Primary lymph node gastrinoma: a case report and review of the literature

Abstract

Background: Gastrinoma is a rare form of neuroendocrine neoplasm. The presence of a primary lymph node localization of gastrinoma is a much debated and controversial topic in the literature, as regards whether these cases represent metastatic disease from an as yet unidentified primary tumor, or the de novo occurrence of a gastrinoma in a lymph node.

Case presentation: We report the case of a 24-year-old male with intense epigastric pain treated at the beginning with high dose proton pump inhibitors. Further workup with CT and subsequent laparotomy revealed a single peripancreatic lymph node. Histological examination highlighted a well-differentiated neuroendocrine tumor.

Conclusion: This case underlines that the primitive lymph node gastrinoma is a distinct nosological entity with a precise location in the context of rare neuroendocrine tumors that should be considered when specific symptoms are associated with the identification of isolated lymph nodes, after excluding any possible primitive locations of neoplastic localization.

Keywords: Gastrinoma; NEN (neuroendocrine neoplasm); Primitive lymph node.

Fig. 1

Computed tomography (CT) scan of the abdomen. a, b Axial sections from abdominal contrast CT demonstrating a 13 mm lesion (arrow) adjacent to the second part of the duodenum, corresponding to an area of abnormal octreotide uptake

Fig. 2

a Bisected lymph node almost fully superseded by a neuroendocrine tumor. b Histopathology of NET showing subtotal replacement of the lymph node by the NET (HE staining; magnification × 100) immunohistochemistry showed strongly positive staining for synaptophysin (c) and weakly positive for gastrin (d) (magnification × 100)