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Angiomatoid Malignant Fibrous Histiocytoma -A Case Report-

血管瘤樣惡性纖維組織細胞瘤─病例報告─

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摘要


血管瘤樣惡性纖維組織細胞瘤(AMFH)是一種罕見的低度至中度惡性腫瘤,好發於年輕人的四肢。一位二十三歲女性近一年來左手掌有一慢慢長大、具壓痛感、帶紫色、堅實的丘疹。組織病理檢查可見AMFH典型特徵,即:(1)由類纖維母細胞及類組織細胞所組成的不規則質塊及結節,(2)局部出血性囊腔及(3)以淋巴球及漿細胞為主的慢性發火細胞浸潤,形成類似淋巴結構造。局部腫瘤細胞CD68免疫染色呈陽性,顯示細胞來源可能是組織細胞。病灶處於是進行廣泛切除,往下切至肌膜下層,並保留安全邊緣1公分,皮膚缺陷覆以腹股溝自由皮瓣。我們報告此病例,並回顧相關文獻。文中並比較與惡性纖維組織細胞瘤的差異。

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並列摘要


Angiomatoid malignant fibrous histiocytoma (AMFH) is a rare, low-to-intermediate-grade malignant tumor that occurs primarily in the extremities of young individuals. A 23-year-old woman had a slow-growing violaceous tender firm papule at left palm for 1year. Histopathology showed characteristic findings of AMFH, namely (1) irregular solid masses and nodules of fibroblast-and histiocyte-loke cells, (2) focal areas of hemorrhagic cysts and (3) chronic inflammatory infiltrate consisted mainly of lymphocytes and plasma cells, forming lymphoid follicles. The tumor cells were focally positive for CD68 immunostaining, suggesting possible histiocytic origin. Wide excision deep to the subfascial layer and with a safety margin of 1cm was performed, and the skin defect was covered with a free groin flap. We present the case and review the literature. Differences to malignant fibrous histiocytoma are also compared.

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