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Surgical and oncological management of renal medullary carcinoma in a young patient: a case report

Renal medullary carcinoma (RMC) is a rare form of renal cell carcinoma that has a poor prognosis. It is known to be associated with sickle cell trait or disease, although the exact underlying mechanisms are still unclear. The diagnosis is made through immunochemical staining for SMARCB1 (INI1). In t...

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Autores principales: Courcier, Jean, De La Taille, Alexandre, Bertolo, Riccardo, Amparore, Daniele, Erdem, Selcuk, Kara, Onder, Marchioni, Michele, Pavan, Nicola, Roussel, Eduard, Mamodaly, Maria, Campi, Riccardo, Ingels, Alexandre
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10185901/
https://www.ncbi.nlm.nih.gov/pubmed/37205186
http://dx.doi.org/10.3389/fonc.2023.1073728
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author Courcier, Jean
De La Taille, Alexandre
Bertolo, Riccardo
Amparore, Daniele
Erdem, Selcuk
Kara, Onder
Marchioni, Michele
Pavan, Nicola
Roussel, Eduard
Mamodaly, Maria
Campi, Riccardo
Ingels, Alexandre
author_facet Courcier, Jean
De La Taille, Alexandre
Bertolo, Riccardo
Amparore, Daniele
Erdem, Selcuk
Kara, Onder
Marchioni, Michele
Pavan, Nicola
Roussel, Eduard
Mamodaly, Maria
Campi, Riccardo
Ingels, Alexandre
author_sort Courcier, Jean
collection PubMed
description Renal medullary carcinoma (RMC) is a rare form of renal cell carcinoma that has a poor prognosis. It is known to be associated with sickle cell trait or disease, although the exact underlying mechanisms are still unclear. The diagnosis is made through immunochemical staining for SMARCB1 (INI1). In this report, we present a case of a 31-year-old male patient with sickle cell trait who was diagnosed with stage III right RMC. Despite the poor prognosis, the patient survived for a remarkable duration of 37 months. Radiological assessment and follow-up were primarily performed using 18F-FDG PET/MRI. The patient underwent upfront cisplatin-based cytotoxic chemotherapy before surgical removal of the right kidney and retroperitoneal lymph node dissection. Identical adjuvant chemotherapy was administered post-surgery. Disease relapses were detected in the retroperitoneal lymph nodes; these were managed with chemotherapy and surgical rechallenges. We also discuss the oncological and surgical management of RMC, which currently relies on perioperative cytotoxic chemotherapy strategies, as there are no known alternative therapies that have been shown to be superior to date.
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spelling pubmed-101859012023-05-17 Surgical and oncological management of renal medullary carcinoma in a young patient: a case report Courcier, Jean De La Taille, Alexandre Bertolo, Riccardo Amparore, Daniele Erdem, Selcuk Kara, Onder Marchioni, Michele Pavan, Nicola Roussel, Eduard Mamodaly, Maria Campi, Riccardo Ingels, Alexandre Front Oncol Oncology Renal medullary carcinoma (RMC) is a rare form of renal cell carcinoma that has a poor prognosis. It is known to be associated with sickle cell trait or disease, although the exact underlying mechanisms are still unclear. The diagnosis is made through immunochemical staining for SMARCB1 (INI1). In this report, we present a case of a 31-year-old male patient with sickle cell trait who was diagnosed with stage III right RMC. Despite the poor prognosis, the patient survived for a remarkable duration of 37 months. Radiological assessment and follow-up were primarily performed using 18F-FDG PET/MRI. The patient underwent upfront cisplatin-based cytotoxic chemotherapy before surgical removal of the right kidney and retroperitoneal lymph node dissection. Identical adjuvant chemotherapy was administered post-surgery. Disease relapses were detected in the retroperitoneal lymph nodes; these were managed with chemotherapy and surgical rechallenges. We also discuss the oncological and surgical management of RMC, which currently relies on perioperative cytotoxic chemotherapy strategies, as there are no known alternative therapies that have been shown to be superior to date. Frontiers Media S.A. 2023-05-02 /pmc/articles/PMC10185901/ /pubmed/37205186 http://dx.doi.org/10.3389/fonc.2023.1073728 Text en Copyright © 2023 Courcier, De La Taille, Bertolo, Amparore, Erdem, Kara, Marchioni, Pavan, Roussel, Mamodaly, Campi and Ingels https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Courcier, Jean
De La Taille, Alexandre
Bertolo, Riccardo
Amparore, Daniele
Erdem, Selcuk
Kara, Onder
Marchioni, Michele
Pavan, Nicola
Roussel, Eduard
Mamodaly, Maria
Campi, Riccardo
Ingels, Alexandre
Surgical and oncological management of renal medullary carcinoma in a young patient: a case report
title Surgical and oncological management of renal medullary carcinoma in a young patient: a case report
title_full Surgical and oncological management of renal medullary carcinoma in a young patient: a case report
title_fullStr Surgical and oncological management of renal medullary carcinoma in a young patient: a case report
title_full_unstemmed Surgical and oncological management of renal medullary carcinoma in a young patient: a case report
title_short Surgical and oncological management of renal medullary carcinoma in a young patient: a case report
title_sort surgical and oncological management of renal medullary carcinoma in a young patient: a case report
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10185901/
https://www.ncbi.nlm.nih.gov/pubmed/37205186
http://dx.doi.org/10.3389/fonc.2023.1073728
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