Scientific PapersSurgical Strategy for Insulinomas in Multiple Endocrine Neoplasia Type I
Section snippets
Patients and Methods
From 1975 to 1996, 35 patients with histologically confirmed functioning insulinoma were surgically treated in our institution. Three patients (8.6%) were diagnosed to have MEN-I because of the presence of concomitant endocrinopathy and/or positive family history. There were 2 men and 1 woman with ages of 23, 29, and 41 years, respectively. Their clinical presentation, preoperative localization studies, surgical treatment, pathology of resected specimen, and postoperative outcome are reported.
Results
All patients presented with neuroglycopenic symptoms from 1 to 3 months. One patient had a family history of primary hyperparathyroidism, and another patient had a history of pituitary tumor before presentation. Hypoglycemia with inappropriately elevated insulin level were documented during overnight fasting or a 72-hour supervised fasting hypoglycemic test. Hypercalcemia with elevated parathyroid hormone was found at the time of presentation, and the diagnosis of MEN-I was suspected in all
Comments
Whenever insulinomas are multiple, MEN-I should be suspected. Approximately 4% to 10% of patients with hyperinsulinism will have MEN-I,1, 2, 3and 10% to 15% of MEN-I patients will acquire insulinomas as the only functional component of the pancreatic disease.3, 4, 5In contrast to sporadic disease, in which about 90% of patients will have a solitary insulinoma, about 90% of patients with MEN-I have multiple insulinomas.[5]In a review of 60 patients reported in the English literature, 76% of
References (11)
- et al.
Functioning insulinoma: incidence, recurrence and long-term survival of patients. A 60-year study
Mayo Clin Proc
(1991) Neuroendocrine tumors of the pancreas and duodenum
Curr Probl Surg
(1994)- et al.
Insulinomas associated with multiple endocrine neoplasia type Ithe need for a different surgical approach
Surgery
(1991) The surgical management of hyperparathyroidism and endocrine disease of the pancreas in the multiple endocrine neoplasia type I patient
J Intern Med
(1995)- et al.
Surgery for benign insulinomaan international review
World J Surg
(1990)
Cited by (34)
New therapies for patients with multiple endocrine neoplasia type 1
2021, Annales d'EndocrinologiePatient characteristics and clinical outcomes following initial surgical intervention for MEN1 associated pancreatic neuroendocrine tumours: A systematic review and exploratory meta-analysis of the literature
2019, PancreatologyCitation Excerpt :Fourteen studies [4,27,29,36,37,39–46,49] did not report a specific primary or secondary outcome measure (Supplementary Table 1). The entire data set was composed of sixteen non-comparative studies [4,5,16,36–48] with a median MINORS grading score of 7.5 out of 16 (range: 3–9) and eleven [6,21,27–35] comparative studies with a median MINORS score of 14 out of 24 (range: 11–20)20. All non-comparative studies failed to blind participants and to provide a prospective calculation of sample size.
Multiple endocrine neoplasia type 1
2019, Principles of Bone BiologyOperation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate
2017, Surgery (United States)Citation Excerpt :Indeed, in the literature,6-9,11-16 about 65% of MEN1 insulinomas reported throughout the past 20 years have been treated by DPR, while enucleation or minimal pancreatic resection was performed in 28%, and only 7% were treated by PD or total pancreatectomy. A comparison between enucleation and DPR regarding the successful cure of hypoglycemic/hyperinsulinemic syndrome and postoperative complications can be appreciated from 10 retrospective case series, all with a mean follow-up >9 years.6-9,11-16 The results clearly show that enucleation is followed by a lesser cure rate and greater risk of reoperation due to the persistence or recurrence of hypoglycemic/hyperisulinemic syndrome or oncologic progression of nonfunctioning PNETs or gastrinomas than after DPR (Table III).
Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: Review of literature
2011, Endocrine PracticeCitation Excerpt :Because of the potential surgical complications, pancreatoduodenectomy is rarely the standard of care for MEN 1-associated insulinoma (41,65). The overall morbidity and occurrence of pancreatic fistulas, however, are similar in comparison with use of other surgical procedures when experienced surgical teams are operating (20,31,54,61-67). Surgical treatment of MEN 1-related insulinomas is associated with persistence or recurrence of hypoglycemic syndrome in 4% to 40% of cases, in contrast with sporadic insulinomas (55,58,62,63).
Multiple Endocrine Neoplasia Type 1
2008, Principles of Bone Biology: Volume 1-2, Third Edition