Castleman Disease: An Update on Classification and the Spectrum of Associated Lesions

Department of Pathology, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94304, USA.
Advances in anatomic pathology (Impact Factor: 3.23). 08/2009; 16(4):236-46. DOI: 10.1097/PAP.0b013e3181a9d4d3
Source: PubMed


Since its initial description, researchers have expanded the spectrum of Castleman disease to include not only the classic and well-recognized hyaline-vascular type, but also the plasma cell type and multicentric types of broader histologic range, including human herpes virus-8-associated Castleman disease. These less common subtypes of Castleman disease are less familiar, and may be under-recognized. Also of practical importance, current authors are restructuring the classification of multicentric Castleman disease to accommodate the emerging pathogenic role of human herpes virus-8 and its association with the recently described plasmablastic variant. In addition to an increased risk of lymphoma, patients with Castleman disease also are at increased risk for other related neoplasms, including Kaposi sarcoma and follicular dendritic cell tumors, which are of prognostic and therapeutic relevance. This review focuses on the histologic diagnosis of Castleman disease, current and emerging concepts in its pathogenesis and classification, and associated histopathologic entities.

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    • "Often, a single penetrating vessel is seen in the center of the follicle. Plasmacytoid dendritic cell collections may be found to be associated with these lesions [2]. The aetiology of hyaline vascular CD remains unclear, but some relationship has been reported with dysplastic follicular dendritic cells [7]. "
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    ABSTRACT: Localised nasopharyngeal Castleman disease has rarely been reported. We present a case involving a 23-year-old female, describe the clinical, imaging, and histopathologic features of this challenging diagnosis, and review the literature.
    Full-text · Article · Jul 2014
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    • "Approximately 90% of all localized forms are the HV type, but most of the systemic forms are the PC type [13]. Some studies suggest that the histopathologic type should primarily be used for classification purposes [14, 15]. However, no differences in outcomes were noted when comparing the PC and the HV types in the patient groups with UC or MC disease [5]. "
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    ABSTRACT: Castleman's disease (CD) is a rare lymphoproliferative disorder that can involve single or multiple lymph nodes in the body. Especially, the localized form of CD is known to be well-controlled by using a surgical resection. On occasion, the surgeon may confront an abdominal and retroperitoneal mass of unknown origin. Thus, we present this case in which we treated a 16-year-old female patient for CD and investigated how to evaluate and manage the situation from the standpoint of CD. Also, we give a review of the pathology, clinical manifestation, diagnosis, and treatment of CD.
    Full-text · Article · Apr 2014 · Annals of Coloproctology
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    • "The plasmablastic variant of the disease is commonly associated with HHV-8 and tends to have a poor prognosis. It occurs more frequently in patients who are HIV positive, although there seems to be no correlation with CD4 count [15]. The multicentric and, especially, HHV-8-positive types are risk factors for the development of Hodgkin lymphoma, non-Hodgkin lymphoma and Kaposi sarcoma. "
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    ABSTRACT: Castleman's disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman's disease have been reported in the literature. A 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10x9x5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman's disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected. Castleman's disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.
    Full-text · Article · Mar 2014 · Journal of Medical Case Reports
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