Composite Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia and Follicular Lymphoma: A Clinicopathological Study of Six Cases.

BACKGROUND: Composite small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL) and follicular lymphoma (FL) is extremely rare, and only 13 cases have been reported previously. METHODS: We identified 6 cases of composite SLL/CLL and FL in our database and studied their clinical, histologic, immunophenotypic, and cytogenetic features. A literature review of the existing cases was also conducted. RESULTS: The patients included 4 males and 2 females, with a median age of 72 years. Four patients presented with lymphadenopathy and 2 with extranodal diseases. Lymphocytosis was seen in 2 cases. Serum lactate dehydrogenase levels were within normal range in all but one case. There were 2 histologic patterns: SLL/CLL predominant pattern (type I) and FL predominant or mixed pattern (type II). The type I pattern was exclusively associated with in situ follicular neoplasia (ISFN). The SLL/CLL showed typical morphology and immunophenotype in all the cases. The FL component included low grade (n = 3), ISFN (n = 2), and primary cutaneous FL (n = 1). Four cases had staging bone marrow biopsies including 3 cases with involvement by SLL/CLL and 1 case with involvement by SLL/CLL and FL. Four patients received treatments, one was under clinical surveillance, and one had no available information. All patients were alive after a median follow-up of 22 months. CONCLUSIONS: This is the largest case serial of composite SLL/CLL and FL. The CL affects elderly individuals, presents with advanced clinical stage, and appears to have a relatively indolent clinical course.

Central nervous system lymphoma in immunocompetent patients: the North Shore-Long Island Jewish Health System experience.

Of the 74 immunocompetent patients diagnosed between July 2004 and June 2011 at the North Shore University Hospital and Long Island Jewish Medical Center with primary central nervous system lymphoma, 71 (95.9%) had diffuse large B-cell lymphomas (DLBCL). The median patient age was 68 years (range: 19-87 years) with a slight male preponderance (1.1:1). The overall median survival time was 21 months. For patients older than 70 years, the median survival time was 8 months while for those 70 years or younger, the median survival time was 27 months (p<0.01). Female patients had a worse prognosis than male patients (p<0.05, median survival time, 17 months compared to 23 months). We had enough data from 52 of these 71 patients to define the lymphomas as either germinal center B-cell-like (GCB) or activated B-cell-like (ABC) DLBCL. Of these 52 patients, 42 (80.8%) had ABC DLBCL while only 10 (19.2%) had GCB DLBCL. The patients in the GCB subgroup seemed to survive longer than the patients in the ABC subgroup, although the difference did not reach statistical significance. No statistically significant difference in overall survival was seen between patients with BCL-6 positive or negative DLBCL; or between patients with BCL-2 positive or negative DLBCL.

Sarcomatoid renal cell carcinoma with divergent sarcomatoid growth patterns: a case report and review of the literature.

A 71-year-old woman who presented with left abdominal pain was found to have a noncalcified renal mass with a perisplenic extension on imaging studies. Histologically, the tumor showed predominantly malignant spindle cells with extensive osteoid and chondroid matrix production. Various growth patterns resembling rhabdomyosarcoma, malignant fibrous histiocytoma, and fibrosarcoma were also observed. Immunohistochemistry showed positive staining of the neoplastic cells for cytokeratin and focally positive staining for CD10 and CD117 (c-Kit). Electron microscopic examination revealed a poorly differentiated neoplasm with both mesenchymal and epithelial features. The tumor was diagnosed as a sarcomatoid renal cell carcinoma with overgrowth of the sarcomatoid component (World Health Organization: renal cell carcinoma, unclassified). To our knowledge, sarcomatoid renal cell carcinoma with such a broad morphologic phenotype in a single case has not been documented. Furthermore, the CD117 expression in a sarcomatoid renal cell carcinoma that was observed in this case merits further investigation.

Signet-ring cell carcinoma of the ampulla of Vater.

Signet-ring cell carcinoma (SRCC) of the alimentary canal is a variant of adenocarcinoma that has a poor clinical prognosis. SRCC of the ampulla of Vater is extremely uncommon with < 12 cases reported in the literature. SRCC of the ampulla of Vater occurs in an older age group (mean age 60 yr) when compared to SRCC of the stomach (under 45 yr), but is similar to SRCC of the large intestine. We report a case of SRCC of the ampulla of Vater with unusual histopathologic features in a 56-yr-old woman who presented with a small tumor at the orifice of the ampulla, associated with extensive lymphovascular invasion and multiple regional lymph node involvement.