Clinicopathologic study of intraabdominal extranodal follicular dendritic cell sarcoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and immunophenotype of intraabdominal extranodal follicular dendritic cell sarcoma (FDCS) and the relationship with Epstein-Barr virus (EBV).</p><p><b>METHODS</b>The clinical and histologic features of 4 cases of FDCS were evaluated. Immunohistochemical study was performed using standard EnVision method for CD21, CD23, CD35, S-100 protein, CD68, HLA-DR, vimentin, epithelial membrane antigen, desmin, CD34 and CD117. In-situ hybridization for EBV-encoded RNA (EBER) was carried out in 2 cases.</p><p><b>RESULTS</b>The age of patients ranged from 28 to 63 years (mean=42 years). The male-to-female ratio was 3:1. The clinical presentation was abdominal discomfort, pain or mass. Radiologic examination revealed concurrent lesions in stomach and left lobe of liver in 1 patient, while non-specific intraabdominal masses were detected in the remaining cases (in which the tumor was later found to be located in the appendix, mesentery of jejunum and omentum). Two cases were misdiagnosed as gastrointestinal stromal tumor before operation. Grossly, the tumors appeared as large solid nodules, with a mean diameter of 10.8 cm. Three of the cases showed areas of necrosis. Histologically, there were plump spindle, ovoid to epithelioid cells associated with scattered multinucleated giant cells. The tumor cells were arranged mostly in storiform pattern, whorls, fascicles or solid sheets. Lymphocytic infiltrates with perivascular cuffing were noted in all cases, resulting in a distinctive biphasic pattern. Two tumors showed significant cytologic atypia, with mitotic figures (including atypical mitotic figures) readily demonstrated. The remaining case (occurring in liver) was composed of scattered large atypical cells embedded in a dense inflammatory background, mimicking inflammatory pseudotumor. Immunohistochemical study showed that all cases were positive for CD21, CD23 and vimentin. There was focal expression of CD35, S-100 protein, CD68, HLA-DR and epithelial membrane antigen. The staining for CD34 and CD117 was negative. In-situ hybridization for EBER was negative in 2 cases tested.</p><p><b>CONCLUSIONS</b>Intraabdominal extranodal FDCS is extremely rare. Familiarity with its characteristic histologic features and immunophenotype is important in distinguishing the tumor from other intraabdominal spindle cell lesions (such as gastrointestinal stromal tumor). Hepatic FDCS may show inflammatory pseudotumor-like features, resulting in misinterpretation. Non-hepatic intraabdominal FDCS seems to have little association with EBV infection.</p>

A clinicopathological study of perianal Paget's disease associated with internal rectal adenocarcinoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathological features and the immunohistochemical phenotype of perianal Paget's disease (PPD) associated with internal anorectal adenocarcinoma, with emphasis on the histogenesis of Paget's cells.</p><p><b>METHODS</b>The clinical and pathologic features of three cases of PPD with rectal adenocarcinoma were investigated. Periodic-acid-Schiff (PAS), alcian-blue and mucicarmine staining with and without diastase digestion were performed. The immunohistochemical study was performed on selected sections by a panel of antibodies including carcinoembryonic antigen (CEA), CK7, CK8, CK10/13, CK20 and gross cystic disease fluid protein 15 (GCDFP15).</p><p><b>RESULTS</b>All three cases occurred in middle to old age male patients complaining of anal bleeding. Digital physical examination revealed ulcerated or cauliflower-like masses in the anus just distal to the dentate line. Perianal skin erythematous patches were found in two cases, and small discrete granules in one case. Histologically, the anorectal neoplasm was either a moderately or poorly differentiated adenocarcinoma. Two types of Paget's cells were noted, namely the classical type characterized by a polygonal shape with vesicular nuclei and abundant pale cytoplasm, and the signet ring type characterized by eccentrically displaced nucleus. Both the rectal adenocarcinoma cells and Paget's cells showed strong positivity for PAS, AB and mucicarmine, which were resistant to the diastase digestion. Immunohistochemically, they were both positive for CEA, CK7, CK8 and CK20, but negative for CK10/13 and GCDFP15.</p><p><b>CONCLUSIONS</b>The CK20(+)-GCDFP15(-) type Paget's cells in PPD were derived from the direct intraepithelial Pagetoid spread of anorectal adenocarcinomas. PPD was more frequently associated with internal carcinomas than any other type of extramammary Paget's disease. It is recommended that clinicians should carefully examine the anus or rectum in the presence of PPD to ascertain if it is associated with an internal carcinoma.</p>

Twenty-four cases of carcinoma in pleomorphic adenoma in the salivary gland / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To study the clinical characteristics, treatment and prognosis 24 cases of carcinoma in pleomorphic adenoma in salivary gland.</p><p><b>METHODS</b>The clinical data of 24 patients with carcinoma in pleomorphic adenoma treated in our hospital from September 1974 to July 1995 were analyzed.</p><p><b>RESULTS</b>The overall 5-year survival rate was 66.7%. The five-year survival rates of patients with carcinoma in pleomorphic adenoma in the major and minor salivary glands were 63.6% and 2/2, respectively.</p><p><b>CONCLUSION</b>Operation is the optimal treatment and extensive resection at the initial operation is suggested. For lumps in the submaxillary gland, preventive neck dissection should be considered. Postoperative radiotherapy can not improve the local-control rate.</p>