Sakhadeo Uma, Yadav Subhash C, Bakshi Ganesh Kailsas, Prakash Gagan, Katdare Aparna, Menon Santosh, Desai Sangeeta B
Department of Surgical Pathology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India.
Department of Surgical Oncology, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, Maharashtra, India.
Indian J Urol. 2021 Oct-Dec;37(4):350-354. doi: 10.4103/iju.iju_217_21. Epub 2021 Oct 1.
Eosinophilic solid cystic renal cell carcinoma (ESC-RCC) is a recently described entity, which demonstrates distinct clinical, pathological and molecular features. We present a series of three cases, the first to be reported from the Indian subcontinent. All three patients were over 50 years of age; and presented with a large kidney mass. One patient had a locally advanced disease while the other two presented with metastases. Microscopic examination revealed a tumor displaying solid-cystic and/or papillary areas composed of clear as well as eosinophilic cells in all three cases. On immunohistochemistry, all the three cases showed a unique CK20+/α-methyl-acyl-CoA-racemase + immunophenotype. Melan-A was focally positive in Case 2. Cytokeratin 7 was focally but strongly positive in Case 3. The two patients with metastatic disease were diagnosed on core biopsies and were advised oral tyrosine kinase inhibitor therapy. The third patient underwent upfront radical nephrectomy. Due to its peculiar morphology and immunoprofile, the diagnosis of ESC-RCC can be confidently made even on a core biopsy. Most cases reported till date had an indolent course. The metastatic presentation in two of our patients emphasizes the need to gather further evidence to ascertain the biological behavior of this emerging entity.
嗜酸性实性囊性肾细胞癌(ESC-RCC)是一种最近才被描述的实体瘤,具有独特的临床、病理和分子特征。我们报告了三例该疾病,这是印度次大陆首次报道的病例。所有三名患者均超过50岁,均表现为较大的肾脏肿块。一名患者为局部晚期疾病,另外两名患者出现转移。显微镜检查显示,所有三例肿瘤均表现为实性囊性和/或乳头状区域,由透明细胞和嗜酸性细胞组成。免疫组化显示,所有三例均表现出独特的CK20+/α-甲基酰基辅酶A消旋酶+免疫表型。病例2中Melan-A呈局灶性阳性。病例3中细胞角蛋白7呈局灶性但强阳性。两名转移性疾病患者通过芯针活检确诊,并接受口服酪氨酸激酶抑制剂治疗。第三名患者接受了一期根治性肾切除术。由于其独特的形态和免疫表型,即使在芯针活检时也能可靠地诊断ESC-RCC。迄今为止报道的大多数病例病程呈惰性。我们两名患者出现转移,这强调需要收集更多证据来确定这种新出现实体瘤的生物学行为。