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嗜酸性实性和囊性肾细胞癌:一种罕见的、未被充分认识的惰性实体。

Eosinophilic solid and cystic renal cell carcinoma: A rare under-recognized indolent entity.

作者信息

Kamboj Meenakshi, Gupta Gurudutt, Pasricha Sunil, Rawal Sudhir, Sharma Anila, Durga Garima, Mehta Anurag

机构信息

Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India.

Department of GenitoUro-Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India.

出版信息

Indian J Pathol Microbiol. 2021 Oct-Dec;64(4):799-801. doi: 10.4103/IJPM.IJPM_938_20.

Abstract

Eosinophilic solid and cystic renal cell carcinoma (ESCRCC) is an under-recognized, emerging new entity of sporadic renal neoplasms, with an approximate incidence of 0.2% of renal tumors. A total of 60 cases have been reported in the literature till date. ESCRCC are usually seen in adult females, with a low stage and indolent behavior, and rare incidence of recurrence or metastasis. They are solid and cystic tumors with variably sized cysts resembling eosinophilic RCC, showing a characteristic positive immune-expression for PAX-8, CK20 (in ~80% cases) and/or Melan-A (in ~6.7%), with negative CK7 and CA-IX expression. They consistently harbor TSC1 or TSC2 mutations in all tumors, which is a proposed molecular marker for this entity. We here present the first reported case of this rare tumor from India. The tumor was positive for PAX-8, and showed diffuse strong positivity for Melan-A, while was negative for CK7 and CK20. It was an early-stage tumor (T1), managed with partial nephrectomy, with no evidence of any recurrence/metastasis after 1 year of follow-up.

摘要

嗜酸性实性和囊性肾细胞癌(ESCRCC)是一种未被充分认识的、新出现的散发性肾肿瘤实体,约占肾肿瘤的0.2%。迄今为止,文献中总共报道了60例。ESCRCC通常见于成年女性,分期低,行为惰性,复发或转移发生率低。它们是实性和囊性肿瘤,有大小不一的囊肿,类似嗜酸性肾细胞癌,对PAX-8、CK20(约80%的病例)和/或Melan-A(约6.7%)呈特征性阳性免疫表达,CK7和CA-IX表达阴性。所有肿瘤均一致存在TSC1或TSC2突变,这是该实体的一个拟议分子标志物。我们在此报告印度首例该罕见肿瘤病例。肿瘤PAX-8阳性,Melan-A呈弥漫性强阳性,而CK7和CK20阴性。这是一例早期肿瘤(T1),行部分肾切除术治疗,随访1年后无任何复发/转移迹象。

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