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

Frontiers in Oncology

Oncology

Alexandre Ingels¹  Alexandre De La Taille¹  Jean Courcier²  Onder Kara³  Eduard Roussel⁴  Riccardo Bertolo⁵  Selcuk Erdem⁶  Daniele Amparore⁷  Maria Mamodaly⁸  Riccardo Campi⁹  Nicola Pavan^1,10  Michele Marchioni^1,11 

[1] Department of Urology, Henri Mondor Hospital, University of Paris Est Créteil (UPEC), Créteil, France;Department of Urology, Henri Mondor Hospital, University of Paris Est Créteil (UPEC), Créteil, France;Université Paris-Saclay, CEA, CNRS, Inserm, BioMaps, Villejuif, France;Department of Urology, Kocaeli University School of Medicine, Kocaeli, Türkiye;Department of Urology, Onze-Lieve-Vrouwziekenhuis (OLV) Hospital, Aalst, Belgium;Department of Urology, University Hospitals Leuven, Leuven, Belgium;Department of Urology, San Carlo Di Nancy Hospital, Rome, Italy;Division of Urologic Oncology, Department of Urology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Türkiye;Division of Urology, Department of Oncology, School of Medicine, San Luigi Hospital, University of Turin, Orbassano, Italy;Pathology Department, University of Paris Est Créteil (UPEC), Henri Mondor Hospital, Créteil, France;Unit of Urological Robotic Surgery and Renal Transplantation, Careggi Hospital, University of Florence, Florence, Italy;Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy;Urology Clinic, Department of Medical Surgical and Health Science, University of Trieste, Trieste, Italy;Urology Unit, Department of Medical, Oral and Biotechnological Sciences, "G. d'Annunzio" University of Chieti, Chieti, Italy;

关键词: non-clear cell RCC;    renal medullary carcinoma;    SMARCB1;    chemotherapy;    onco-urological management;   

DOI  :  10.3389/fonc.2023.1073728

 received in 2022-10-18, accepted in 2023-03-31,  发布年份 2023

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

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Copyright © 2023 Courcier, De La Taille, Bertolo, Amparore, Erdem, Kara, Marchioni, Pavan, Roussel, Mamodaly, Campi and Ingels