[Show abstract][Hide abstract] ABSTRACT: Rare cases of Merkel cell carcinoma have been encountered in lymph nodes with unknown extranodal primary, which exhibit similar morphologic and immunophenotypic features to those in primary cutaneous Merkel cell carcinomas. However, it is uncertain whether the nodal Merkel cell carcinoma is a primary tumor of the lymph node or represents a metastasis from an occult or regressed extranodal lesion. To establish an accurate diagnosis of the nodal Merkel cell carcinoma can be challenging because of significant morphologic mimics, including lymphoblastic lymphoma and metastatic small cell carcinoma. Moreover, there is no consensus for a diagnostic term, and many different terms have been used, which can be confusing and may not fully reflect the nature of nodal Merkel cell carcinoma. In this study, we investigated the detailed clinicopathologic features of 22 nodal Merkel cell carcinomas, with comparison to 763 primary cutaneous cases retrieved from the literature. Overall, the nodal and cutaneous Merkel cell carcinomas shared similar clinical presentations, morphologic spectrum, and immunophenotype; both were mostly seen in elderly male with a typical neuroendocrine morphology. Most of cases expressed CK20, synaptophysin, and chromogranin A; and PAX5 and TdT were also positive in majority of cases. However, nodal Merkel cell carcinomas had a significantly lower association with Merkel cell polyomavirus than cutaneous cases (31% vs 76%, P=0.001). Therefore, these two entities may arise from overlapping but not identical biological pathways. We also recommend the use of the diagnostic term 'Merkel cell carcinoma of lymph node' to replace many other names used.Modern Pathology advance online publication, 10 January 2014; doi:10.1038/modpathol.2013.250.
No preview · Article · Jan 2014 · Modern Pathology
[Show abstract][Hide abstract] ABSTRACT: Immunohistochemical expression of napsin A in primary pulmonary mucinous tumors is not well established. Napsin A immunoreactivity was evaluated in 43 mucin-producing adenocarcinomas of the lung consisting of 18 tumors formerly classified as mucinous bronchioloalveolar carcinoma, 15 colloid adenocarcinomas, 5 solid predominant adenocarcinomas with mucin production, and 5 adenocarcinomas with signet ring cell features, as well as in 25 extrapulmonary mucinous adenocarcinomas of different anatomic sites. Immunohistochemical expression of thyroid transcription factor 1 (TTF-1) was also compared. Thirty-three percent of mucinous lung tumors exhibited positive immunoreactivity for napsin A, whereas 42% expressed TTF-1. All 25 extrapulmonary mucinous adenocarcinomas lacked expression of napsin A and TTF-1. Mucin-producing neoplasms of the lung infrequently express napsin A, suggesting that immunohistochemical assessment of napsin A may have limited diagnostic usefulness for distinguishing primary and metastatic mucinous adenocarcinomas involving the lung.
No preview · Article · Feb 2013 · American Journal of Clinical Pathology
[Show abstract][Hide abstract] ABSTRACT: Objective
Bronchobiliary fistula (BBF) is abnormal between the bronchial and biliary tree. Misdiagnosis and delayed treatment may have a high mortality rate. Although some characteristics relevant to BBF have been introduced, difficulties and controversies in diagnosis and treatment need further investigation. MethodologyIn this study, we summarize the diagnostic and surgical treatment experience of a 65-year-old male patient who presented with an irritating cough and biliptysis. Since he had the history of aortic valve replacement surgery and schistosomial cirrhosis, the provisional diagnosis was BBF, which might be secondary to a foreign body reaction or liver cirrhosis and chronic cholecystitis and cholelithiasis. ResultsDuring the surgery, it was discovered that the right lobe of liver was significantly atrophied with irregular contours and the gallbladder was adjacent to the diaphragm with a fistula between the fundus of gallbladder and the right semi-diaphragm. These results confirmed that the BBF in this patient resulted from cholelithiasis associated with serious schistosomial cirrhosis. He was successfully treated with a right hepatic lobe resection and Roux-en-Y hepaticojejunostomy. The diaphragm defect was repaired by imbrication of the repaired liver and lower lobe's edges. ConclusionsA high index of clinical suspicion can be generated by patients who present with recurring irritating cough and production of bile-like sputum. The manifestations and responses to conservative treatment are crucial in clinical judgment and surgical intervention is the primary treatment choice when other approaches have failed or there are complications from the underlying disease.
No preview · Article · Sep 2012 · Surgical Practice
[Show abstract][Hide abstract] ABSTRACT: Cancer of the parotid gland is relatively rare, but carries poor prognosis owing to its prevailing distant metastases. In addition to the disease's basic epidemiology and pathology, we review some current discoveries of its tumorigenesis molecular mechanism. Based on published salivary gland cancer clinical trial data, non-surgical antitumor efficacies amongst a range of chemotherapy, radiation, and concurrent therapy regimens are compared. We also present the current development status of novel radiation therapy and targeted therapeutics, focusing on intensity-modulated radiation therapy (IMRT), and epidermal growth factor receptor (EGFR) and vascular endothelial growth factor (VEGF) blockages, which are showing promise for improving parotid gland cancer management.
[Show abstract][Hide abstract] ABSTRACT: Mucinous adenocarcinomas (MAs) of various origins may have a similar histologic appearance and frequently metastasize to distant sites, which often causes diagnostic problems in surgical pathology practice. The immunohistochemical profiles of MAs of various origins have not been well studied. We investigated the expression of 10 immunohistochemical markers (CK7, CK20, CDX-2, β-catenin, MUC-1, MUC-2, MUC-6, ER, WT-1, and PAX-8) in 175 cases of MA, including 69 cases from the lower gastrointestinal (GI) tract, 41 from the upper GI tract, 27 from gynecologic organs, 4 from the urinary bladder, 18 from the breast, and 16 from the lung. We found that lower GI MAs (colon, rectum, and anus) frequently expressed CDX-2 (42 of 42, 100%; 33 of 42 with homogenous positivity, 79%), MUC-2 (42 of 42; 100%), CK20 (41 of 42; 98%), and β-catenin (nuclear) (27 of 42; 64%) and rarely expressed MUC-6 (2 of 42; 5%) and CK7 (8 of 42; 19%). Most of the CK7-positive cases were from the rectum and anus (7 of 8; 88%). The expression of these markers in appendiceal MAs was similar to that of low GI tract MAs, except for a lower percentage of homogenous CDX-2 (3 of 27; 11%) and nuclear β-catenin (3 of 27; 11%) expression. Unlike their lower GI tract counterparts, the upper GI tract MAs (ampulla, pancreas/biliary tree, and stomach/esophagus) frequently expressed CK7 (38 of 41; 93%) and MUC-6 (31 of 41; 76%) and were rarely homogenously positive for CDX-2 (4 of 41; 10%) and nuclear positive for β-catenin (8 of 41; 19%). Breast MAs were frequently positive for CK7 (18 of 18; 100%), MUC-1 (18 of 18; 100%), MUC-2 (18 of 18; 100%), ER (16 of 18; 89%), MUC-6 (9 of 18; 50%), and WT-1 (9 of 18; 50%). Lung MAs were frequently positive for CK7 (16 of 16; 100%) and MUC-1 (15 of 16; 94%). Gynecologic MAs were positive for CK7 (25 of 27; 93%) and PAX-8 (13 of 27; 48%). We conclude that homogenous CDX-2 and nuclear β-catenin expressions are commonly seen in lower GI tract MAs. In contrast, appendiceal MAs are usually heterogenously positive for CDX-2 and show cytoplasmic positivity for β-catenin. Unlike lower GI tract MAs, upper GI tract MAs are frequently positive for CK7 and MUC-6. As is the case in appendiceal MAs, the upper GI tract MAs may also be heterogenously positive for CDX-2. Breast MAs are positive for ER and WT-1, whereas gynecologic MAs are positive for PAX-8 and negative for WT-1.
No preview · Article · Aug 2011 · The American journal of surgical pathology
[Show abstract][Hide abstract] ABSTRACT: Chronic graft-versus-host disease (cGVHD) is considered an autoimmune-like disease mediated by donor CD4(+) T cells, but the origin of the autoreactive T cells is still controversial. In this article, we report that the transplantation of DBA/2 donor spleen cells into thymectomized MHC-matched allogeneic BALB/c recipients induced autoimmune-like cGVHD, although not in control syngeneic DBA/2 recipients. The donor-type CD4(+) T cells from the former but not the latter recipients induced autoimmune-like manifestations in secondary allogeneic BALB/c as well as syngeneic DBA/2 recipients. Transfer of donor-type CD4(+) T cells from secondary DBA/2 recipients with disease into syngeneic donor-type or allogeneic host-type tertiary recipients propagated autoimmune-like manifestations in both. Furthermore, TCR spectratyping revealed that the clonal expansion of the autoreactive CD4(+) T cells in cGVHD recipients was initiated by an alloimmune response. Finally, hybridoma CD4(+) T clones derived from DBA/2 recipients with disease proliferated similarly in response to stimulation by syngeneic donor-type or allogeneic host-type dendritic cells. These results demonstrate that the autoimmune-like manifestations in cGVHD can be mediated by a population of donor CD4(+) T cells in transplants that simultaneously recognize Ags presented by both donor and host APCs.
Full-text · Article · Jan 2011 · The Journal of Immunology
[Show abstract][Hide abstract] ABSTRACT: We aimed to highlight a new anatomical variation of the recurrent laryngeal nerve (RLN), and to emphasize its implications for thyroid surgery.
A prospective study was carried out in a group of 3,078 consecutive thyroidectomies from 1998 to 2008. Total, near-total, subtotal, and partial thyroidectomy were performed for various thyroid diseases. The RLN was routinely identified and exposed in its entire course until the entry into the larynx. The postoperative complications of patients with different variations were compared.
4,241 RLNs were successfully identified in all patients unilaterally or bilaterally. In addition to extralaryngeal branching and nonrecurrent laryngeal nerves, an unreported variation was identified in 44 RLNs (1.04%) at their entries into the larynx. The variation happened at the trunk or the branches of the RLN entering the larynx far from the posterior of cricothyroid joint, and the entry was higher than the superior cornu of the thyroid cartilage and the arch of the cricoid. The median distance from the entry to the posterior of cricothyroid joint was more than 5 mm. As the trunk or the branches had to travel along the lateral edge of the upper 1/3 of the thyroid before entering the larynx, the incidence of RLN palsy was higher than that in extralaryngeal branching variations (p < .05).
This newly discovered variation of the RLN is more vulnerable to injury and should be brought to the attention of surgeons.
No preview · Article · Dec 2010 · Journal of Investigative Surgery
[Show abstract][Hide abstract] ABSTRACT: Myopericytoma is a rare, histologically distinctive tumor that shows evidence of differentiation toward perivascular myoid cells. Myopericytoma is largely considered a neoplasm of skin and soft tissues, with examples of this lesion involving visceral sites being extremely limited. We present the clinical and pathologic details of an unusual case of myopericytoma occurring in the kidney. Histologically, the tumor was richly vascularized and composed of a perivascular proliferation of oval to spindle-shaped cells with bland cytologic features. The neoplastic cells were arranged in a concentric fashion around vascular lumina and also surrounded dilated, branching vessels, with a glomangiopericytomatous appearance. Mitotic figures were inconspicuous, and necrosis was absent. Perivascular myoid differentiation was supported by positive immunoreactivity for muscle-specific and smooth muscle actins, and absence of reactivity for desmin. The present case serves to expand the anatomical distribution of myopericytoma and also broadens the spectrum of primary mesenchymal neoplasms that may be encountered in the kidney.
[Show abstract][Hide abstract] ABSTRACT: We immunohistochemically studied TCL1 protein expression in different histologic types of 63 testicular germ cell tumors: 23 seminomas, 14 embryonal carcinomas, 4 teratomas, 2 yolk sac tumors, and 20 mixed germ cell tumors. The 20 mixed germ cell tumors contained components of seminoma (n = 10), embryonal carcinoma (n = 18), teratoma (n = 16), yolk sac tumor (n = 7), and choriocarcinoma (n = 3). We also examined 40 cases of intratubular germ cell neoplasia, unclassified type (IGCNU). Positive immunoreactivity for TCL1 was observed in 91% of the seminoma samples (30/33) and all IGCNU cases. In contrast, no TCL1 expression was detected among the nonseminomatous germ cell tumors. In the context of testicular germ cell neoplasia, the presence of TCL1 protein appears restricted to IGCNU and seminoma, suggesting association with an undifferentiated state and loss of protein expression with tumor differentiation. Immunohistochemical evaluation of TCL1 expression may have usefulness in substantiating a diagnosis of IGCNU or seminoma and in the separation of seminoma from nonseminomatous germ cell tumors.
Preview · Article · May 2010 · American Journal of Clinical Pathology
[Show abstract][Hide abstract] ABSTRACT: Adrenal neoplasms composed of more than one cell type and demonstrating a mixed histologic appearance are exceedingly rare. We report the clinical and pathologic features of a morphologically distinctive tumor of the adrenal gland composed of cortical, chromaffin, and neural cells. Histologically, the tumor consisted of intermixed areas of proliferating cortical cells resembling adrenal cortical adenoma, neoplastic chromaffin cells consistent with pheochromocytoma, and a ganglioneuromatous stroma. The presence of the cortical, medullary, and neural components within the tumor was confirmed by immunohistochemical studies. The present case serves to broaden the morphologic spectrum of mixed tumors that may be encountered in the adrenal gland.
No preview · Article · Apr 2010 · Annals of diagnostic pathology
[Show abstract][Hide abstract] ABSTRACT: The presence of a vermiform appendix in an inguinal hernia sac is termed as Amyand's hernia. Although rare, mistakes in diagnosis and treatment can cause catastrophic results. Charts of patients with inguinal hernia were reviewed, and four cases of Amyand's hernia were confirmed. The clinical presentation, anesthetic, and perioperative management of Amyand's hernia were further analyzed. The mean age of patients was over 70 years, and all were males. None of the patients were diagnosed preoperatively. All the patients had little abdominal complaint only with a right inguinal mass and dragging sensation for several hours. Due to the short time after incarceration and significant cardiovascular and pulmonary comorbidities, manual reduction was attempted first in three patients. With complete preoperative evaluation and careful perioperative support, all patients underwent appendectomy and Bassini's hernia repair through a groin incision. Based on age-related organ failure and associated chronic medical illnesses of geriatric patients, the difficulties in the diagnosis and treatment are also summarized and analyzed.
No preview · Article · Dec 2009 · Journal of Investigative Surgery
[Show abstract][Hide abstract] ABSTRACT: Idiopathic sclerosing encapsulating peritonitis (ISEP) is a rare cause of small intestinal obstruction. Histologically, ISEP is characterized by a thick fibrotic membrane encasing the small bowel without any apparent pathophysiological factors. While ISEP is predominantly present congenitally in female adolescents from subtropical region, it has been identified throughout the world. Evidence-based effective diagnostics and treatments are pitifully thin. We experienced six cases, four males and two females, which exhibited symptoms in their later thirties and forties. Five patients presented with acute and subacute intestinal obstruction, and one patient with cryptorchidism and seminoma was referred. Due to the limitation in distention and motility of bowel loops of ISEP, imaging exams may not be very reliable for accurate diagnosis and estimation of obstruction progress. They were successfully treated with the mesenteric plication and intraluminal splinting procedures. Two cases had an uneventful postoperative period, and the returns of normal bowel function were delayed in the other four patients. Two recurrences of small bowel obstructions were noted over a mean follow-up period of 33 months with mild symptoms. This article reviews the patterns of clinical presentations, diagnostic clues, and theories of potential risk factors of ISEP as well as its controversial surgical managements.
No preview · Article · Sep 2009 · Journal of Investigative Surgery
[Show abstract][Hide abstract] ABSTRACT: We report a mucinous borderline tumor arising from a mixed epithelial and stromal tumor of left kidney (MESTK). The patient was an 82-year-old woman who presented with gross hematuria and recurrent urinary tract infection for years. The patient had a cytoscopy with a retrograde pyelogram, which indicated a dilated left kidney with a central mass lesion. Subsequently, she underwent a radical left nephrectomy. Cross-sections of left kidney showed a 4.5 x 3.5 x 1.5 cm ill-defined cystic lesion with mucinous and solid areas. Histologic sections of the lesion showed numerous variable-sized dilated cysts with fibrous, fatty, vascular, and smooth muscle stroma. The cysts were lined by a various types of epithelium, including single layer of flat, cuboidal and mucinous epithelium, urothelium, intestinal epithelium, and endocervical epithelium. In areas, the mucinous epithelium showed complex proliferation with stratification, papillae formation, and nuclear atypia, resembling that of an ovarian mucinous borderline tumor, a colorectal tubular adenoma, or a low-grade appendiceal mucinous carcinoma. Immunohistochemically, the mucinous borderline tumor showed a colorectal phenotype, being strongly positive for CK20, CDX-2, and MUC2. There was no invasive mucinous tumor identified. We believe that this case represents the first reported example of mucinous borderline tumor arising from a MESTK.
No preview · Article · Sep 2009 · Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin
[Show abstract][Hide abstract] ABSTRACT: Dedifferentiated liposarcoma (DDL), occurring in up to 10% of well differentiated liposarcoma cases, has similar histologic features to that of undifferentiated high-grade pleomorphic sarcoma (UHGPS); the former develops in a background of atypical lipomatous tumors/well differentiated liposarcoma, whereas the latter shows no specific line of differentiation. The retroperitoneum and thigh represent the most common anatomic locations for both the sarcomas. Despite their morphologic similarity, the issue of whether these 2 sarcomas share overlapping immunohistochemical and molecular features has not been well studied. We examined the expression of the lipogenic tumor-related markers peroxisome proliferator-activated receptor gamma (PPAR-gamma), CDK4, and MDM2 in 15 cases of DDL and 45 cases of retroperitoneal/thigh UHGPS. Patients with DDL ranged from 31 to 82 years (mean 63 y) with a male:female ratio of 5:3. Patients with UHGPS ranged from 14 to 80 years (mean 52 y) with a male:female ratio of 3:2. All 15 DDLs expressed CDK4 and MDM2 (100%), and 8 of 15 cases expressed PPAR-gamma (53%). Twenty-three of 45 (51%) UHGPS expressed at least 1 of these 3 markers. We also studied MDM2 and CDK4 gene amplification by fluorescence in situ hybridization in 28 immunohistochemically positive cases, including 5 DDLs and 23 UHGPSs. All 5 cases of DDL showed MDM2 and/or CDK4 amplification (100%), whereas 6 of 45 UHGPSs showed MDM2 and/or CDK4 amplification (13%). Our results demonstrate that (1) the lipogenic tumor markers CDK4 and MDM2 can be used as surrogate immunohistochemical markers for the diagnosis of malignant lipomatous tumors with high sensitivity; (2) approximately 26% of retroperitoneal/thigh UHGPS cases that were positive for PPAR-gamma, CDK4, or MDM2 by immunohistochemistry showed characteristic CDK4 and MDM2 gene amplification, suggesting that a subset of UHGPS cases represent DDL despite lacking histologic evidence of lipoblasts.
No preview · Article · Aug 2009 · The American journal of surgical pathology
[Show abstract][Hide abstract] ABSTRACT: Keratins are intermediate filaments that provide mechanical support and fulfill a variety of additional functions in epithelial cells. Keratins show outstanding degree of molecular diversity. In humans, 54 functional keratin genes exist. Twenty common types of keratins are expressed in highly specific patterns related to epithelial type and stage of cellular differentiation. In general, keratins are classified as high-molecular-weight keratins (expressed in normal stratified epithelium and tumors derived from it) and low-molecular-weight keratins (expressed in normal simple epithelium and tumors derived from it). Histologically, endocrine organs belong to simple epithelium; thus, endocrine tissues usually express low-molecular-weight keratins. When an endocrine organ undergoes malignant transformation, its keratin profile usually remains constant. However, keratin expression in endocrine organs and endocrine tumors is much more complicated because of their diversified histogenesis. In this review article, we will first briefly review the molecular biology and protein chemistry of the keratins. We will then review the expression patterns of keratins in normal endocrine tissue and endocrine neoplasms.
No preview · Article · Mar 2009 · Endocrine Pathology
[Show abstract][Hide abstract] ABSTRACT: While focal myxoid areas are occasionally observed in solitary fibrous tumors, neoplasms of this type exhibiting extensive myxoid change are considered exceedingly uncommon. Due to their rarity, the biologic behavior of myxoid solitary fibrous tumor has not been determined. Three cases of myxoid solitary fibrous tumor are described in order to better characterize the clinical and pathologic features of this uncommon variant of solitary fibrous tumor. The tumors occurred in one man and two women, with ages of 37, 47, and 58 years, respectively. Sites of involvement included the retroperitoneum, pelvis, and soft tissue of the neck. Histologically, all cases were characterized predominantly by the presence of myxoid stroma comprising 70% to 100% of the tumor. The tumor cells were predominantly spindled in all cases, and arranged randomly, in loose fascicles, or in anastomosing strands imparting a microcystic/reticular appearance. The lesional cells had a bland cytologic appearance and low mitotic count. All tumors lacked necrosis and areas of increased cellularity. By immunohistochemistry, all cases were positive for CD34, CD99, and bcl-2, and negative for keratin, epithelial membrane antigen, desmin, actin, smooth muscle actin, and S-100 protein. To date, all cases have followed a benign course without evidence of recurrence or metastasis with a follow-up duration ranging from 50 to 87 months. The data suggest that myxoid solitary fibrous tumors are associated with an indolent clinical course and favorable prognosis.
No preview · Article · Feb 2009 · Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin