Esposito Melisa, Koroscil Thomas
Internal Medicine, Kettering Health Network, Dayton, USA.
Endocrinology, Wright State University Boonshoft School of Medicine, Dayton, USA.
Cureus. 2023 Jun 21;15(6):e40715. doi: 10.7759/cureus.40715. eCollection 2023 Jun.
Multiple endocrine neoplasia type 2A (MEN2A) is a rare hereditary condition characterized by medullary thyroid cancer, pheochromocytoma, and primary hyperparathyroidism. The current standard of treatment of hyperparathyroidism involves surgical removal of visibly enlarged glands, and auto-transplantation of remnant tissue is often considered to minimize the risk of iatrogenic post-surgical hypocalcemia if multiple glands are enlarged. Rarely, hyperparathyroidism may recur due to hyperplasia or adenoma formation in the auto-transplanted tissue. The following case portrays a 51-year-old male, with a history of MEN2A status post total parathyroidectomy with cryopreservation and subsequent auto-transplantation of remnant parathyroid tissue to the left arm 18 years prior, who presented to establish care due to the insidious development of asymptomatic hypercalcemia. Workup included a laboratory examination showing elevated intact parathyroid hormone (PTH) and left arm ultrasound revealing three areas of enlarged parathyroid tissue at the transplant site, raising suspicion for the development of recurrent primary hyperparathyroidism in auto-transplanted tissue. The patient ultimately underwent a re-do subtotal parathyroidectomy of auto-transplanted tissue with surgical pathology confirming hyperplastic parathyroid tissue. This case highlights the significance of indefinite vigilant surveillance in this patient population, as a recurrence of hyperparathyroidism may occur even after decades of remission.
2A型多发性内分泌腺瘤病(MEN2A)是一种罕见的遗传性疾病,其特征为甲状腺髓样癌、嗜铬细胞瘤和原发性甲状旁腺功能亢进。目前,甲状旁腺功能亢进的标准治疗方法是手术切除明显肿大的腺体,如果多个腺体肿大,通常会考虑对残余组织进行自体移植,以尽量降低医源性术后低钙血症的风险。罕见的是,甲状旁腺功能亢进可能会因自体移植组织中增生或腺瘤形成而复发。以下病例描述了一名51岁男性,有MEN2A病史,18年前接受了甲状旁腺全切术并冷冻保存,随后将残余甲状旁腺组织自体移植到左臂,因无症状高钙血症的隐匿发展前来就诊以接受治疗。检查包括实验室检查显示完整甲状旁腺激素(PTH)升高,左臂超声显示移植部位有三个甲状旁腺组织肿大区域,怀疑自体移植组织中复发性原发性甲状旁腺功能亢进的发生。患者最终接受了自体移植组织再次次全甲状旁腺切除术,手术病理证实为增生性甲状旁腺组织。该病例强调了对该患者群体进行长期密切监测的重要性,因为即使在数十年缓解后,甲状旁腺功能亢进仍可能复发。