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生长抑素瘤综合征:是否存在一种临床实体?

Somatostatinoma syndrome: does a clinical entity exist?

作者信息

Stacpoole P W, Kasselberg A G, Berelowitz M, Chey W Y

出版信息

Acta Endocrinol (Copenh). 1983 Jan;102(1):80-7. doi: 10.1530/acta.0.1020080.

DOI:10.1530/acta.0.1020080
PMID:6297217
Abstract

We report here 2 patients with somatostatin-secreting tumours and hypersomatostatinaemia. One subject, a 36 year old woman with diabetes, flushing, labile blood pressure and diarrhea, had elevated basal plasma levels of somatostatin-like immunoreactivity (SLIR) and calcitonin. Plasma SLIR increased further following tolbutamide administration. Plasma levels of prostaglandin E2 (PGE2) and pancreatic polypeptide (PP), normal in the basal state, showed exaggerated responses to pentagastrin and secretin, respectively. Immunocytochemistry of the tumour tissue revealed cells containing somatostatin-, calcitonin-, PGE2- and PP-like immunoreactivity. The other patient, a 52 year old male, had an SLIR-secreting tumour of the proximal duodenum and elevated basal and post-tolbutamide SLIR levels but no signs or symptoms suggestive of increased SLIR production. Tumour tissue revealed cells containing somatostatin- and calcitonin-like immunoreactivity. We conclude that patients with somatostatinomas do not always exhibit a predictable syndrome. Patients with these tumours may exhibit a range of clinical, biochemical and immunocytochemical features typical of endocrine tumours of mixed-cell origin, such that the dominant signs and symptoms associated with these neoplasms cannot readily be ascribed to overproduction of any single hormone.

摘要

我们在此报告2例分泌生长抑素的肿瘤及高生长抑素血症患者。其中1例为36岁女性,患有糖尿病、潮红、血压不稳定及腹泻,基础血浆生长抑素样免疫反应性(SLIR)和降钙素水平升高。给予甲苯磺丁脲后,血浆SLIR进一步升高。基础状态下正常的前列腺素E2(PGE2)和胰多肽(PP)血浆水平,分别对五肽胃泌素和促胰液素显示出过度反应。肿瘤组织的免疫细胞化学显示含有生长抑素、降钙素、PGE2和PP样免疫反应性的细胞。另1例患者为52岁男性,患有十二指肠近端分泌SLIR的肿瘤,基础及甲苯磺丁脲给药后SLIR水平升高,但无提示SLIR分泌增加的体征或症状。肿瘤组织显示含有生长抑素和降钙素样免疫反应性的细胞。我们得出结论,生长抑素瘤患者并不总是表现出可预测的综合征。这些肿瘤患者可能表现出一系列典型的混合细胞起源内分泌肿瘤的临床、生化和免疫细胞化学特征,因此与这些肿瘤相关的主要体征和症状不能轻易归因于任何单一激素的过度分泌。

相似文献

1
Somatostatinoma syndrome: does a clinical entity exist?生长抑素瘤综合征:是否存在一种临床实体?
Acta Endocrinol (Copenh). 1983 Jan;102(1):80-7. doi: 10.1530/acta.0.1020080.
2
Somatostatin-28 like immunoreactivity in normal and tumour tissue from duodenum and pancreas.十二指肠和胰腺正常组织及肿瘤组织中类生长抑素-28免疫反应性
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Hypercalcitonemia revealing a somatostatinoma.高钙血症提示生长抑素瘤。
Ann Endocrinol (Paris). 2010 Dec;71(6):553-7. doi: 10.1016/j.ando.2010.05.001.
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Five cases of somatostatinoma: clinical heterogeneity and diagnostic usefulness of basal and tolbutamide-induced hypersomatostatinemia.五例生长抑素瘤:临床异质性及基础和甲苯磺丁脲诱导的高生长抑素血症的诊断价值
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引用本文的文献

1
Somatostatin analogs and gallstones: a retrospective survey on a large series of acromegalic patients.生长抑素类似物与胆结石:对大量肢端肥大症患者的回顾性调查
J Endocrinol Invest. 2008 Aug;31(8):704-10. doi: 10.1007/BF03346419.
2
Basal plasma somatostatin in biliary stone patients.胆石症患者的基础血浆生长抑素
Klin Wochenschr. 1984 Jun 15;62(12):595-7. doi: 10.1007/BF01728179.
3
Somatostatin.生长抑素
Br Med J (Clin Res Ed). 1987 Aug 1;295(6593):288-90. doi: 10.1136/bmj.295.6593.288.
4
Co-secretion of calcitonin gene-related peptide, gastrin-releasing peptide and ACTH by a carcinoid tumour metastasizing to the cerebellum.一个转移至小脑的类癌肿瘤同时分泌降钙素基因相关肽、胃泌素释放肽和促肾上腺皮质激素。
Postgrad Med J. 1987 Feb;63(736):123-30. doi: 10.1136/pgmj.63.736.123.