Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature.

AIMS: To study the clinicopathologic features of Stewart-Treves syndrome (STS) in postmastectomy patients including the epidemiology, presentation, morphology, differentiation, pathogenesis and therapeutic options. METHODS AND RESULTS: Ten cases of STS in postmastectomy patients were retrospectively identified in our archives, and immunohistochemistry for CD34, CD31, D2-40, HHV-8, CK, EMA and Ki-67 was performed. All ten patients presented with lymphedema after mastectomy as the first sign. Physical examination revealed multiple raised, pinkish-red papulo-vesicular lesions or ulceration as the early evidence of tumor in the field where radiation therapy was introduced. Microscopic examination revealed infiltrative proliferation of vessels and the heteromorphic tumor cells expressed CD34, CD31 and D2-40. Despite the various treatment modalities, 5 patients died in an average of 19 months, 4 patients survived to the last follow-up (9-31 months), and 1 patient got lost. CONCLUSIONS: STS is a fatal complication of postmastectomy lymphedema. Patients with STS have very poor prognosis. The key to improve patient's survival is the early diagnosis through a high alert of this disease by primary care physicians and comprehensive physical examination of patients with pertinent history and suspicious clinical presentations followed by prompt biopsy for definitive diagnosis.

Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases.

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.