Di Blasi A, Ferrara G, Gogglia P
Dipartimento di Oncologia, U.O. di Anatomia Patologica, Azienda Ospedaliera G. Rummo, Benevento.
Pathologica. 2003 Apr;95(2):119-22.
The Authors report a case of Renal Capsuloma with a histologic biphasic pattern constituted of epithelioid and spindle cells. The lesion is observed in a nephrectomy specimen as incidental finding concurrent with a Papillary Transitional Cell Carcinoma of calices and renal pelvis. The lesion presents a distinct muscular immunophenotype (actin+, desmin+), with coexpression of the melanogenesis marker (HMB45+) in a lower cellular population. This combined immunophenotype is a characteristic feature of the Perivascular Epithelioid Cell's (PEC) lesions. Actually Renal Capsuloma is a PEC's lesion (Pecoma) closely related to Angiomyolipoma (AML). Particularly Capsuloma is thought as a variant of AML predominantly composed by muscular spindle cells (monotypic spindle cell-leiomyoma like variant of AML). In the presented case the spindle cell component is associated with an epithelioid cellular population which also shows a distinct muscular differentiation. The Authors have not found in the literature the association between Renal Capsuloma and Papillary Transitional Cell Carcinoma of the renal pelvis.
作者报告了一例具有上皮样细胞和梭形细胞构成的组织学双相模式的肾包膜瘤病例。该病变在肾切除标本中作为偶然发现，与肾盂和肾盏的乳头状移行细胞癌同时存在。该病变呈现出独特的肌肉免疫表型（肌动蛋白阳性、结蛋白阳性），在较低细胞群体中黑色素生成标志物（HMB45阳性）共表达。这种联合免疫表型是血管周上皮样细胞（PEC）病变的特征性表现。实际上，肾包膜瘤是一种与血管平滑肌脂肪瘤（AML）密切相关的PEC病变（PEComa）。特别是，包膜瘤被认为是AML的一种变体，主要由肌肉梭形细胞组成（AML的单型梭形细胞 - 平滑肌瘤样变体）。在本病例中，梭形细胞成分与上皮样细胞群体相关，上皮样细胞群体也显示出明显的肌肉分化。作者在文献中未发现肾包膜瘤与肾盂乳头状移行细胞癌之间的关联。
