Primary Nodal Gastrinomas

Abstract

Background.—The term primary lymph node gastrinoma was first used to describe a group of patients with gastrin-producing tumors present in lymph nodes located in a well-defined anatomic region. The patients had no known primary tumors in the pancreas or gastrointestinal tract and had disease-free survival for up to 18 years. The anatomic region in question has a triangular shape that extends from the cystic and common bile ducts to the second and third portion of the duodenum and the neck and body of the pancreas. The term gastrinoma triangle was coined to identify the area; in addition, it was postulated that lymph nodes located in the gastrinoma triangle normally contained neuroendocrine cells capable of secreting gastrin and other neuropeptides. From its inception, the postulate became the subject of controversy. Design.—To extend previous observations, we examined the lymph nodes located in the gastrinoma triangle of 20 autopsy cases for the presence of neuroendocrine cells, as determined by immunohistochemistry, using antibodies to a panneuroendocrine substance (eg, synaptophysin) and a specific neuropeptide (eg, gastrin). Scanning for positive cells was performed by 2 observers (M.E.H. and M.C.C.). We compared the findings in these lymph nodes with lymph nodes obtained from axillary and inguinal dissections during surgical procedures. Results.—In all, 417 lymph nodes were studied. Five of the 20 gastrinoma triangle cases contained synaptophysin reactive cells, whereas 3 had gastrin reactive cells. None of the axillary and inguinal lymph nodes contained neuroendocrine cells. Conclusion.—Our findings support the hypothesis of entrapment of neuroendocrine cells during development and the presence of primary nodal gastrinomas.

Management of Primary Lymph Nodal Gastrinoma With Liver Metastases Resulting in Zollinger-Ellison Syndrome

“…Gastrinoma with Zollinger-Ellison syndrome is very rare in childhood and may originate from different locations: pancreatic parenchyma, lymphatic tissue, and submucosal duodenum within gastrinoma triangle. [1][2][3][4] Gastrinoma in lymph node makes it challenging to discriminate between primary and metastatic disease. 68 Ga-DOTA-peptide PET/CT is the investigation of choice for localization of primary and metastatic disease in gastrinoma and other NETs.…”

“…Existence of primary lymph nodal gastrinomas in several case reports/series without recurrence after complete surgical resection in long-term surveillance supported the hypothesis that sporadic gastrinoma arise from stem cells from the ventral pancreatic bud that gets dispersed into lymph tissue through migration within triangle area and stains for chromogranin, synpatophysin, and specific neuropeptide (eg, gastrin). Gastrinoma with Zollinger-Ellison syndrome is very rare in childhood and may originate from different locations: pancreatic parenchyma, lymphatic tissue, and submucosal duodenum within gastrinoma triangle [1][2][3][4]. Gastrinoma in lymph node makes it challenging to discriminate between primary and metastatic disease.68 Ga-DOTA-peptide PET/CT is the investigation of choice for localization of primary and metastatic disease in gastrinoma and other NETs 5,6.…”

Sporadic, Primary Lymph Node Gastrinoma

“…Pathologic data suggest that pancreatic lymph nodes contain neuroendocrine cells that have the same potential as pancreatic neuroendocrine cells and, in theory, can give rise to primary nodal gastrinomas. 5 Etiology of these primary lymph node gastrinomas remains controversial particularly because it may alter the patient's therapy. If the tumor arises primarily in a lymph node then enucleation may be curative, and this has been described.…”

Primary Lymph Node Malignant Gastrinoma with Metastasis to Virchow's Node: A Case Report

“…The gastrinoma triangle, a term coined in 1984 by Dr. Stabile, is bounded by the common bile duct, the second and third portions of the duodenum, and the pancreas 2 . Analysis by both Herrmann et al 4 and Perrier et al 1 have immunohistochemically examined patients without known gastrinomas and found that lymph nodes from this triangle stain for gastrin, chromogranin A, pancreatic protein, and synaptophysin, specific makers for neuroendocrine cells. It is believed that nests of cells become entrapped within these lymph nodes when the ventral pancreatic bud rotates during fetal life 3 .…”

“…Although controversial, some authors believe that primary lymph node gastrinomas exist. [1][2][3][4] In support of this theory, 40% to 50% of patients without known primary lesions can be biochemically cured with peripancreatic lymphadenectomy. 2 We present a case of a patient with refractory peptic ulcer disease and elevated serum gastrin levels who on an octreotide scan demonstrated uptake near the pancreas as well as a focus within the left supraclavicular neck.…”