Zhonghua bing li xue za zhi = Chinese journal of pathology · 2002
[A clinicopathological study of fifteen epithelioid angiosarcoma].
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Liu Mei, Ba Enping, Zhao Po, Li Xianghong
Аннотация
OBJECTIVE: To study the clinicopathological features of epithelioid angiosarcoma (EA) and to propose its differential diagnosis.
METHODS: 15 EAs were observed by means of HE staining, immunohistochemical staining (ABC method) and in part by electron microscopy.
RESULTS: Twelve cases occurred in men and three in women. Clinical symptoms included a focal mass with pain, weight loss and weakness. The tumors were located in the retroperitoneum (n = 3), extremities (n = 3), femur (n = 4), thyroid (n = 2), supraobit (n = 1), soft tissue of thorax (n = 1), soft tissue of neck (n = 1). Radiographic evaluation demonstrated solid to cystic neoplasms ranging from 3 to 20 cm in diameter. Histologically, the epithelioid cells were arranged diffusely or in nests with hemorrhage and focal necrosis. Atypical tumor cells lined the irregular spaces and single or small nests of cells grouped around a lumen containing red blood cells. FVIII, CD34, CD31,CK and EMA were positive in tumor cells and SMA was positive in pericytes. Ultrastructurally, intracytoplasmic vacuoles, Weibel-Palades, intermediate filaments and basal lamina could be seen. Surgical resection was the treatment of choice, occasionally with adjuvant chemotherapy. Follow-up. Two patients are presently alive 19 months and 7 years following diagnosis. Twelve died from 2 weeks to 38 months with a median interval of 19 months after lumpectomy. One patient was lost to follow up. There were 8 cases which had metastases to lungs, bones, lymph nodes and abdominal cavity.
CONCLUSIONS: EA is a high-grade sarcoma. Endothelial derivation can be confirmed by immunohistochemical staining and ultrastructural findings. The differential diagnosis of EA includes metastatic carcinoma, epithelioid hemangioendothelioma, melanoma, epithelioid sarcoma etc.
METHODS: 15 EAs were observed by means of HE staining, immunohistochemical staining (ABC method) and in part by electron microscopy.
RESULTS: Twelve cases occurred in men and three in women. Clinical symptoms included a focal mass with pain, weight loss and weakness. The tumors were located in the retroperitoneum (n = 3), extremities (n = 3), femur (n = 4), thyroid (n = 2), supraobit (n = 1), soft tissue of thorax (n = 1), soft tissue of neck (n = 1). Radiographic evaluation demonstrated solid to cystic neoplasms ranging from 3 to 20 cm in diameter. Histologically, the epithelioid cells were arranged diffusely or in nests with hemorrhage and focal necrosis. Atypical tumor cells lined the irregular spaces and single or small nests of cells grouped around a lumen containing red blood cells. FVIII, CD34, CD31,CK and EMA were positive in tumor cells and SMA was positive in pericytes. Ultrastructurally, intracytoplasmic vacuoles, Weibel-Palades, intermediate filaments and basal lamina could be seen. Surgical resection was the treatment of choice, occasionally with adjuvant chemotherapy. Follow-up. Two patients are presently alive 19 months and 7 years following diagnosis. Twelve died from 2 weeks to 38 months with a median interval of 19 months after lumpectomy. One patient was lost to follow up. There were 8 cases which had metastases to lungs, bones, lymph nodes and abdominal cavity.
CONCLUSIONS: EA is a high-grade sarcoma. Endothelial derivation can be confirmed by immunohistochemical staining and ultrastructural findings. The differential diagnosis of EA includes metastatic carcinoma, epithelioid hemangioendothelioma, melanoma, epithelioid sarcoma etc.