Persistent Primary Hyperparathyroidism Secondary to an Ectopic Mediastinal Adenoma in a Young Adult: A Case Report.

IF 0.6 Q4 ENDOCRINOLOGY & METABOLISM Journal of the ASEAN Federation of Endocrine Societies Pub Date : 2023-01-01 Epub Date: 2023-07-15 DOI:10.15605/jafes.038.02.16
Karl Homer Nievera, Rebecca Alba
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Abstract

Primary hyperparathyroidism commonly affects elderly women. When present in the young population, it is usually asymptomatic, most frequently due to a parathyroid adenoma and the definitive management is surgical excision. Uncommonly, 5-10% of patients fail to achieve long-term cure after initial parathyroidectomy and 6-16% of them is due to an ectopic parathyroid adenoma that will require focused diagnostic and surgical approaches. We report a 21-year-old male who had bilateral thigh pain. Work-up revealed bilateral femoral fractures, brown tumors on the arms and multiple lytic lesions on the skull. Serum studies showed hypercalcemia (1.83 mmol/L), elevated parathyroid hormone [(PTH) 2025.10 pg/mL], elevated alkaline phosphatase (830 U/L), normal phosphorus (0.92 mmol/L) and low vitamin D levels (18.50 ng/mL). Bone densitometry showed osteoporotic findings. Sestamibi scan showed uptake on the left superior mediastinal region consistent with an ectopic parathyroid adenoma. Vitamin D supplementation was started pre-operatively. Patient underwent parathyroidectomy with neck exploration; however, the pathologic adenoma was not visualized and PTH levels remained elevated post-operatively. Chest computed tomography with intravenous contrast was performed revealing a mediastinal location of the adenoma. A repeat parathyroidectomy was done, with successful identification of the adenoma resulting in a significant drop in PTH and calcium levels. Patient experienced hungry bone syndrome post-operatively and was managed with calcium and magnesium supplementation. A high index of suspicion for an ectopic adenoma is warranted for patients presenting with hypercalcemia and secondary osteoporosis if there is persistent PTH elevation after initial surgical intervention. Adequate follow-up and monitoring is also needed starting immediately in the post-operative period to manage possible complications such as hungry bone syndrome.

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继发于异位纵隔腺瘤的持续性原发性甲状旁腺功能亢进1例。
原发性甲状旁腺功能亢进常见于老年妇女。当出现在年轻人群时,它通常是无症状的,最常见的是由于甲状旁腺瘤,最终的治疗是手术切除。罕见的是,5-10%的患者在初始甲状旁腺切除术后未能实现长期治愈,其中6-16%是由于异位甲状旁腺瘤,需要集中诊断和手术方法。我们报告一位21岁的男性,他有双侧大腿疼痛。检查发现双侧股骨骨折,手臂上有棕色肿瘤颅骨上有多处溶解性损伤。血清检查显示高钙血症(1.83 mmol/L)、甲状旁腺激素(PTH)升高(2025.10 pg/mL)、碱性磷酸酶升高(830 U/L)、磷正常(0.92 mmol/L)、维生素D低(18.50 ng/mL)。骨密度测定显示骨质疏松。Sestamibi扫描显示左侧上纵隔区摄取与异位甲状旁腺瘤一致。术前开始补充维生素D。患者行甲状旁腺切除术伴颈部探查;然而,病理性腺瘤未可见,术后甲状旁腺素水平仍然升高。胸部计算机断层扫描和静脉造影显示了腺瘤的纵隔位置。再次进行甲状旁腺切除术,成功识别腺瘤,导致甲状旁腺激素和钙水平显著下降。患者术后出现饥饿骨综合征,并给予钙镁补充治疗。对于出现高钙血症和继发性骨质疏松症的患者,如果在最初的手术干预后有持续的甲状旁腺激素升高,应高度怀疑异位腺瘤。术后也需要立即开始适当的随访和监测,以控制可能出现的并发症,如饥饿骨综合征。
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来源期刊
CiteScore
1.20
自引率
0.00%
发文量
22
审稿时长
8 weeks
期刊介绍: The Journal of the ASEAN Federation of Endocrine Societies (JAFES) is an OPEN ACCESS, internationally peer-reviewed, English language, medical and health science journal that is published in print two times a year by the ASEAN Federation of Endocrine Societies. It shall serve as the endocrine window between the ASEAN region and the world, featuring original papers and publishing key findings from specialists and experts of endocrinology.
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