1型多发性内分泌肿瘤中的淋巴结胃泌素瘤——一项诊断挑战。

Lymph node gastrinoma in multiple endocrine neoplasia type 1 - a diagnostic challenge.

作者信息

Müssig K, Wehrmann M, Horger M, Bares R, Häring H-U, Gallwitz B, Petersenn S

机构信息

Division of Endocrinology, Diabetology, Nephrology, Angiology, and Clinical Chemistry, Department of Internal Medicine, University Hospital of Tübingen, Tübingen, Germany.

出版信息

Exp Clin Endocrinol Diabetes. 2008 Oct;116(9):554-7. doi: 10.1055/s-2008-1062727. Epub 2008 Apr 1.

DOI:10.1055/s-2008-1062727

PMID:18523912

Abstract

BACKGROUND

Gastrinomas are the most frequent hormonally-active neuroendocrine tu-mours in patients with multiple endocrine neoplasia type 1 (MEN1).

CASE REPORT

We report on the diagnostic difficulties in a 62-year-old female patient with MEN1 and lymph node gastrinoma. At six and twelve months after resection of a lymph node gastrinoma, no signs of recurrence were observed. Basal and peak gastrin levels during secretin stimulation test were normalized. Extensive explorations, including gastrointesinal endoscopy, endoscopic ultrasonography, and Ga-68-DOTATOC-PET/CT, did not reveal a primary duodenal or pancreatic tumour.

CONCLUSION

Localization of gastrinomas in patients with MEN1 is challenging due to their small size, frequent duodenal location, and multiplicity. Therefore, while some studies support the existence of primary lymph node gastrinoma in patients with sporadic disease, this diagnosis should not be made in MEN1 patients. In both cases, however, extensive follow-ups are required.

摘要

背景

胃泌素瘤是1型多发性内分泌腺瘤病（MEN1）患者中最常见的具有激素活性的神经内分泌肿瘤。

病例报告

我们报告了一名62岁患有MEN1和淋巴结胃泌素瘤的女性患者的诊断困难情况。在切除淋巴结胃泌素瘤后的6个月和12个月，未观察到复发迹象。促胰液素刺激试验期间的基础和峰值胃泌素水平恢复正常。包括胃肠内镜检查、内镜超声检查和Ga-68-DOTATOC-PET/CT在内的广泛检查未发现十二指肠或胰腺原发性肿瘤。