Division of Endocrinology, University of Ottawa, Ottawa, ON, Canada.
Int J Gen Med. 2013 Dec 6;7:21-7. doi: 10.2147/IJGM.S51302. eCollection 2013.
To report the case of a 28-year-old woman who presented with hypercalcemia (total calcium =4.11 mmol/L), elevated parathyroid hormone (PTH) 24.6 pmol/L, normal parathyroid hormone-related peptide 7.8 pg/mL, and a 63 mm × 57 mm, poorly differentiated neuroendocrine carcinoma (small-cell type) pancreatic mass with liver metastases.
Hypercalcemia was acutely managed with intravenous fluids, pamidronate and calcitonin. Investigations for multiple endocrine neoplasia type 1 and parathyroid adenoma were initiated. The identified neuroendocrine tumor was treated with cisplatinum/etoposide chemotherapy.
The pancreatic mass (56 mm × 49 mm) and metastases decreased in size with chemotherapy and calcium levels normalized. Eight months later, calcium increased to 3.23 mmol/L, PTH increased to 48.2 pmol/L, and the pancreatic mass increased in size to 67 mm × 58 mm. The patient was given a trial of cinacalcet but was unable to tolerate it. Chemotherapy was restarted and resulted in a decrease in the pancreatic mass (49 mm × 42 mm), a reduction in PTH levels (16.6 pmol/L), and calcium levels (2.34 mmol/L).
Ectopic PTH secreting tumors should be considered when there is no parathyroid related cause for an elevated PTH. Recognizing the association between PTH and hypercalcemia of malignancy may lead to an earlier detection of an undiagnosed malignancy.
报告 1 例 28 岁女性病例,其表现为高钙血症(总钙=4.11mmol/L)、甲状旁腺激素(PTH)升高 24.6pmol/L、甲状旁腺激素相关肽 7.8pg/mL 正常,胰腺有一个 63mm×57mm、分化差的神经内分泌癌(小细胞型)肿块,伴肝转移。
通过静脉补液、帕米膦酸和降钙素急性治疗高钙血症。开始进行多发性内分泌肿瘤 1 型和甲状旁腺腺瘤的检查。确定的神经内分泌肿瘤采用顺铂/依托泊苷化疗治疗。
化疗后,胰腺肿块(56mm×49mm)和转移灶的大小减小,钙水平恢复正常。8 个月后,钙增至 3.23mmol/L,PTH 增至 48.2pmol/L,胰腺肿块增大至 67mm×58mm。给予患者西那卡塞试验治疗,但无法耐受。重新开始化疗后,胰腺肿块(49mm×42mm)减小,PTH 水平(16.6pmol/L)降低,钙水平(2.34mmol/L)降低。
当没有甲状旁腺相关原因引起 PTH 升高时,应考虑异位 PTH 分泌性肿瘤。认识到 PTH 与恶性肿瘤高钙血症之间的关系可能会更早地发现未诊断的恶性肿瘤。