Kumar Mayank, Nalwa Aasma, Yadav Taruna, Elhence Poonam, Pandey Himanshu, Rao Meenakshi
Department of Pathology, Autonomous State Medical College, Ayodhya, India.
Department of Pathology, All India Institute of Medical Sciences, Jodhpur, India.
J Kidney Cancer VHL. 2022 Jan 1;9(1):9-14. doi: 10.15586/jkcvhl.v9i1.162. eCollection 2022.
Primary renal chondrosarcomas are rare tumors that are high-grade in nature and, unfortunately, have poorly understood pathogenesis and extremely low prognosis. The coexistence of a discrete malignancy in the urinary bladder is even rarer, with the occurrence of distinct papillary urothelial carcinoma in the urinary bladder in this case. The clinical presentation is nonspecific, and the primary radiological investigations have a limited scope in providing specific diagnosis of this entity. The final diagnosis is possible on thorough histopathological examination of the resected specimen, which requires extensive sampling and meticulous reporting. As of now, the only way to achieve a better prognosis is by early diagnosis. It is necessary to keep the possibility of occurrence of sarcomas at rare sites in the differential diagnoses. The cytogenetic and molecular abnormalities associated with this entity need to be elucidated to achieve a more satisfactory outcome concerning the overall management of the patient.
原发性肾软骨肉瘤是一种罕见的肿瘤，本质上属于高级别肿瘤，不幸的是，其发病机制尚不清楚，预后极差。膀胱中同时存在一种独立的恶性肿瘤则更为罕见，本例中膀胱出现了明显的乳头状尿路上皮癌。临床表现不具特异性，初步的影像学检查在对该实体进行特异性诊断方面作用有限。最终诊断需通过对切除标本进行全面的组织病理学检查来实现，这需要广泛取材和细致报告。截至目前，实现更好预后的唯一方法是早期诊断。在鉴别诊断中必须考虑到罕见部位发生肉瘤的可能性。需要阐明与该实体相关的细胞遗传学和分子异常情况，以便在患者的整体管理方面取得更满意的结果。
