Primary Chondrosarcoma in L-shaped Crossed Fused Renal Ectopia Coexisting with Papillary Urothelial Carcinoma in Urinary Bladder - An Enigmatic Entity with Poor Prognosis.
{"title":"Primary Chondrosarcoma in L-shaped Crossed Fused Renal Ectopia Coexisting with Papillary Urothelial Carcinoma in Urinary Bladder - An Enigmatic Entity with Poor Prognosis.","authors":"Mayank Kumar, Aasma Nalwa, Taruna Yadav, Poonam Elhence, Himanshu Pandey, Meenakshi Rao","doi":"10.15586/jkcvhl.v9i1.162","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":44291,"journal":{"name":"Journal of Kidney Cancer and VHL","volume":"9 1","pages":"9-14"},"PeriodicalIF":1.9000,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8571990/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Kidney Cancer and VHL","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.15586/jkcvhl.v9i1.162","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0
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.