Leandro Blas1,2, Javier Roberti3, Jorgelina Petroni1, Liliana Reniero1, Federico Cicora1. 1. FINAER (Foundation for Research and Assistance in Kidney Disease), Palestina 525, 1182, Buenos Aires, Argentina. 2. Urology Department, Hospital Alemán, Buenos Aires, Argentina. 3. FINAER (Foundation for Research and Assistance in Kidney Disease), Palestina 525, 1182, Buenos Aires, Argentina. javierroberti@gmail.com.
Abstract
PURPOSE OF THE REVIEW: We present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition. RECENT FINDINGS: There is a majority of male patients, of African descent, in the second or third decade of life. In differential diagnosis, other tumors, such as malignant rhabdoid tumor (MRT), vinculin-anaplastic lymphoma kinase (VCL-ALK) translocation renal cell carcinoma, and collecting duct carcinoma, may present difficulties. Abnormalities of tumor suppressor gene SMARCB1 have been found in RMC. Reported symptoms were hematuria, pain, weight loss, respiratory distress, palpable mass, cough, and fever. Most patients present with metastases at diagnosis. There is no definite recommended treatment, and protocols are extrapolated from other malignancies, with nephrectomy and systemic therapies being most frequently used. Response to treatment and prognosis remain very poor. RMC is a rare and aggressive tumor. Definitive diagnosis requires histological assessment and the presence of sickle-cell hemoglobinopathies.
PURPOSE OF THE REVIEW: We present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition. RECENT FINDINGS: There is a majority of male patients, of African descent, in the second or third decade of life. In differential diagnosis, other tumors, such as malignant rhabdoid tumor (MRT), vinculin-anaplastic lymphoma kinase (VCL-ALK) translocation renal cell carcinoma, and collecting duct carcinoma, may present difficulties. Abnormalities of tumor suppressor gene SMARCB1 have been found in RMC. Reported symptoms were hematuria, pain, weight loss, respiratory distress, palpable mass, cough, and fever. Most patients present with metastases at diagnosis. There is no definite recommended treatment, and protocols are extrapolated from other malignancies, with nephrectomy and systemic therapies being most frequently used. Response to treatment and prognosis remain very poor. RMC is a rare and aggressive tumor. Definitive diagnosis requires histological assessment and the presence of sickle-cell hemoglobinopathies.
Authors: David S Tourigny; Mark Zucker; Minsoo Kim; Paul Russo; Jonathan Coleman; Chung-Han Lee; Maria I Carlo; Ying-Bei Chen; A Ari Hakimi; Ritesh R Kotecha; Ed Reznik Journal: Front Oncol Date: 2022-06-28 Impact factor: 5.738