Matrone Antonio, Brancatella Alessandro, Marchetti Piero, Vasile Enrico, Boggi Ugo, Elisei Rossella, Cetani Filomena, Marcocci Claudio, Vitti Paolo, Latrofa Francesco
Department of Clinical and Experimental Medicine, Unit of Endocrinology I, University of Pisa, I-56124 Pisa, Italy.
Department of Clinical and Experimental Medicine, Division of Metabolism and Cell Transplantation, University of Pisa, I-56124 Pisa, Italy.
Mol Clin Oncol. 2018 Mar;8(3):449-452. doi: 10.3892/mco.2017.1542. Epub 2017 Dec 29.
Absence of neoplastic disease in the organ-recipient is required in order to allow organ transplantation. Due to its rarity, no data regarding management of patients with Multiple endocrine neoplasia type 1 (MEN1) and end-stage renal failure candidates for kidney transplantation are available. A 36 year-old man was referred to the present hospital with MEN1, with a neuroendocrine pancreatic tumor and primary hyperparathyroidism and associated Alport syndrome with end stage renal failure. The present study aimed to establish the eligibility of the patient for a kidney transplantation. The neuroendocrine tumor had been treated with duodenopancreatectomy two years earlier and hyperparathyroidism by parathyroidectomy. The review of the literature did not provide data regarding the eligibility for kidney transplantation of patients harboring a neuroendocrine pancreatic tumor in the context of MEN1. Due to the end-stage renal failure, neuroendocrine markers were unreliable and the investigation therefore relied on imaging studies, which were unremarkable. Young age, low-grade tumor, low expression of Ki67, absence of metastatic lymph nodes, onset in the setting of a MEN1 were all positive prognostic factors of the neuroendocrine tumor. Normal serum calcium ruled out persistent primary hyperparathyroidism. Overall, hemodyalisis is known to significantly reduce life expectancy. Benefits of kidney transplantation overcome the risk of neuroendocrine tumor recurrence in a young patient bearing MEN1.
器官移植需要器官接受者不存在肿瘤性疾病。由于其罕见性，目前尚无关于1型多发性内分泌肿瘤（MEN1）合并终末期肾衰竭且适合肾移植的患者的治疗数据。一名36岁男性因MEN1被转诊至本院，患有神经内分泌胰腺肿瘤、原发性甲状旁腺功能亢进，并伴有Alport综合征及终末期肾衰竭。本研究旨在确定该患者是否适合进行肾移植。两年前，神经内分泌肿瘤已通过十二指肠胰腺切除术进行治疗，甲状旁腺功能亢进通过甲状旁腺切除术进行治疗。文献回顾未提供在MEN1背景下患有神经内分泌胰腺肿瘤的患者进行肾移植的适用性数据。由于终末期肾衰竭，神经内分泌标志物不可靠，因此该研究依赖于影像学检查，结果无异常。年轻、肿瘤分级低、Ki67表达低、无转移性淋巴结、在MEN1背景下发病均为神经内分泌肿瘤的阳性预后因素。血清钙正常排除了持续性原发性甲状旁腺功能亢进。总体而言，已知血液透析会显著缩短预期寿命。肾移植的益处超过了患有MEN1的年轻患者神经内分泌肿瘤复发的风险。
