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
Department of Urology, Henri Mondor Hospital, University of Paris Est Créteil (UPEC), Créteil, France.
Université Paris-Saclay, CEA, CNRS, Inserm, BioMaps, Villejuif, France.
Front Oncol. 2023 May 2;13:1073728. doi: 10.3389/fonc.2023.1073728. eCollection 2023.
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.
肾髓质癌（RMC）是一种预后较差的罕见肾细胞癌形式。已知它与镰状细胞性状或疾病有关，尽管确切的潜在机制仍不清楚。诊断通过SMARCB1（INI1）免疫化学染色进行。在本报告中，我们介绍了一例31岁患有镰状细胞性状的男性患者，他被诊断为III期右肾髓质癌。尽管预后不佳，但该患者存活了长达37个月。主要使用18F-FDG PET/MRI进行放射学评估和随访。患者在手术切除右肾和腹膜后淋巴结清扫术前接受了基于顺铂的细胞毒性化疗。术后给予相同的辅助化疗。在腹膜后淋巴结中检测到疾病复发；这些通过化疗和再次手术治疗。我们还讨论了肾髓质癌的肿瘤学和手术管理，目前其依赖围手术期细胞毒性化疗策略，因为迄今为止尚无已知的替代疗法被证明更优越。
