Zollinger ellison syndrome due to primary nodal gastrinoma located outside the limits of the conventional gastrinoma triangle: report of a case
Keywords:Primary nodal gastrinoma, Zollinger ellison syndrome, Gastrinoma triangle
Primary nodal gastrinoma is a rare entity and the diagnosis is often contemplative when no other non - nodal primary site can be identified despite thorough investigations and operative exploration. Here we report one such case wherein a primary nodal gastrinoma was diagnosed as an entity of exclusion. Additionally, the location of the disease outside the confines of the conventional gastrinoma triangle further contributes to the rarity of the presentation. A young male patient had presented to us with history of multiple operations in the past for recurrent upper abdominal pain presumably consequential to peptic ulcer disease viz a trucal vagotomy and gastrojejunostomy, duodenal ulcer perforation surgery and a cholecystectomy. CT scan and endoscopic USG showed a preaortic calcified node located outside the limits of the gastrinoma triangle. A raised serum gastrin level and an endoscopic guided FNAC confirmed the diagnosis of a gastrinoma. A 68 Ga-DOTANOC PET CT revealed an exclusive nodal uptake with no discenable primary lesion. Normalization of gastrin levels after removal of the involved pre-aortic node further pointed to the diagnosis of primary nodal gastrinoma. A high index of clinical suspicion is warranted especially in a history of multiple surgeries for recurrent upper abdominal pain and location of the lesion outside the confines of the ‘Gastrinoma Triangle’ should not be deterrent for the diagnosis.
Shao QQ, Zhao BB, Dong LB, Cao HT, Wang WB. Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies. World J Gastroenterol. 2019 Aug 28;25(32):4673-81.
Arnold WS, Fraker DL, Alexander HR, Weber HC, Norton JA, Jensen RT. Apparent lymph node primary gastrinoma. Surgery. 1994 ;116(6):1123-9.
Toppino M, Corno F, Serra GC, Mistrangelo M, Guglielminotti P, Bolla C, et al. Extrahepatic gastrinoma. Report of a clinical case. Minerva Gastroenterol Dietol. 1996;42(3):161-7.
Anderson MA, Carpenter S, Thompson NW, Nostrant TT, Elta GH, Scheiman JM. Endoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of the pancreas. Am J Gastroenterol. 2000;95(9):2271-7.
Gabriel S, Garrigue P, Dahan L, Castinetti F, Sebag F, Baumstark K, et al. Prospective evaluation of 68 Ga-DOTATATE PET/CT in limited disease neuroendocrine tumours and/or elevated serum neuroendocrine biomarkers. Clin Endocrinol (Oxf). 2018;89(2):155-63.
Poultsides GA, Huang LC, Chen Y, Visser BC, Pai RK, Jeffrey RB, et al. Pancreatic neuroendocrine tumors: radiographic calcifications correlate with grade and metastasis. Ann Surg Oncol. 2012;19(7):2295-303.
Chen Y, Deshpande V, Ferrone C, Blaszkowsky LS, Parangi S, Warshaw AL, et al. Primary lymph node gastrinoma: A single institution experience. Surgery. 2017;162(5):1088-94.
Delcore R, Cheung LY, Friesen SR. Outcome of lymph node involvement in patients with the Zollinger-Ellison syndrome. Ann Surg. 1988;208(3):291-8.
Bhagavan BS, Slavin RE, Goldberg J, Rao RN. Ectopic gastrinoma and Zollinger-Ellison syndrome. Hum Pathol. 1986;17(6):584-92.
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(5):650-7.
Odelowo OO, Nidiry JJ, Zulu SH. Primary lymph node gastrinoma: a case report. J Natl Med Assoc. 2003;95(2):168-71.
Jaenigen B, Kayser G, Steinke B, Thomusch O. Five-Year Long-Term Followup of a Primary Lymph node Gastrinoma: Is a Pancreaticoduodenectomy Justified? Case Rep Med. 2009;2009:762791.
Harper S, Carroll RW, Frilling A, Wickremesekera SK, Bann S. Primary lymph node gastrinoma: 2 cases and a review of the literature. J Gastrointest Surg. 2015;19(4):651-5.
Farley DR, van Heerden JA, Grant CS, Thompson GB. Extrapancreatic gastrinomas. Surgical experience. Arch Surg. 1994;129(5):506-11.
Zogakis TG, Gibril F, Libutti SK, Norton JA, White DE, Jensen RT, et al. Management and outcome of patients with sporadic gastrinoma arising in the duodenum. Ann Surg. 2003;238(1):42-8.
Delcore R, Friesen SR. Zollinger-Ellison syndrome. A new look at regression of gastrinomas. Arch Surg. 1991;126(5):556-8.
Thompson NW, Vinik AI, Eckhauser FE. Microgastrinomas of the duodenum. A cause of failed operations for the Zollinger-Ellison syndrome. Ann Surg. 1989;209(4):396-404.
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? Mayo Clinic Pancreatic Surgery Group. Surgery. 1995;118(6):957-65.
Herrmann ME, Ciesla MC, Chejfec G, DeJong SA, Yong SL. Primary nodal gastrinomas. Arch Pathol Lab Med. 2000;124(6):832-5.
Singh D, Lal SB, Sood A, Gupta R, Kumar R, Vashishta RK, et al. Management of Primary Lymph Nodal Gastrinoma With Liver Metastases Resulting in Zollinger-Ellison Syndrome. Clin Nucl Med. 2019;44(1):36-9.
Stabile BE, Morrow DJ, Passaro E Jr. The gastrinoma triangle: operative implications. Am J Surg. 1984;147(1):25-31.