Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil

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 mo...

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Main Authors: Marina Cavalcanti Maroja Silvino, Camila Motta Venchiarutti Moniz, Gustavo Henrique Munhoz Piotto, Sheila Siqueira, Ariel Galapo Kann, Carlos Dzik
Format: Article
Language:English
Published: SAGE Publishing 2013-08-01
Series:Rare Tumors
Subjects:
Online Access:http://www.pagepress.org/journals/index.php/rt/article/view/4751
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author Marina Cavalcanti Maroja Silvino
Camila Motta Venchiarutti Moniz
Gustavo Henrique Munhoz Piotto
Sheila Siqueira
Ariel Galapo Kann
Carlos Dzik
author_facet Marina Cavalcanti Maroja Silvino
Camila Motta Venchiarutti Moniz
Gustavo Henrique Munhoz Piotto
Sheila Siqueira
Ariel Galapo Kann
Carlos Dzik
author_sort Marina Cavalcanti Maroja Silvino
collection DOAJ
description 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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spelling doaj.art-2e61cff4d68e4e3a9b4a8ba467c98dae2022-12-21T23:59:00ZengSAGE PublishingRare Tumors2036-36052036-36132013-08-0153e44e4410.4081/rt.2013.e442581Renal medullary carcinoma response to chemotherapy: a referral center experience in BrazilMarina Cavalcanti Maroja Silvino0Camila Motta Venchiarutti Moniz1Gustavo Henrique Munhoz Piotto2Sheila Siqueira3Ariel Galapo Kann4Carlos Dzik5Instituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São PauloInstituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São PauloInstituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São PauloHospital das Clínicas, Faculdade de Medicina da Universidade de São PauloInstituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São PauloInstituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São PauloRenal 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.http://www.pagepress.org/journals/index.php/rt/article/view/4751kidney neoplasms, renal medullary carcinoma, carcinoma, renal cell, sickle cell trait.
spellingShingle Marina Cavalcanti Maroja Silvino
Camila Motta Venchiarutti Moniz
Gustavo Henrique Munhoz Piotto
Sheila Siqueira
Ariel Galapo Kann
Carlos Dzik
Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil
Rare Tumors
kidney neoplasms, renal medullary carcinoma, carcinoma, renal cell, sickle cell trait.
title Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil
title_full Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil
title_fullStr Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil
title_full_unstemmed Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil
title_short Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil
title_sort renal medullary carcinoma response to chemotherapy a referral center experience in brazil
topic kidney neoplasms, renal medullary carcinoma, carcinoma, renal cell, sickle cell trait.
url http://www.pagepress.org/journals/index.php/rt/article/view/4751
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AT gustavohenriquemunhozpiotto renalmedullarycarcinomaresponsetochemotherapyareferralcenterexperienceinbrazil
AT sheilasiqueira renalmedullarycarcinomaresponsetochemotherapyareferralcenterexperienceinbrazil
AT arielgalapokann renalmedullarycarcinomaresponsetochemotherapyareferralcenterexperienceinbrazil
AT carlosdzik renalmedullarycarcinomaresponsetochemotherapyareferralcenterexperienceinbrazil