Chen I-Yu, Yang Sheau-Fang, Chen Fang-Ming, Chai Chee-Yin
Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.
Kaohsiung J Med Sci. 2005 Jun;21(6):277-81. doi: 10.1016/S1607-551X(09)70201-3.
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.
血管周上皮样细胞肿瘤（PEComa）是一组罕见肿瘤，由具有特征性血管周分布的上皮样细胞组成，并共同表达黑素生成标志物HMB-45和肌肉标志物。目前尚无关于PEComa生物学行为的文献记载参数。我们报告一例16岁女孩发生的具有明显恶性特征的腹盆腔PEComa。组织学上，肿瘤表现出PEComa典型的形态表型特征。尽管肿瘤细胞的细胞学表现相对温和，但广泛坏死、存在淋巴结转移以及周围组织浸润均提示为恶性。在对原发性病变进行减瘤手术后不久，肿瘤复发并伴有多处淋巴结病，以及术后辅助化疗，进一步表明其侵袭性生物学行为。
