- 期刊
Abdominopelvic Perivascular Epithelioid Cell Tumor with Overt Malignancy: A Case Report
腹腔骨盆腔惡性血管旁類上皮細胞瘤－病例報告
摘要
血管旁類上皮細胞瘤(perivascular epithelioid cell tumor, PEComa)包含一群由類上皮細胞所構成的罕見疾病。這些類上皮細胞的特徵是會圍繞著血管分布，並且可以同時表現出具黑色素分泌功能的標記，以及肌肉組識的標記。到目前為止，並沒有指標可以來預測血管旁類上皮細胞瘤的生物行為。 我們提出了一個發生在16歲年輕女孩身上，具有明顯惡性行為的血管旁類上皮細胞瘤病例報告。在組識學上，這個腫瘤具有典型的血管旁類上皮細胞瘤之特徵。雖然構成這個腫瘤的腫瘤細胞型態看起來相當的良性，但大範圍的壞死、合併淋巴結的轉移、以及侵犯到鄰近的組識等現象都再再地顯示出這是一個惡性腫瘤。在做完減積手術及術後輔助性化學治療後沒多久，腫瘤便又復發了，且伴隨多處的淋巴腺轉移，更進一步證實了此一腫瘤的具侵襲性行為。
並列摘要
Perivascular epithelioid cell tumor (PEComa) is a group of rare tumors composed of epithelioid cells with characteristic perivascular distribution and co-expression of the melanogenic marker HMB-45 and muscular markers. There are no documented parameters referring to the biologic behavior of PEComa. We report an abdominopelvic PEComa with overt malignancy in a 16-year-old girl. Histologically, the tumor showed the typical morphophenotypic characteristics of PEComa. Though the cytologic appearance of the tumor cells was relatively bland, the extensive necrosis, presence of lymph node metastases, and surrounding tissue invasion were all indicative of malignancy. Relapse of the tumor with multiple lymphadenopathy shortly after debulking surgery for the primary lesion, and postoperative adjuvant chemotherapy, further denoted its aggressive behavior.
延伸閱讀
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