Renal Medullary Carcinoma: a Report of the Current Literature

被引:23
作者
Blas, Leandro [1 ,2 ]
Roberti, Javier [1 ]
Petroni, Jorgelina [1 ]
Reniero, Liliana [1 ]
Cicora, Federico [1 ]
机构
[1] FINAER Fdn Res & Assistance Kidney Dis, Palestina 525, RA-1182 Buenos Aires, DF, Argentina
[2] Hosp Aleman, Dept Urol, Buenos Aires, DF, Argentina
关键词
Renal medullary carcinoma; Kidney cancer; Sickle cell; COLLECTING DUCT CARCINOMA; SICKLE-CELL TRAIT; TUMORS; ADOLESCENT; CHILDHOOD; PHENOTYPE; DIAGNOSIS; PATIENT;
D O I
10.1007/s11934-019-0865-9
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Purpose of the ReviewWe present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition.Recent FindingsThere 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.SummaryRMC is a rare and aggressive tumor. Definitive diagnosis requires histological assessment and the presence of sickle-cell hemoglobinopathies.
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