Lymph Node Gastrinoma in Multiple Endocrine Neoplasia Type 1 – a Diagnostic Challenge

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

Key words

primary lymph node gastrinoma - MEN1 - neuroendocrine tumor - ⁶⁸Ga-DOTATOC - somatostatin receptor - sst - gastrin - secretin stimulation test

References

• 1 Arnold WS, Fraker DL, Alexander HR, Weber HC, Norton JA, Jensen RT. Apparent lymph node primary gastrinoma.  Surgery. 1994;  116 1123-1129 , ; discussion 1129–1130
• 2 Bartsch DK, Fendrich V, Langer P, Celik I, Kann PH, Rothmund M. Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.  Ann Surg. 2005;  242 757-764 , ; discussion 764–766
• 3 Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C. et al . Guidelines for diagnosis and therapy of MEN type 1 and type 2.  J Clin Endocrinol Metab. 2001;  86 5658-5671
• 4 Frucht H, Norton JA, London JF, Vinayek R, Doppman JL, Gardner JD, Jensen RT, Maton PN. Detection of duodenal gastrinomas by operative endoscopic transillumination. A prospective study.  Gastroenterology. 1990;  99 1622-1627
• 5 Herrmann ME, Ciesla MC, Chejfec G, DeJong SA, Yong SL. Primary nodal gastrinomas – an immunohistochemical study in support of a theory.  Arch Pathol Lab Med. 2000;  124 832-835
• 6 Huai JC, Zhang W, Niu HO, Su ZX, MacNamara JJ, Machi J. Localization and surgical treatment of pancreatic insulinomas guided by intraoperative ultrasound.  Am J Surg. 1998;  175 18-21
• 7 Jensen RT, Niederle B, Mitry E, Ramage JK, Steinmuller T, Lewington V, Scarpa A, Sundin A, Perren A, Gross D, O’Connor JM, Pauwels S, Kloppel G. Frascati Consensus Conference . Gastrinoma (duodenal and pancreatic).  Neuroendocrinology. 2006;  84 173-182
• 8 Kann PH, Kann B, Fassbender WJ, Forst T, Bartsch DK, Langer P. Small neuroendocrine pancreatic tumors in multiple endocrine neoplasia type 1 (MEN1): least significant change of tumor diameter as determined by endoscopic ultrasound (EUS) imaging.  Exp Clin Endocrinol Diabetes. 2006;  114 361-365
• 9 Lamberts SW, Bakker WH, Reubi JC, Krenning EP. Somatostatin-receptor imaging in the localization of endocrine tumors.  N Engl J Med. 1990;  323 1246-1249
• 10 Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT. Possible primary lymph node gastrinoma: occurrence, natural history, and predictive factors: a prospective study.  Ann Surg. 2003;  237 650-657 , ; discussion 657–659
• 11 Norton JA. Surgical treatment and prognosis of gastrinoma.  Best Pract Res Clin Gastroenterol. 2005;  19 799-805
• 12 Pereira PL, Roche AJ, Maier GW, Huppert PE, Dammann F, Farnsworth CT, Duda SH, Claussen CD. Insulinoma and islet cell hyperplasia: value of the calcium intraarterial stimulation test when findings of other preoperative studies are negative.  Radiology. 1998;  206 703-709
• 13 Perrier ND, Batts KP, Thompson GB, Grant CS, Plummer TB. An immunohistochemical survey for neuroendocrine cells in regional pancreatic lymph nodes: A plausible explanation for primary nodal gastrinomas?.  Surgery. 1995;  118 957-966
• 14 Rockall AG, Reznek RH. Imaging of neuroendocrine tumours (CT/MR/US).  Best Pract Res Clin Endocrinol Metab. 2007;  21 43-68
• 15 Thompson NW. Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both.  J Intern Med. 1998;  243 495-500
• 16 Veldhuis JD, Norton JA, Wells Jr SA, Vinik AI, Perry RR. Surgical versus medical management of multiple endocrine neoplasia (MEN) type I.  J Clin Endocrinol Metab. 1997;  82 357-364
• 17 Wu PC, Alexander HR, Bartlett DL, Doppman JL, Fraker DL, Norton JA, Gibril F, Fogt F, Jensen RT. A prospective analysis of the frequency, location, and curability of ectopic (nonpancreaticoduodenal, nonnodal) gastrinoma.  Surgery. 1997;  122 1176-1182

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