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与转移性胰腺神经内分泌肿瘤(NET)分泌甲状旁腺激素相关肽(PTHrP)相关的恶性高钙血症。

Malignant hypercalcaemia related to parathyroid hormone-related peptide (PTHrP) secretion from a metastatic pancreatic neuroendocrine tumour (NET).

作者信息

Symington Megan, Davies Louise, Kaltsas Gregory, Weickert Martin O

机构信息

The ARDEN NET Centre, ENETS CoE, University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK.

出版信息

BMJ Case Rep. 2017 Mar 22;2017:bcr2017219692. doi: 10.1136/bcr-2017-219692.

Abstract

A 54-year-old woman presented to our centre with acute abdominal pain and vomiting. Routine blood tests showed severe hypercalcaemia (>4 mmol/L). Serum parathyroid hormone (PTH) was suppressed. CT scan detected a pancreatic mass and some liver lesions, initially suspicious for metastatic pancreatic adenocarcinoma. Liver biopsy however revealed the presence of a well-differentiated, grade 1, metastatic neuroendocrine tumour (NET) where prognosis is considerably better. Serum PTHrP was raised, indicating paraneoplastic hypercalcaemia, most likely secondary to the pancreatic NET. Following injection of a short-acting somatostatin analogue octreotide, serum PTHrP levels normalised within 24 hours, causing a rapid drop of serum calcium below the lower limit of normal and an immediate compensatory rise of serum PTH. Ongoing treatment with long-acting somatostatin analogues together with replacement with calcium carbonate, vitamin D3 and once weekly alendronic acid resulted in stable normal adjusted calcium levels over a 3-month follow-up period.

摘要

一名54岁女性因急性腹痛和呕吐前来我院就诊。常规血液检查显示严重高钙血症(>4 mmol/L)。血清甲状旁腺激素(PTH)受到抑制。CT扫描发现胰腺肿块和一些肝脏病变,最初怀疑为转移性胰腺腺癌。然而,肝脏活检显示存在高分化1级转移性神经内分泌肿瘤(NET),其预后要好得多。血清甲状旁腺激素相关蛋白(PTHrP)升高,提示副肿瘤性高钙血症,很可能继发于胰腺NET。注射短效生长抑素类似物奥曲肽后,血清PTHrP水平在24小时内恢复正常,导致血清钙迅速降至正常下限以下,血清PTH立即出现代偿性升高。长期使用长效生长抑素类似物治疗,同时补充碳酸钙、维生素D3和每周一次阿仑膦酸钠,在3个月的随访期内,调整后的血钙水平保持稳定正常。

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Paraneoplastic endocrine syndromes: a review.副肿瘤性内分泌综合征:综述
Endocr Pathol. 2003 Winter;14(4):303-17. doi: 10.1385/ep:14:4:303.

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