- 期刊
Retroperitoneal Malignant Neoplasm with Perivascular Epithelioid Differentiation: Report of a Case
後腹腔惡性腫瘤（具有血管旁類上皮細胞分化）：病例報告
摘要
一名83歲的台灣男性被發現有一個巨大的後腹腔腫瘤，接受手術切除。組織學檢查發現此腫瘤是由兩種的細胞排列而成，包括類上皮細胞及梭形細胞且主要是沿著血管周邊排列；但其他不好的組織學特點，包括核的多形性，腫瘤細胞壞死，非典型細胞分裂和增加的細胞分裂數目也被發現到。此腫瘤細胞會同時表現肌源性及和黑色素細胞的標記。另外我們也發現腫瘤細胞具有細胞質的CD1a染色。因此最後診斷爲惡性腫瘤表現血管旁類上皮細胞分化（即惡性血管旁類上皮細胞分化腫瘤）。手術後，病人一直在定期血液透析。之後懷疑有局部復發和肝轉移的現象。術後6個月，病人因末期腎病死於急性肺水腫。
關鍵字
無資料並列摘要
An 83-year-old man presented with a huge retroperitoneal mass and underwent surgical excision. Histological examination revealed a biphasic growth pattern of epithelioid and spindle tumor cells predominantly arranged in the perivascular region; other worrisome features including nuclear pleomorphism, geographic necrosis, atypical mitoses and increased mitoses were also noted. The tumor cells were positive for both muscular (desmin and smooth muscle actin) and melanocytic (HMB-45 and Melan-A) markers. CD1a expression in the cytoplasm was also seen. A malignant tumor with perivascular epithelioid differentiation (malignant perivascular epithelioid cell tumor, malignant PEComa) was diagnosed. After operation, the patient was kept on regular hemodialysis. Suspected local recurrence and liver metastasis were also noted, and he later died of acute pulmonary edema from end stage renal disease (ESRD) 6 months after the operation.
延伸閱讀
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