Pancreatic tumors in multiple endocrine neoplasia type 1: Clinical presentation and surgical treatment

Abstract

Among 33 patients with endocrine pancreatic tumors due to multiple endocrine neoplasia type 1 (MEN-1), 19 (58%) patients had hypergastrinemia, 7 (21%) patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning lesions. At least one gross tumor was found in all patients undergoing pancreatic surgery, including those with negative localization studies prior to operation. The patients also had additional macroscopic tumors as well as numerous microadenomas, and the lesions frequently were positive for immunostaining with multiple hormones, mainly pancreatic polypeptide, insulin, glucagon, and somatostatin. Duodenal endocrine lesions were found in 4 of 5 investigated patients and stained with gastrin and somatostatin antibodies. Distal, mainly subtotal pancreatic resection, was performed in 18 patients, eventually combined with caput tumor enucleation or duodenotomy, while a few patients underwent only tumor enucleation or a Whipple procedure. The long-term outcome of operation was most favorable in patients with hyperinsulinism; only 1 patient had clinical recurrence. Patients with hypergastrinemia experienced only transitory lowering of serum gastrin values after pancreatic surgery and 47% of them had or developed metastases. Such tumor spread was seen in 57% of the patients with non-functioning lesions. Nine patients died from progressive tumor disease during follow-up. Consistent with previous studies, we found that surgery is indicated in MEN-1 patients with hyperinsulinism even if a lesion is not visualized by radiology. In addition, these indications should be extended to also include patients with only biochemical markers of disease, including elevations of gastrin, as these indicate the presence of gross tumors.(ABSTRACT TRUNCATED AT 250 WORDS)

Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis

“…In smaller series from the last decade, distant metastases are reported in 6-22% (Bartsch et al 2005, Davi et al 2011, Lopez et al 2011, whereas in a report from 1992 distant metastases were observed in 57% (Grama et al 1992). Mean tumor size in this latter series was 6.7 cm (Grama et al 1992).…”

“…Reports regarding the prognostic significance of pancreatic tumor size vary. No relation between tumor size and metastases, malignancy or overall survival is found in several series reporting on MEN1-related dpNETs (hormonally active and NF; Grama et al 1992, Lowney et al 1998, Lairmore et al 2000, Bartsch et al 2005, Kouvaraki et al 2006, Lopez et al 2011. In the subset of NF-pNETs, larger tumor size was related to a higher rate of metastases and a decreased overall survival (Triponez et al 2006a,b).…”

Neuroendocrine Tumors of the Pancreas

“…This may in part explain the shift in relative incidence from F-PNETs to NF-PNETs in recent years. Whereas in early single-center series, NF-PNETs comprise 18%-66% of all neuroendocrine tumors of the pancreas [27][28][29][30][31][32], recent large, singlecenter or epidemiologic studies classify 68%-90% as NFPNETs [21,26,[33][34][35].…”

The Impact of Clinical and Genetic Screenings on the Management of the Multiple Endocrine Neoplasia Type 1

“…3,13,14 Gastrinoma in MEN1 is the most prevalent functioning PET, seen in 30% to 75% of MEN1 cases 5 . The gastrinomas in MEN1 mostly occur in the duodenum (up to 90%) and is frequently multifocal, in contrast to unifocal sporadic gastrinomas.…”