Primary Chondrosarcoma in L-shaped Crossed Fused Renal Ectopia Coexisting with Papillary Urothelial Carcinoma in Urinary Bladder - An Enigmatic Entity with Poor Prognosis.

IF 1.9 Q3 ONCOLOGY Journal of Kidney Cancer and VHL Pub Date : 2022-01-01 DOI:10.15586/jkcvhl.v9i1.162
Mayank Kumar, Aasma Nalwa, Taruna Yadav, Poonam Elhence, Himanshu Pandey, Meenakshi Rao
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Abstract

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.

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l型交叉融合肾异位的原发性软骨肉瘤与膀胱乳头状尿路上皮癌共存-一种预后不良的神秘实体。
原发性肾软骨肉瘤是一种罕见的高级别肿瘤,不幸的是,其发病机制尚不清楚,预后极低。单独的恶性肿瘤在膀胱中共存的情况更罕见,在本病例中,膀胱中出现明显的乳头状尿路上皮癌。临床表现是非特异性的,主要的放射学检查在提供这种实体的具体诊断方面具有有限的范围。最终诊断是可能的彻底的组织病理学检查切除标本,这需要广泛的抽样和细致的报告。到目前为止,获得更好预后的唯一方法是早期诊断。在鉴别诊断时,应注意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,以便在患者的整体管理方面取得更令人满意的结果。
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自引率
6.20%
发文量
22
审稿时长
4 weeks
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