Khera Sudeep, Elhence Poonam, Yadav Taruna, Pandey Himanshu
Department of Pathology, All India Institute of Medical Sciences, Jodhpur, India.
Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, India.
Ochsner J. 2022 Summer;22(2):182-187. doi: 10.31486/toj.21.0065.
Tubulocystic renal cell carcinoma is a lesser-known neoplastic entity compared to other common histologic variants of renal cell carcinoma. The World Health Organization identified tubulocystic renal cell carcinoma as a newly recognized renal tumor in 2016. We report a case of tubulocystic renal cell carcinoma in a young adult. A 21-year-old male presented with the chief complaint of a lump on the right side of his abdomen since childhood. Magnetic resonance imaging revealed an enlarged right kidney with multiple large multiloculated cysts with hemorrhagic contents and enhancing peripheral nodular solid components with enhancing septa in some of the cysts, suggesting the possibility of multifocal intracystic papillary renal cell carcinoma. Imaging showed a Bosniak type IV cystic lesion in the right kidney. Right radical nephrectomy was performed. Grossly, the kidney was almost replaced by variable-sized cystic lesions with thick septations filled with serous fluid to gelatinous material. The tubules and cysts were lined by a single layer of flat, hobnail, and cuboidal cells with high-grade nuclear features. No ovarian-type stroma was identified. In places, a papillary component was also identified. Tubulocystic renal cell carcinoma was diagnosed based on microscopy and immunohistochemistry results. Tubulocystic renal cell carcinoma is a rare entity that was previously called Bellini duct carcinoma and low-grade collecting duct carcinoma. Because of the limited number of cases reported, tubulocystic renal cell carcinoma needs to be followed to determine the biologic behavior and metastatic potential of these tumors so that management guidelines for such cases can be developed.
与肾细胞癌的其他常见组织学变体相比，管状囊性肾细胞癌是一种鲜为人知的肿瘤实体。2016年，世界卫生组织将管状囊性肾细胞癌确定为一种新发现的肾肿瘤。我们报告一例年轻成人的管状囊性肾细胞癌病例。一名21岁男性自童年起就以腹部右侧肿块为主诉就诊。磁共振成像显示右肾增大，有多个大的多房性囊肿，内含出血成分，部分囊肿的周边结节状实性成分及分隔有强化，提示多灶性囊内乳头状肾细胞癌的可能性。影像学检查显示右肾为博斯尼亚克IV型囊性病变。遂行右肾根治性切除术。大体上，肾脏几乎被大小不一的囊性病变所取代，有厚的分隔，内充满浆液性液体至胶冻样物质。小管和囊肿内衬单层扁平、鞋钉样和立方形细胞，具有高级别核特征。未发现卵巢样间质。在某些部位，还发现了乳头状成分。根据显微镜检查和免疫组化结果诊断为管状囊性肾细胞癌。管状囊性肾细胞癌是一种罕见的实体，以前称为贝氏管癌和低级别集合管癌。由于报道的病例数量有限，需要对管状囊性肾细胞癌进行随访，以确定这些肿瘤的生物学行为和转移潜能，从而制定此类病例的管理指南。
