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The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease.
Surgery. 2004 Dec; 136(6):1205-11.S

Abstract

BACKGROUND

The management of multiple endocrine neoplasia, type 1 (MEN-1) pancreatoduodenal neuroendocrine neoplasms (NENs) is controversial. An aggressive surgical approach is intended to control the functional syndromes and malignant potential for nodal or distant metastasis.

METHODS

The results of treating 39 patients with MEN-1 pancreatoduodenal NENs over a 35-year period are available from chart reviews and patient interviews. This study focuses on pattern of disease, disease recurrence, and long-term functional outcomes.

RESULTS

Between 1967 and 2003, 39 patients ages 19 to 58 years (mean age, 37) had abdominal operations for their pancreatoduodenal NENs: 26 with Zollinger-Ellison syndrome, 4 with hypoglycemia, 3 with both Zollinger-Ellison syndrome and hypoglycemia, and 6 with nonfunctional neoplasms. Fifteen of these 39 patients had malignant disease on initial abdominal operation; 24 of 39 patients have not required abdominal reoperation, 17 of whom have available follow-up data. Of these 17 patients, 11 have biochemical evidence of disease recurrence (increased serum concentrations of gastrin, insulin, or pancreatic polypeptide), while 6 have no biochemical evidence of recurrence. A total of 30 abdominal reoperations were performed in 15 patients; 14 of 15 patients undergoing 1 or more reoperations developed evident malignant disease by their most recent operation. Nine of 13 reoperative patients with follow-up data have evidence of disease recurrence. Functional outcomes available in 20 patients showed that 10 patients require insulin and that 6 require oral hypoglycemic medications. Ninety percent have no abdominal pain or nausea/vomiting, while 4 are unable to return to work secondary to this disease.

CONCLUSIONS

Treatment of MEN-1 pancreatoduodenal NENs is met with frequent recurrence and some treatment-related morbidity and mortality. Most patients (22 of 39) eventually demonstrated malignant growth, but, with this strategy, few died of this disease.

Authors+Show Affiliations

Division of Endocrine Surgery, Department of Surgery, University of Michigan, Taubman Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109-0331, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

15657577

Citation

Hausman, Mark S., et al. "The Surgical Management of MEN-1 Pancreatoduodenal Neuroendocrine Disease." Surgery, vol. 136, no. 6, 2004, pp. 1205-11.
Hausman MS, Thompson NW, Gauger PG, et al. The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease. Surgery. 2004;136(6):1205-11.
Hausman, M. S., Thompson, N. W., Gauger, P. G., & Doherty, G. M. (2004). The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease. Surgery, 136(6), 1205-11.
Hausman MS, et al. The Surgical Management of MEN-1 Pancreatoduodenal Neuroendocrine Disease. Surgery. 2004;136(6):1205-11. PubMed PMID: 15657577.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease. AU - Hausman,Mark S,Jr AU - Thompson,Norman W, AU - Gauger,Paul G, AU - Doherty,Gerard M, PY - 2005/1/20/pubmed PY - 2005/3/25/medline PY - 2005/1/20/entrez SP - 1205 EP - 11 JF - Surgery JO - Surgery VL - 136 IS - 6 N2 - BACKGROUND: The management of multiple endocrine neoplasia, type 1 (MEN-1) pancreatoduodenal neuroendocrine neoplasms (NENs) is controversial. An aggressive surgical approach is intended to control the functional syndromes and malignant potential for nodal or distant metastasis. METHODS: The results of treating 39 patients with MEN-1 pancreatoduodenal NENs over a 35-year period are available from chart reviews and patient interviews. This study focuses on pattern of disease, disease recurrence, and long-term functional outcomes. RESULTS: Between 1967 and 2003, 39 patients ages 19 to 58 years (mean age, 37) had abdominal operations for their pancreatoduodenal NENs: 26 with Zollinger-Ellison syndrome, 4 with hypoglycemia, 3 with both Zollinger-Ellison syndrome and hypoglycemia, and 6 with nonfunctional neoplasms. Fifteen of these 39 patients had malignant disease on initial abdominal operation; 24 of 39 patients have not required abdominal reoperation, 17 of whom have available follow-up data. Of these 17 patients, 11 have biochemical evidence of disease recurrence (increased serum concentrations of gastrin, insulin, or pancreatic polypeptide), while 6 have no biochemical evidence of recurrence. A total of 30 abdominal reoperations were performed in 15 patients; 14 of 15 patients undergoing 1 or more reoperations developed evident malignant disease by their most recent operation. Nine of 13 reoperative patients with follow-up data have evidence of disease recurrence. Functional outcomes available in 20 patients showed that 10 patients require insulin and that 6 require oral hypoglycemic medications. Ninety percent have no abdominal pain or nausea/vomiting, while 4 are unable to return to work secondary to this disease. CONCLUSIONS: Treatment of MEN-1 pancreatoduodenal NENs is met with frequent recurrence and some treatment-related morbidity and mortality. Most patients (22 of 39) eventually demonstrated malignant growth, but, with this strategy, few died of this disease. SN - 0039-6060 UR - https://www.unboundmedicine.com/medline/citation/15657577/The_surgical_management_of_MEN_1_pancreatoduodenal_neuroendocrine_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0039606004005410 DB - PRIME DP - Unbound Medicine ER -