Composite hemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma.

Eight cases of a previously uncharacterized vascular neoplasm, showing varying combinations of benign, low-grade malignant, and malignant vascular components are described. Seven tumors occurred in the dermis and/or subcutis and one occurred in the oral submucosa. The patients were all adults with a median age of 39.5 years (range, 21-71 years). Five patients were men. The tumors arose predominantly in the hands and feet, and the lesions were usually of several years duration. The tumors were composed of a complex admixture of histologic components that varied from tumor to tumor, such that no two tumors looked precisely the same. This was due to variation in the proportions of each component as well as the manner in which each component was distributed throughout each lesion. The predominant histologic components were epithelioid hemangioendothelioma (HE) and retiform HE, which were each present in seven of the tumors. Areas of spindle cell HE were identified in four lesions. Angiosarcoma-like elements were identified in seven tumors. One of the tumors was associated with an arteriovenous malformation and one was associated with an area of lymphangioma circumscriptum. Of six cases with follow up (median duration, 6.5 years), three have recurred locally and, to date, only one has metastasized. We think composite HE is best regarded as a low-grade malignant vascular neoplasm, and the available data suggest that it behaves more favorably than conventional angiosarcoma. The existence of these composite lesions has led to careful reexamination of the concept of HE. The term HE, in that it is currently synonymous with a low-grade malignant vascular tumor, should be reserved for lesions that have true metastatic potential, albeit with low frequency.

Advanced prostate cancer with normal serum prostate-specific antigen values.

Prostate-specific antigen is a specific and sensitive marker of prostate cancer, and most patients with advanced prostate cancer have elevated serum prostate-specific antigen values. Over a period of 45 months, 976 cases of advanced prostate cancer (stages C and D) were investigated to determine the serum prostate-specific antigen level at presentation. Eight cases of advanced prostate cancer are documented in which the serum prostate-specific antigen values were within normal limits. Six of the eight cases showed the features of invasive moderately differentiated acinar carcinoma; one case of small-cell carcinoma and one of cribriform carcinoma were noted. Direct immunohistochemical assessment of tumor tissue was performed, and the correlation between monoclonal and polyclonal antibody staining was assessed. In seven of the eight cases, monoclonal antibodies showed no convincing staining, while all but one (small cell) were positive for polyclonal stains.

Cytopathology of follicular dendritic cell tumors.

Follicular dendritic cell tumors are rarely described entities, arising from antigen-presenting immune accessory cells, found within B-lymphocyte follicles in nodal and extranodal sites. We report two cases, one associated with Castleman's disease, in whom fine-needle aspiration biopsy was performed followed by surgical biopsy. The diagnosis was confirmed using immunoreactivity with CD21 and CD35 antibodies and by ultrastructural demonstration of interdigitating cell processes with desmosomes. Both the cytologic and histologic findings are presented, and the differential diagnoses are discussed. Awareness of this entity and recognition of the pathologic features may lead to a presumptive diagnosis which must be confirmed using imunohistochemistry and/or electron microscopy.

Synovial sarcoma of the parotid gland. A case report with clinicopathological analysis and review of the literature.

Primary sarcomas of the major salivary glands are exceptionally uncommon. Synovial sarcoma is a distinctive neoplasm which usually arises in the extremities in the region of joints. Although synovial sarcomas arising in the head and neck region are well described, tumours actually originating in and around the major salivary glands are exceedingly rare, with few cases reported in the literature. We report a synovial sarcoma in the right parotid gland of a 67-year-old man. The literature pertaining to salivary gland sarcomas and synovial sarcoma of the head and neck region is reviewed.

HHV-8 is not associated with follicular dendritic cell tumours.

Follicular dendritic cell tumours are rare malignancies derived from the follicular dendritic cells of lymphoid follicles. These tumours have been associated with Epstein-Barr virus infections and with the hyaline vascular subtype of Castleman's disease. Because many examples of Castleman's disease have been associated with Kaposi's sarcoma associated herpes virus (HHV-8), this study uses polymerase chain reaction technology to examine five cases of follicular dendritic cell tumours for HHV-8. One of these cases had previously been documented to arise from pre-existing Castleman's disease. HHV-8 DNA was not detected in any of the follicular dendritic cell tumours examined, or in the original case of Castleman's disease. These findings suggest that HHV-8 plays no role in the aetiology of follicular dendritic cell tumours and the cause of this tumour remains obscure.

HHV-8 (KSHV) is not associated with bacillary angiomatosis.

AIMS: Bacillary angiomatosis is a rare pseudoneoplastic angioproliferative lesion occurring in patients with AIDS. This condition has been associated with Bartonella henselae and Bartonella quintana infections. Human herpesvirus 8 (HHV-8) is thought to be the causative agent of Kaposi's sarcoma, a vasoproliferative neoplasm, also commonly found in patients with AIDS. The presence of HHV-8 in a cohort of patients with bacillary angiomatosis was investigated. METHODS: Eight cutaneous cases of biopsy confirmed bacillary angiomatosis were assessed for HHV-8 using standard solution phase polymerase chain reaction (PCR). RESULTS: No case of bacillary angiomatosis harboured HHV-8 DNA. CONCLUSIONS: HHV-8 was not demonstrated in the lesions of bacillary angiomatosis and therefore does not appear to play a role in the pathogenesis of this pseudoneoplastic angioproliferative disorder. This finding might be useful in the distinction of bacillary angiomatosis from Kaposi's sarcoma, because lesions from patients with Kaposi's sarcoma almost always contains HHV-8 DNA.

Follicular dendritic cell sarcoma. Clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized.

BACKGROUND: The goal of this study was to characterize the clinicopathologic features of follicular dendritic cell sarcoma, a very uncommon neoplasm. METHODS: The 17 cases were collected from the consultation and surgical pathology files of the authors, including 8 previously reported cases. The histologic and immunohistochemical features and outcome were analyzed. RESULTS: The patients had a median age of 40 years, with a slight female predominance. Seven patients presented with enlarged lymph nodes, and ten presented with tumor in extranodal sites. Two cases were associated with hyaline-vascular Castleman's disease. The tumors had an average greatest dimension of 6.7 cm. The most common histologic feature was a storiform or fascicular array of spindle, ovoid, or polygonal cells with oval nuclei, delicate nuclear membrane, vesicular or granular chromatin, distinct nucleoli, indistinct cell borders, and frequently fibrillary cytoplasm. There were often scattered multinucleated forms. The tumor cells sometimes formed sheets, circular whorls, follicle-like structures, trabeculae, or pseudovascular spaces. There was a sprinkling of small lymphocytes, with or without cuffing around blood vessels. The neoplastic cells were immunoreactive for CD21 (17 of 17 cases), CD35 (17 of 17 cases), desmoplakin (10 of 17 cases), epithelial membrane antigen (14 of 16 cases), S-100 protein (6 of 17 cases), and CD68 (2 of 17 cases), but not cytokeratin. Ultrastructural studies showed villous processes connected by desmosomes. Only one harbored the Epstein-Barr virus. Among 13 patients with a median follow-up of 3 years, local recurrence occurred in 6, metastasis in 6, and 3 died of disease. CONCLUSIONS: Follicular dendritic cell sarcoma exhibits distinctive histologic features that permit its presumptive recognition, but a firm diagnosis requires confirmation with special studies. Because it has a significant recurrent and metastatic potential (the latter risk having been previously underestimated), it should be viewed as an intermediate grade malignancy. An intraabdominal location is associated with a particularly aggressive clinical course.

Von Recklinghausen's disease associated with a primary malignant schwannoma of the breast.

A case of a primary malignant schwannoma of the breast is reported. This case and the review of the literature illustrate the problems of diagnosing and treating this rare malignancy.