Chang Hyeyoon, Jung Wonkyung, Kang Youngran, Jung Woon Yong
Department of Pathology, Korea University Guro Hospital, Korea University College of Medicine, Seoul, Korea.
Korean J Pathol. 2012 Oct;46(5):499-502. doi: 10.4132/KoreanJPathol.2012.46.5.499. Epub 2012 Oct 25.
Heavily pigmented perivascular epithelioid cell tumors (PEComa) are rare, only eight cases of which have been reported. Unlike typical epithelioid angiomyolipoma, most of these tumors have been encountered in female patients without tuberous sclerosis. The long-term prognosis thereof is undetermined. Cytological similarity and heavy melanin pigment make it difficult for pigmented PEComa to be differentiated from pigmented clear cell renal cell carcinoma or malignant melanoma. The immunoprofile of tumor cells, such as human melanoma black-45 expression, as well as the absence or presence of other melanocytic or epithelial markers, are helpful in determining a differential diagnosis. Here we report a case of heavily pigmented PEComa of the right kidney and review the literature describing this tumor. In this case, the immunoprofile and clinical features corresponded well to those described in the literature. Since the prognosis of such disease has not yet been established, close follow-up of this patient was recommended.
重度色素沉着的血管周围上皮样细胞瘤（PEComa）很罕见，仅报道过8例。与典型的上皮样血管平滑肌脂肪瘤不同，这些肿瘤大多见于无结节性硬化症的女性患者。其长期预后尚不确定。细胞学上的相似性和大量黑色素沉着使得色素沉着的PEComa难以与色素沉着的透明细胞肾细胞癌或恶性黑色素瘤相鉴别。肿瘤细胞的免疫表型，如人黑色素瘤黑色45表达，以及其他黑素细胞或上皮标志物的有无，有助于进行鉴别诊断。在此，我们报告1例右肾重度色素沉着的PEComa病例，并复习描述该肿瘤的文献。在本病例中，免疫表型和临床特征与文献报道相符。由于此类疾病的预后尚未明确，建议对该患者进行密切随访。
