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      Renal Medullary Carcinoma Response to Chemotherapy: a Referral Center Experience in Brazil

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          Renal medullary carcinoma (RMC) is rare, accounting for less than 1% of all renal neoplasms. Case reports suggest RMC is highly aggressive, poorly responsive to chemotherapy, often metastatic at diagnosis, affects young men with sickle cell trait, and median overall survival (mOS) is less than 12 months. We report the epidemiological characteristics, treatments performed, response rate to each treatment and mOS of five patients with RMC. All patients had sickle cell trait, four were male, three had metastatic disease at diagnosis and mean age at diagnosis was 25 years. Non-metastatic patients were submitted to nephrectomy. Two patients had partial response to first line chemotherapy including cisplatin and gemcitabine. There was no response to sunitinib or second line chemo - therapy; mOS was 6 months. Due to its rarity, case series are the only evidence available to discuss the treatment for RMC. In our experience, only cisplatin and gemcitabine based regimen offered response.

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          Renal medullary carcinoma. The seventh sickle cell nephropathy.

          Over the last 22 years, we have encountered 34 examples of a highly aggressive neoplasm with a microscopic morphology that is highly predictive of finding sickled erythrocytes in the tissue. With the exception of one patient, all are believed to have had sickle cell trait or, in one case, hemoglobin SC disease. These 33 patients are the subject of this report and, where their race was known, they were all blacks between the ages of 11 and 39 years. Between the ages of 11 and 24 years, males predominated by 3 to 1. Beyond age 24, however, the tumors occurred equally in men and women. The dominant tumor mass was in the medulla and ranged from 4 to 12 cm in diameter. Mean size was 7 cm; median, 6 cm. Peripheral satellites in the renal cortex and pelvic soft tissues, as well as venous and lymphatic invasion, were usually present. The lesions exhibited a reticular, yolk sac-like, or adenoid cystic appearance, often with poorly differentiated areas in a highly desmoplastic stroma admixed with neutrophils and usually marginated by lymphocytes. The tumors had usually metastasized when first discovered, and none was confined to the kidney at the time of nephrectomy. The mean duration of life after surgery was 15 weeks. These tumors probably arise in the calyceal epithelium in or near the renal papillae, the same site that produces the more familiar picture of unilateral hematuria in patients with sickle cell trait. We have concluded that renal medullary carcinoma represents another example of renal disease associated with sickle cell disorders. The other six are unilateral hematuria, papillary necrosis, nephrotic syndrome, renal infarction, inability to concentrate urine, and pyelonephritis.
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            Renal medullary carcinoma: clinical, pathologic, immunohistochemical, and genetic analysis with pathogenetic implications.

            To investigate the pathologic, clinical, and genetic features of renal medullary carcinomas (RMCs) in search of clues to their pathogenesis. We analyzed 40 RMCs for clinical features, for immunohistochemical expression using a panel of markers, and for genetic changes using comparative genomic hybridization. Patients presented at 5 to 32 years of age, and 82% were African American. All patients tested had sickle cell trait or disease. Seven patients presented with suspected renal abscess or urinary track infection without a clinically recognizable mass. Of the 15 tumors able to be analyzed, all were positive for epithelial markers CAM 5.2 and epithelial membrane antigen. All were negative for high-molecular-weight cytokeratin 34betaE12. Cytokeratins 7 and 20 and carcinoembryonic antigen were heterogeneous and variable. Ulex was focally positive in a minority of cases. Eight of 12 tumors showed significant positivity for TP53 protein (greater than 25% nuclear positivity). All tumor tested (n = 8) were strongly positive for vascular endothelial growth factor and hypoxia inducible factor. Of nine tumors analyzed for genetic gains and losses using comparative genomic hybridization, eight showed no changes and one showed loss of chromosome 22. Survival ranged from 2 weeks to 15 months (mean 4 months). These findings suggest that RMC is clinically and pathologically distinct from collecting duct carcinoma. The hypothesis that chronic medullary hypoxia secondary to hemoglobinopathy may be involved in the pathogenesis of RMC is suggested by strong vascular endothelial growth factor and hypoxia inducible factor expression and positivity for TP53.
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              Significant responses to platinum-based chemotherapy in renal medullary carcinoma.

              Most patients with renal medullary carcinoma (RMC) have advanced disease at presentation and rarely respond to radiation or chemotherapy. We describe two adolescents with metastatic disease who had significant responses to cisplatin or carboplatin in combination with gemcitabine and paclitaxel.

                Author and article information

                Rare Tumors
                Rare Tumors
                Rare Tumors
                PAGEPress Publications, Pavia, Italy
                20 August 2013
                01 July 2013
                : 5
                : 3
                [1 ]Instituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São Paulo
                [2 ]Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo , Brazil
                Author notes
                Rua Ministro Gastão Mesquita 250, City of São Paulo, ZIP code 05012010, Brazil. Tel. +55.11.997589494. E-mail: masilvino85@
                ©Copyright M. Cavalcanti Maroja Silvino et al.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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