Gastrinomas represent the most common funtional pancreaticoduodenal neuroendocrine tumor in MEN1 patients, however, there is lack of consensus regarding appropriate timing and extent of operation for these patients. An optimal strategy has been difficult to define due to the uncommon nature of the disease, inconsistency of operative approaches, absence of controlled studies, limited follow-up, and incomplete understanding of the natural history of the disease. It has been demonstrated that the majority of MEN1-associated gastrinomas are located in the duodenum and often occur concomitantly with non-gastrin producing pancreatic neoplasms. Therefore, evaluation of the duodenum for removal of these tumors is critical to the operative strategy, regardless of the extent of pancreatectomy planned. This chapter reviews outcomes of studies focused on MEN1 patients with hypergastrinemia with particular attention to the incidence of gastrinoma-associated nodal metastases and the rationale for performance of a regional lymphadenectomy at the time of operative exploration. Although there are relatively few studies examining this issue, available data demonstrate a high occurrence of gastrinoma lymph node metastases in MEN1 patients. Furthermore, these are often micrometastases and commonly used preoperative imaging modalities have a low sensitivity for detecting this disease. Collectively, the available evidence suggest that in addition to removal of the primary tumor(s), a formal anatomically based regional lymphadenectomy may result in more durable reduction of gastrin hypersecretion and offer potential long-term oncologic benefit (GRADE B).
Multiple endocrine neoplasia type 1 Gastrinoma Hypergastrinemia Lymph node metastases
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