9, 31]. ...
Article
Full-text available
POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman’s disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis. Treatment is based on extent of the disease. Radiotherapy is used for localized disease. Systemic therapy is required for disseminated disease, with bone marrow involvement by clonal plasma cells, or in patients who progress shortly after radiation. Upfront autologous stem cell transplantation is the treatment of choice for transplant-eligible patients. Outcomes are typically superior to that of standard myeloma. Herein, using a case vignette, we outline the latest evidence regarding the prognostication and management of POEMS syndrome, with a focus on its relapsing-remitting course.
... On the other end of the spectrum were patients (n 5 23) with sheetlike plasmacytosis and increased numbers of follicles with large hyperplastic GCs. 32 These cases, which represent the "plasmacytic" (PC) subtype of iMCD, also have occasional regressed GCs and mild vascularity. A subset of cases (n 5 19) demonstrated histologic features that were intermediate between the HyperV and PC subtypes with regressed lymphoid follicles and plasmacytosis, which were considered "mixed." ...
Article
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Key Points An international panel established the first ever diagnostic criteria for iMCD based on review of 244 clinical cases and 88 tissue samples. The criteria require multicentric lymphadenopathy with defined histopathology, ≥2 clinical/laboratory changes, and exclusion of iMCD mimics.
... Nearly, 50% of these patients may present with systemic manifestations of anemia, fevers, weight loss, fatigue, or hyperglobulinemia. [9] Cutaneous symptoms such as paraneoplastic pemphigus, erythema multiforme, or lichen planus have been described. [10] These patients are more likely to have multifocal disease and have worse outcomes. ...
Article
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Unicentric Castleman disease (UCD) is a relatively rare lymphoproliferative disease, which commonly presents as a mediastinal mass and less frequently involves abdomen, pelvis, and retroperitoneum. We report a case of a 64-year-old man with newly diagnosed low-volume, Gleason 3 + 3 = 6 prostate adenocarcinoma, who in considering active surveillance versus treatment was found to have a left perivesical and iliac chain lymphadenopathy concerning for potential metastatic involvement. He underwent magnetic resonance imaging with ferumoxytol to assist in the diagnostic evaluation to better characterize his lymphadenopathy. Subsequently, he underwent robotic-assisted laparoscopic bilateral pelvic lymph node dissection and resection of left perivesical mass exhibiting hyaline vascular variant of UCD.
... The hyaline-vascular variant accounted for 76% of unicentric disease, similar to previously published reports (76-91%). 13,15,16 The hyaline-vascular variant occurred in a unicentric distribution in 78% of cases (n 5 14) (multicentric in 22%), similar to a previously published report (74%), 15 although lower than that quoted elsewhere in the radiology literature. 8,10 There has been a suggestion that heterogeneous enhancement is more common in lesions .5.0 cm. 10 We found no association between the size of the lesion and enhancement pattern (homogeneous vs heterogeneous) or the degree of enhancement, although our evaluation was limited owing to our small sample size. ...
Article
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Objective: To analyse imaging features of subtypes of Castleman disease (CD), emphasizing differentiating features from lymphoma. Methods: Institutional review board-approved, Health Insurance Portability and Accountability Act compliant, retrospective study examined 30 patients with CD. 30 patients (females, 20; mean age, 46 years; range, 22-87 years) with histopathologically confirmed CD and pre-treatment imaging formed the analytic cohort. Imaging at presentation in all patients [CT, 30; positron emission tomography (PET)/CT, 5; MR, 4; ultrasound, 3] and subsequent imaging in three cases that developed lymphoma was reviewed by two radiologists in consensus. Results: Subtypes: hyaline-vascular (n = 18); multicentric not otherwise specified (NOS) (n = 6); human herpesvirus 8 associated (n = 2); mixed unicentric (n = 2); pure plasma-cell variant (n = 1); and unicentric NOS (n = 1). Distribution: unicentric (n = 17); and multicentric (n = 13). Nodal sites-unicentric: 13 thoracic, 3 abdominal and 1 cervical; multicentric: 9 abdominal, 8 thoracic, 6 cervical, 5 inguinal, 4 axillary and 4 supraclavicular. On CT, differentiating features from lymphoma were calcification (n = 8; 26.7%) and heterogeneous enhancement (n = 5; 19.2%). No association between CD subtype, degree or enhancement pattern, or calcification was noted. On PET/CT (n = 5), nodes were typically fluorine-18 fludeoxyglucose avid (n = 4). On ultrasound (n = 3), nodes were hypoechoic, homogeneous with posterior acoustic enhancement. On MR (n = 4), nodes were hypointense (n = 2) to isointense (n = 2) on T1 weighted images and isointense (n = 1) to hyperintense (n = 3) on T2 weighted images. All (n = 4) demonstrated homogeneous enhancement. Three cases developed non-Hodgkin's lymphoma, two of the three had larger spleens, and these cases had effusions/ascites. Conclusion: CD can be unicentric or multicentric and involve nodes above and below the diaphragm. Patients with CD can develop lymphoma. Advances in knowledge: Assessing individual risk of developing lymphoma in patients with CD is difficult, although the findings of splenomegaly, pleural effusion and ascites may be suggestive.
... Las otras linfadenopatías con arquitectura folicular que pueden interpretarse erróneamente como linfomas son la enfermedad de Castleman y la transformación progresiva de centros germinales; sin embargo, ambas tienen características histológicas que hace posible identificarlas y distinguirlas. [40][41][42][43][44][45][46][47][48] ...
... Alguns estudos abertos não controlados, onde a DC foi tratada com prednisona isolada, demonstraram resultados divergentes, porém existe um consenso que seu uso é particularmente útil em casos de doença leve a moderada. Em casos de doença agressiva, a resposta ao uso isolado da prednisona é geralmente parcial (19,(22)(23)(24)(25)(26) . Existe um aumento da freqüência de infecção na DC, que é a causa mais comum de morte em pacientes com esta condição. ...
Article
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Castleman's disease (CD) is rare nonmalignant lymphoproliferative illness, of unknown etiology, clinically characterized by isolated or multiple adenomegalies, associated or not with systemic symptoms such as fever and weight loss. These symptoms can lead to a wrong diagnosis of autoimmune illness and the differential diagnosis must be made through histological examination of involved lymph nodes, which shows, in the case of CD, a pattern of plasma cells with hyaline infiltration. A case of a woman of 24-year-old, who initially presented fever, polyarthritis and skin rash, suggestive adult-onset Still's disease is reported; the histological examination confirmed the diagnosis of Castleman's disease.
... The plasma cell variant (PC-CD) has been identified in 9% to 24% of CD cases, and a rare hyalinevascular plasma cell (or mixed) subtype has also been reported [2,8,9]. Clinically, HV-CD is most often found in indolent unicentric CD tumors (UCDs), and a more generalized (multicentric) lymphadenopathy, which frequently presents with constitutional symptoms, is associated with PC-CD [2,10]. ...
Article
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Castleman's disease is a rare and poorly understood disease entity that may resemble more common conditions and represents a clinical challenge to the treating surgeon. In this report, we describe a case of a 61-year-old Caucasian woman with a symptomatic retroperitoneal mass. The specimen obtained from her resection contained a protuberant encapsulated mass, exhibiting microscopic features consistent with localized, unicentric Castleman's disease. These characteristics included architectural features and immunohistochemical findings consistent with the hyaline vascular variant of Castleman's disease. We report a very rare case of a retroperitoneal hyaline vascular type of Castleman's disease. We discuss the diagnostic dilemma Castleman's disease may present to the surgeon, with an emphasis on multidisciplinary management of these patients. We also review current data on pathogenesis, treatment and outcomes.
... This could be a reason for the different FDG-PET/CT results. HV-type is generally unicentric, 10 although multicentric disease has been reported. 11 HV-type is not thought to be related to HHV8; 12 its pathogenesis is still unknown, even if vascular endothelial growth factor may have an important role. ...
Article
Now that [18F] fluorodeoxyglucose positron emission tomography (FDG-PET) has become an established imaging tool in oncology, it is attracting interest in the field of infectious diseases. Several studies have used FDG-PET to examine the pathophysiology of HIV infection as well as other conditions such as lipodystrophic syndrome and HIV-related neurocognitive disorders. In clinical practice, FDG-PET has been proposed to assess fever of unknown origin or with lymphoproliferative disorders such as Castleman disease in individuals with HIV infection.
... 3 A multicentric form with systemic symptoms was initially described by Gaba et al. 8 An expanded review of multicentric CD by Weisenburger et al. 20 noted a selected group of patients with the PC subtype and systemic symptoms who had a reported median survival of only 26 months. Menke et al. 14 retrospectively examined 26 patients with multicentric CD with or without clinical signs of neuropathy. This study demonstrated no significant difference in survival between patients with unicentric disease and the multicentric (PC) type without neuropathy. ...
Article
BACKGROUND Castleman's disease (CD), or angiofollicular lymph node hyperplasia, creates both diagnostic and therapeutic dilemmas for most physicians. For patients with this rare and poorly understood disease, the optimal therapy is unknown. The authors report their experience during the years 1986–1997 with this uncommon clinicopathologic entity.METHODS Sixteen patients with a histologic diagnosis of CD were identified in the pathology database. Unicentric disease was defined as a solitary mass. Multicentric disease compromised patients with widespread lymphadenectomy. Clinical, radiologic, and laboratory data were analyzed to evaluate treatment response.RESULTSThe study group consisted of 16 patients classified into 3 clinicopathologic groups: hyaline-vascular, plasma cell, and “mixed.” Of those patients who underwent complete surgical excision of a unicentric hyaline-vascular CD mass (n = 8), all remain symptom free without clinical or radiographic recurrence. Two patients remain asymptomatic following partial resection or radiation therapy for an unresectable unicentric hyaline-vascular CD mass. Two patients with multicentric hyaline-vascular CD are currently in complete remission following adjuvant therapy. Multicentric plasma cell CD was present in a single patient. This patient (who underwent surgical and systemic therapy) died of disease within 4 months of presentation. Three patients with unicentric hyaline-vascular/plasma cell-CD remain symptom free following either complete resection or observation.CONCLUSIONS The authors recommend surgical resection for patients with the unicentric variant of CD. Surgical removal of a unicentric mass of hyaline-vascular or hyaline-vascular/plasma cell type is curative. Partial resection, radiotherapy, or observation alone may avoid the need for excessively aggressive therapy. Patients with multicentric disease, either hyaline-vascular or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy, the nature of which has yet to be defined. Cancer 1999;85:706–17. © 1999 American Cancer Society.
... CD presents two main histological variants: hyaline vascular type (80-90% of cases) and plasma cell type (10-20%). Hyaline vascular CD is found in 90% of localized forms, but it is rare in MCD, as in our second reported case (2,3,8). ...
Article
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Castleman's Disease is a rare tumour involving lymph node tissues; a case of benign localized disease and a case of rapid progressive multicentric disease are reported. Case report 1: A 19-year-old man presented with four months of hypogastric and left iliac pain. Castleman's Disease was suspected after CT-scan. A CT-guided fine-needle biopsy of the lesion was performed revealing hyaline vascular type Castleman's Disease. The patient underwent open surgery with radical excision of the lesion. No adjuvant therapy was performed after surgery. The patient is alive and disease-free after 24 months. Case report 2: A 58-year-old woman presented with a right axillary palpable lymph node and vague abdominal discomfort. Abdomen CT demonstrated hepatosplenomegaly associated with adenopathy at the hepatic hilus and splenic hilus; dilatation of intra-hepatic biliary ducts was present. The axillary node was excised, the mass at hepatic hilus was biopsied. The diagnosis was Castleman's Disease in both sites. In course of steroid therapy retroperitoneal multiple nodes appeared associated with fast-progressive mechanic jaundice and liver failure. Progressive multi-organ failure arose within 1 week, with irreversible clinical worsening to death.
... Several published cases of ''interesting features'' associated with Castleman disease are likely cases of POEMS syndrome [112][113][114][115]. Multicentric Castleman disease with and without peripheral neuropathy tend to be different; it has even been proposed that the presence or absence of peripheral neuropathy should be part of the multicentric Castleman disease classification system [116]. Those patients with peripheral neuropathy are more likely to have edema and impaired peripheral circulation, [117][118][119][120][121][122][123][124] and they are also more likely to have a monoclonal lambda protein in their serum and/or urine [125]. ...
Article
Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. Risk-adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3-6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti-VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol., 2015. © 2015 Wiley Periodicals, Inc.
... 2,17,30,34 Results of radiotherapy may be more favorable in patients with the plasma cell variant of the disease. 2 Multicentric Castleman disease tends to affect an older population than does unilateral Castleman disease, and it is more often associated with systemic symptoms, abnormalities on laboratory tests, and abnormal examination findings including hepatosplenomegaly, adenopathy, and mo-tor-sensory neuropathy, with the latter portending a worse prognosis. 2,7,17,23,26,28 Median survival durations as short as 26 months have been reported in the multicentric Castleman disease group, whereas patients with the hyaline-vascular variant may follow a more benign course and have better long-term survival rates. 2,35 In either case, the clinical course may be variable. ...
Article
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Castleman disease is a rare lymphoproliferative disease of unknown cause. In most cases, afflicted patients present with a mediastinal mass although the disease may manifest in numerous other sites, including intracranially and rarely intraspinally. The authors report on the case of a 19-year-old woman who presented with a large paraspinal mass emanating from the T7–8 neural foramen. The morphological and signal characteristics revealed on magnetic resonance imaging were similar to those of nerve sheath tumors. The patient underwent open biopsy sampling of the lesion, and results of a pathological evaluation revealed hyaline-vascular type Castleman disease. She underwent radiotherapy and remains symptom-free with a radiographically stable lesion 1 year later. Although the disease has been reported to mimic a meningioma when encountered in intracranial locations, to the authors’ knowledge, this is the first case of the disorder mimicking a nerve sheath tumor. When the diagnosis of Castleman disease is made, good results can be obtained with partial resection and radiotherapy.
Article
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We report here a case of solitary form of Castleman's disease (hyaline-vascular type) with its unusual location in the neck. The case is being reported for its rarity.
Article
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Disease Overview POEMS syndrome is a life‐threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnosis The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. Risk Stratification Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate. Risk‐Adapted Therapy For those patients with a dominant plasmacytoma, first‐line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low‐dose conventional therapy or as high‐dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
Article
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Disease overview POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnosis The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. Risk stratification Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. Risk‐adapted therapy For those patients with a dominant plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
Article
Castleman disease (CD) describes a group of at least four disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), HHV8-associated MCD (HHV8-MCD), and POEMS-associated MCD. iMCD can be further sub-classified into iMCD-TAFRO (thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, organomegaly) or iMCD-NOS (not otherwise specified), who often have thrombocytosis and hypergammaglobulinemia. Advances in diagnosis, classification, pathogenesis, and therapy are substantial since the original description of UCD by Benjamin Castleman in 1954. The advent of effective retroviral therapy and use of rituximab in HHV8-MCD have improved outcomes in HHV8-MCD. Anti-interleukin-6 directed therapies are highly effective in many iMCD patients, but additional therapies are required for refractory cases. Much of the recent progress has been coordinated by the Castleman Disease Collaborative Network (CDCN), and further progress will be made by continued engagement of physicians, scientists, and patients, the last of which can be facilitated by encouraging patients to self-enroll in the CDCN's ACCELERATE natural history registry (#NCT02817997, www.CDCN.org/ACCELERATE).
Chapter
A variety of reactive and neoplastic hematolymphoid disorders may be found in the head and neck. Some lymphomas have a predilection for mucosal, extranodal sites, but almost any lymphoma may present in a neck lymph node. In addition the head and neck are the primary locations for a variety of soft tissue lesions. Soft tissue lesions can be difficult to diagnose given the morphologic overlap. Knowledge of the clinical presentation and location with respect to the head and neck can help narrow the differential diagnosis. This chapter aims to discuss common soft tissue and hematopoietic tumors of the head and neck as they relate to specific sites and specific entities in the differential diagnosis.
Article
Castleman's disease (CD) is a rare atypical lymphoproliferative disorder that is morphologically and clinically heterogenous and is associated with a risk of developing malignant lymphoma. We report the clonality status of CD tissues in 34 patients, including 14 patients infected by the human immunodeficiency virus (HIV). Four patients presented a localized form and 30 presented a multicentric form. Two cases were associated with B-cell lymphoma, 3 cases with Hodgkin's disease, and 9 cases (8 HIV+) with Kaposi's sarcoma. Histologically, 8 cases were of the hyaline-vascular type and 26 were of the plasma cell or mixed types. The Ig and T-cell receptor (TCR) V(D)J rearrangements were analyzed using polymerase chain reaction and Southern blot. Clonal IgH rearrangements were detected in only 4 cases, ie, 2 associated with B-cell lymphoma, 1 with Hodgkin's disease, and 1 case without malignancy. A TCR gamma rearrangement of restricted junctional size was amplified in 1 HIV+ case. Finally, polyclonal VH-JH and V gamma-J gamma rearrangements were detected in the large majority of the cases, irrespective of pathologic subtypes, clinical forms, and HIV status. The lymphoid component in CD is therefore commonly reactive, and the rare occurrence of detectable monoclonal lymphoid contingents may be caused by secondary molecular events.
Article
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Disease Overview Polyneuropathy, organomegaly, endocrinopathy, M‐protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. Diagnosis The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. Risk Stratification Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. Risk‐Adapted Therapy For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
Article
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a rare paraneoplastic disorder. The polyneuropathy can be the presenting symptom and is typically a painful, motor-predominant polyradiculoneuropathy often mimicking chronic inflammatory demyelinating polyradiculoneuropathy. The presence of a lambda monoclonal protein, elevated vascular endothelial growth factor, systemic features, and treatment resistance are clues to the diagnosis. Castleman disease (CD) is seen in a subset of these patients, and when present the neuropathy is similar but less severe. In contrast, in those patients with purely CD, the neuropathy is often a mild, painless distal sensory neuropathy.
Article
POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease is the first step in managing the disease. Increased blood levels of vascular endothelial growth factor are usually confirmatory. This rare disorder should not be missed, especially if the patient has a putative diagnosis of chronic inflammatory polyradiculoneuropathy, a lambda restricted monoclonal gammopathy, and thrombocytosis, and is not responding as expected to immunomodulatory therapy commonly used for chronic inflammatory polyradiculoneuropathy.
Article
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Introduction: Castleman Disease (CD) is a rare lymphopro-liferative disorder with heterogenous clinical and pathological features. It is a rare disease with mention in the rare disease data of the orphanet. It can present as unicentric or multicentric disease. Hyaline vascular variant and plasma cell variant are the two pathological subtypes. Hyaline vascular variant accounts for nearly 80% to 90% of unicentric cases. Hyaline vascular subtype variant has follicular and stroma rich subtype. Aim: To study the histomorphologic spectrum of hyaline vascular variant of CD. Materials and methods: Retrospective cross-sectional, observational study was undertaken from the archival data between January 2009 and April 2015. Only ten cases of hyaline vascular CD were identified after studying the histomorphological characteristics. Both follicular and interfollicular changes were studied in detail. Results: The age of presentation ranged from 17 years to 59 years. Seven out of 10 cases were female. Site of presentation included cervical, inguinal, retroperitoneal, intra-abdominal and axillary. Six cases showed predominant follicular change. Two cases were sclerotic subtype. Two cases showed both follicular and interfollicular changes in equal proportion. Distribution of follicles throughout the lymphnode was seen in eight cases. Uniform sized follicles seen in seven out of ten cases. Small germinal centre with lymphocyte depletion was one of the uniform features seen in all 10 cases. Numerous high endothelial vessels were seen in nine cases. Twinning of germinal centre was seen in two cases. All ten cases showed concentric rings of small lymphocytes. Lollipop pattern was relatively rare feature seen in only two cases. Conclusion: Hyaline vascular variant of CD has considerable morphologic variation with few consistent features seen in most of the cases.
Article
Objective: To define the peripheral neuropathy phenotypes associated with Castleman disease. Methods: We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). We used a cohort of patients with POEMS as controls. Clinical, electrodiagnostic, and laboratory characteristics were collected and compared among patient subgroups. Results: There were 7 patients with CD-PN, 20 with CD-POEMS, and 122 with POEMS. Patients with CD-PN had the mildest neuropathy characterized by predominant sensory symptoms with no pain and mild distal sensory deficits (median Neuropathy Impairment Score of 7 points). Although both patients with CD-POEMS and patients with POEMS had a severe sensory and motor neuropathy, patients with CD-POEMS were less affected (median Neuropathy Impairment Score of 33 and 66 points, respectively). The degree of severity was also reflected on electrodiagnostic testing in which patients with CD-PN demonstrated a mild degree of axonal loss, followed by patients with CD-POEMS and then those with POEMS. Demyelinating features, defined by European Federation of Neurologic Societies/Peripheral Nerve Society criteria, were present in 43% of the CD-PN, 78% of the CD-POEMS, and 86% of the POEMS group. Conclusion: There is a spectrum of demyelinating peripheral neuropathies associated with Castleman disease. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe sensory and motor neuropathy. Compared to the POEMS cohort, those with CD-POEMS neuropathy have a similar but less severe phenotype. Whether these patients respond differently to treatment deserves further study.
Chapter
POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The dominant feature is typically peripheral neuropathy, and the hematologic manifestations can be subtle leading to delayed diagnosis. Although the mechanism of disease is not well understood, the disease is very treatable, and survival is typically excellent. Castleman?s disease (CD) can occur with and without POEMS syndrome. Patients with unicentric CD have an excellent prognosis with local therapies; outcomes are less good, and therapies are less well established for the patients with multicentric CD with the exception of interleukin-6 and interleukin-6 receptor antibodies, which are useful in a subset of patients.
Article
Castleman's disease (CD) of the head and neck is often a diagnostic problem due to paucity of sings and symptoms and its ability to mimic other neoplasms. Two histologic subtypes are described; hyaline vascular and plasma cell. Definitive diagnosis and treatment is possible with complete surgical resection. A solitary neck mass diagnosed as hyaline vascular type CD in a 36-year-old woman was reported. The patient was treated by surgical excision.
Article
Castleman's disease (CD) is an uncommon benign disease that causes progressive lymph node enlargement. Most observations of CD have focused on adult patients and it rarely occurs in children. It usually arises as a solitary mass and is commonly found in the mediastinum and abdomen and less commonly in the head, neck and axilla.The diagnosis of CD is histopathological. We report a child of CD in the neck region who also had a mass at the nasopharenx mimicking nasofaringeal carcinoma and review the published child cases with CD that involved neck region. This report emphasizes that CD should be considered in the differential diagnosis of children presented with neck mass.
Article
Multicentric Castleman's disease (MCD) is an uncommon lymphoproliferative disorder, often associated with a clinically aggressive behavior. No standard treatment has been established, but patients are usually treated with lymphoma-type regimens such as rituximab or combination chemotherapy. Recently, immunotherapies targeting IL-6 have proven effective and have been approved for this indication. However, these agents require long-term administration. Here, we describe the clinical course of two patients, refractory to rituximab and chemotherapy, showing long-term remission (18 and 24 months), following an induction phase with tocilizumab (an anti-IL-6 receptor antibody) and a consolidative phase with high-dose melphalan accompanied by autologous stem cell support. This may prove to be an effective option for this group of patients with an orphan disorder. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.
Article
This is the first reported case of multicentric Castleman's disease (MCD) associated with renal amyloidosis and pure red cell aplasia (PRCA). Inguinal lymph node biopsy showed follicular hyperplasia with prominent germinal centers and plasma cell proliferation in the interfollicular areas. Renal biopsy specimens revealed intraglomerular amyloid deposits, defined as AA amyloidosis. Since amyloid deposits were not present in the gastric and rectal mucosal tissues, his renal disorder was found to be an unusual secondary amyloidosis associated with MCD. Following treatment by plasma exchange, there was progressive deterioration of anemia due to PRCA detected by bone marrow aspiration. Subsequently he was successfully treated with steroid pulse therapy not only for anemia but also for renal function.
Article
Castleman's disease is an atypical lymphoproliferative disorder of unknown origin. It has three histologic types (hyaline vascular, plasma-cell, and mixed) and two clinical types (localized and multicentric). Some systemic diseases may be accompanied with Castleman's disease, but it is unclear if Castleman's disease is relevant to rheumatoid arthritis. We experienced a case of Castleman's disease suffering from rheumatoid arthritis. A 25-year-old female with rheumatoid arthritis was referred to our hospital with a palpable mass lesion in the left upper arm. Excisional biopsy of the mass and arthroscopic synovectomy were performed, and the histological finding was compatible with hyaline-vascular type of Castleman's disease. Herein, we report this case along with brief review of relevant literature.
Article
Castleman's Disease is a rare tumour involving lymph node tissues; a case of benign localized disease and a case of rapid progressive multicentric disease are reported. Case report 1: A 19-year-old man presented with four months of hypogastric and left iliac pain. Castleman's Disease was suspected after CT-scan. A CT-guided fine-needle biopsy of the lesion was performed revealing hyaline vascular type Castleman's Disease. The patient underwent open surgery with radical excision of the lesion. No adjuvant therapy was performed after surgery. The patient is alive and disease-free after 24 months. Case report 2: A 58-year-old woman presented with a right axillary palpable lymph node and vague abdominal discomfort. Abdomen CT demonstrated hepatosplenomegaly associated with adenopathy at the hepatic hilus and splenic hilus; dilatation of intra-hepatic biliary ducts was present. The axillary node was excised, the mass at hepatic hilus was biopsied. The diagnosis was Castleman's Disease in both sites. In course of steroid therapy retroperitoneal multiple nodes appeared associated with fast-progressive mechanic jaundice and liver failure. Progressive multi-organ failure arose within 1 week, with irreversible clinical worsening to death
Article
Castleman disease is a rare idiopathic non-neoplastic lymphoproliferative disorder with 2 clinical (unicentric and multicentric) and 3 histomorphological (hyaline-vascular, plasma-cell and mixed) forms identified. The case report given here describes the 3-year experience with therapy in a patient, male born 1961, diagnosed with multicentric plasma-cell Castleman disease (HIV and HHV-8 negative) with the finding of genera-lized lymphadenopathy and splenomegaly. During first line treatment (R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, 3 cycles in total, 12/2008-2/2009) the development of bilateral upper and lower limb edemas with clinical manifestation of vasculitis occurred and a restaging computed tomography (CT) examination revealed a stable finding of the lymphadenomegaly. Greater success was achieved with thalidomide regimen (CTD: cyclophosphamide, thalidomide, dexamethasone, 10 cycles, 3/2009-1/2010) leading to reduction in the size of the hypervascularized lymph nodes (almost by 50%) as well as their radiopharmaceutical (fluorodeoxyglucose) uptake as seen on a combined positron emission tomography and computed tomography (PET/CT) scan imaging. Thalidomide was given daily at doses between 100 and 200 mg. We returned to the CTD regimen again in April 2010 after a short period of monoclonal antibody tocilizumab treatment (400 mg intravenous in 2-week intervals with 50% dose reduction due to a limited supply of the drug, 5 doses in total) during which edemas reoccurred with a CT scan finding of stable lymphadenomegaly. However, the renewed regimen with thalidomide was stopped after 2.5 cycles due to adverse effects of thalidomide (neuropathy) and corticoids (Cushing syndrome). In September 2010, after enrollement in the Celgenes Compassionate Use Program we were able to start treating the patient with the derivative of thalidomide, lenalidomide, at a dosage of 25 mg on days 1-21 in a 28-day cycle, 15 cycles in total (10/2010-12/2011). The monotherapy with lenalidomide was very well tolerated by the patient without any effects of myelotoxicity, thromboembolism or relapses of edemas and vasculitis, additionally now with apparent improvement of fatic disorder and the patients motor abilities. Thus, lenalidomide represents an attractive alternative agent for patients with Castleman disease after rituximab and cytostatics failures. It has a favourable safety profile and could be therefore considered for administering in first line treatment. Key words: Castleman disease - glucocorticoids - chemotherapy - rituximab - thalidomide - monoclonal antibody - tocilizumab - lenalidomide - positron emission tomography - computed tomography.
Article
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Castleman's disease (CD) is a rare disorder of unknown aetiology characterized by lymph node enlargement. This study evaluated the clinical features, diagnosis and outcomes of surgical treatment in patients with CD. The medical records of 10 patients with histologically confirmed CD who underwent surgery between 2003 and 2008 were retrospectively reviewed. Study endpoints were survival and recurrence. The age of disease onset ranged from 11 to 63 years (median 38 years). Of the 10 patients included in the study, eight were diagnosed with localized (unicentric) CD and two with multicentric CD. All eight patients with localized (unicentric) CD were asymptomatic. Histological evidence indicated the hyaline vascular form of CD in eight patients and the plasmacytic form in the remaining two patients. The primary treatment was complete surgical resection in nine cases, and these patients were alive with no evidence of recurrence or exacerbation at the time of writing (June 2011). Localized (unicentric) CD is usually of the hyaline vascular form and complete surgical excision of the tumour allows full recovery in most cases.
Article
GENEL BİLGİ Mediasten, zengin lenf nodu ve lenfatik ağına sahiptir. Mediasten içindeki farklı organlardan, boy-undan ve diyafragmanın altından mediastinal lenf nodlarına yaygın lenf akımı mevcuttur. Lokalize inflam-atuar hastalık sonucu veya primer lenfatik tümör, toraksın içinden, göğüs duvarından, memeden veya uzak bir organdan mediastinal lenf nodu etkilenir. Mediastendeki lenf nodları üç ayrı grup altında toplanır (1). 1. Anterior Bölüm: Bu gruptaki lenf nodları ikiye ayrılır. I. Sternal (anterior paryetal veya internal mamaryan) grup II. Anterior mediastinal (prevasküler) grup Sternal grup internal mamaryan arterin her iki tarafında bulunur ve ön abdominal, göğüs duvarını, diyafragmanın ön parçasını ve memenin iç kısmını drene ederler. Prevasküler grup, timusun ön ve lateralinden, büyük damarların önünde uzanır. Bunlar daha çok medias-tenin ön lenf nodu grubu olarak da bilinir. Prevasküler lenf nodları, perikardiyumun ön bölümünü, kalbin ön bölümünü, timusu, tiroidi ve mediastinal plevranın ön bölümünü drene ederler. Ayrıca özellikle sol akciğerin üst lobu da buraya drene olur. 2. Visceral (orta) Bölüm: Bu bölümde daha çok akciğer ve özefagusu drene eden lenf nodları bulunur (2-4). Bunun dışında daha az dikkat çeken paryetal grup lenf nodları tanımlanmıştır. Paryetal grup lenf nodları, diyafragmaya yakın perikardın çevresinde bulunur. Ayrica frenik sinirin diyafragmayı geçtiği yerde de bulunurlar.
Article
Giant lymph node hyperplasia (GLNH) or Castle- man disease is a heterogenous group of atypical lympho- proliferative disorders. Two main histologic variants, the hyaline vascular variant and the plasma cell variant, have been recognized. Although localized GLNH can often be managed successfully with surgery, optimal therapy for multifocal disease has yet to be identified. We report two cases of GLNH treated with 2-chloro-deoxyadenosine (2- CDA), a synthetic purine analogue. 2-CDA was utilized based on its relative lymphocytic toxicity and the putative pathophysiologic process in GLNH being either hamarto- matous overgrowth (hyaline-vascular variant) or immune dysfunction and lymphoproliferation (plasma cell variant). One patient with unresectable localid hyaline-vascular GLNH has had a 9-month continuous complete remission following two courses of 2-CDA therapy followed by radiation therapy. The second patient with disseminated plasma cell type had a partial response to two cycles of 2- CDA therapy; however, further cycles were not given due to development of possible early neurotoxicity. Although the optimal management of non-resectable GLNH is yet to be determined, 2-CDA appears to be a viable therapeutic option for patients with this disease process.
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Castleman's disease (CD) is a rare disease of lymph node with two identified forms - the hyaline vascular (HV) type or angiofollicular type and plasma cell (PC) type. It usually presents as localized or systemic lymphadenopathy or even as extra nodal mass and may give rise to several differential diagnoses. Fine needle aspiration cytology (FNAC), as the initial investigation, may be misleading. Excision biopsy usually gives the diagnosis. This study analyzes the clinical, cytological and histological features of CD of HV type. All the cases of CD reported between 2001 and March 2008 have been reviewed. Cytology and histopathology slides of all cases were reviewed by two reporting pathologists independently and the clinical records were analyzed. We had five cases of CD all of which presented as cervical lymphadenopathy of 1 to 5 cms in young patients. Patients were mostly asymptomatic with all relevant investigations within normal limits. The cytology findings mostly showed a predominance of small lymphocytes with capillary fragments. On excision biopsy, all cases were diagnosed as CD of HV type. CD should be kept as a differential of lymphadenopathy, especially in asymptomatic and young patients.
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