Severe, Symptomatic Hypercalcemia Secondary to PTH-secreting Pancreatoblastoma.

JCEM case reports Pub Date : 2024-11-28 eCollection Date: 2024-12-01 DOI:10.1210/jcemcr/luae217
Anand D Gandhi, James D McCallum, Jonathan S Fisher
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Abstract

Hypercalcemia may be induced by a variety of etiologies, most commonly primary hyperparathyroidism. Although primary hyperparathyroidism represents a relatively common endocrinological disorder, ectopic PTH secretion is a rare entity that is less well described in literature. We describe the first case to our knowledge of severe, symptomatic hypercalcemia found to be secondary to a PTH-secreting pancreatoblastoma. The patient initially presented with fatigue and progressive upper extremity intermittent muscular twitching. He was found to have biochemical evidence of primary hyperparathyroidism. A computed tomography scan of the neck and a sestamibi nuclear scan failed to definitively demonstrate a parathyroid adenoma or hyperplasia and bilateral surgical parathyroid exploration was unrevealing for any pathology. Abdominal imaging via computed tomography was obtained for evaluation of progressive postoperative epigastric pain, and the patient was found to have a retroperitoneal mass that, after biopsy, was diagnostic for a pancreatoblastoma. This mass was resected resulting in a fall in intraoperative PTH values and subsequent postoperative hypocalcemia secondary to hungry bone syndrome. Upon follow-up, the patient's parathyroid function recovered and doses of supplemental calcium and vitamin D could be tapered. Ectopic PTH-secreting masses represent a rare entity but should be considered in individuals with unclear etiology of recalcitrant primary hyperparathyroidism.

分泌甲状旁腺激素的胰腺母细胞瘤继发的严重症状性高钙血症。
高钙血症可由多种病因引起,最常见的是原发性甲状旁腺功能亢进。虽然原发性甲状旁腺功能亢进是一种相对常见的内分泌疾病,但异位甲状旁腺激素分泌是一种罕见的疾病,在文献中描述较少。我们描述的第一个病例严重,症状性高钙血症发现继发于甲状旁腺激素分泌胰腺母细胞瘤。患者最初表现为疲劳和进行性上肢间歇性肌肉抽搐。他被发现有原发性甲状旁腺功能亢进的生化证据。颈部的计算机断层扫描和sestamibi核扫描未能明确显示甲状旁腺腺瘤或增生,双侧手术甲状旁腺探查未发现任何病理。通过计算机断层扫描获得腹部成像以评估术后进行性胃脘痛,患者发现腹膜后肿块,经活检诊断为胰腺母细胞瘤。该肿块被切除,导致术中PTH值下降,术后继发于饥饿骨综合征的低钙血症。随访后,患者甲状旁腺功能恢复,补钙和维生素D的剂量可逐渐减少。异位甲状旁腺激素分泌团块是一种罕见的实体,但在病因不明的难治性原发性甲状旁腺功能亢进患者中应予以考虑。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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