Background: This systematic review aimed to define the outcomes of different pancreatic resection procedures for multiple endocrine neoplasia type 1 (MEN1) associated pancreatic neuroendocrine neoplasms (pNENs).
Methods: A search of PubMed, MEDLINE and SCOPUS databases were performed in accordance with PRISMA guidelines.
Results: Twenty-seven studies including 533 patients undergoing initial pancreatic resection for MEN1 associated pNENs were included in this systematic review. Three hundred and sixty-six (68.7%) distal pancreatectomies (DP), 120 (22.5%) sole enucleations (SE) and 47 (8.8%) pancreaticoduodenectomies (PD) were identified. SE was associated with a higher rate of recurrence than DP (25/67, 37% vs 40/190, 21% respectively, P = 0.008) but a lower rate of endocrine insufficiency than PD (1/20, 5% vs 8/21, 38% respectively, P = 0.010). A meta-analysis of major pancreatic resections (PD or DP) vs SE in 15 studies showed that SE is associated with an increased rate of recurrence (Major resection 42/184, 23% vs SE 20/53, 38% RR 0.65 CI 0.43-0.96 P = 0.032) but reduced rate of postoperative endocrine insufficiency (Resection 37/93, 40% vs SE 0/24, 0% RR 7.37 CI 1.57-34.64 P = 0.008). Similarly, insulinomas and functional pNENs overall had lower rates of recurrence and reoperation with major resection. There was no difference in the reoperation rates or survival outcomes after SE compared with major pancreatic resections at follow-up (pooled overall mean duration: 85 months).
Conclusion: Major pancreatic resections for MEN1 associated pNENs have a lower risk of recurrence and a higher risk of postoperative endocrine insufficiency when compared to sole enucleation, but a similar rate of reoperation and survival.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.pan.2019.03.002 | DOI Listing |
Rev Med Chil
June 2024
Departamento de endocrinología, Hospital Clínico San Borja Arriarán, Santiago, Chile.
Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disease with an estimated prevalence of 2 per 100,000. This disease is caused by a mutation in the tumor suppressor gene MEN1, which is located on chromosome 11 and codifies the menin protein. It is characterized by a predisposition of parathyroids, enteropancreatic, and anterior pituitary tumors, affecting the quality of life and lifespan of those who have the disease.
View Article and Find Full Text PDFInt J Surg Case Rep
December 2024
Department of Otorhinolaryngology, Aarhus University Hospital, Aarhus N, Denmark.
Introduction And Importance: Primary hyperparathyroidism (PHPT) is a frequent complication to multiple endocrine neoplasia type 1 (MEN1), presenting challenges due to increased risk of multi-gland disease and recurrence post parathyroidectomy (PTX). This case report examines the management of PHPT in a MEN1 patient, emphasizing possible benefits from intraoperative parathyroid autofluorescence imaging (AF).
Case Presentation: A 21-year-old woman with MEN1 presented with mild hyperparathyroidism symptoms in 2014.
Int J Surg Case Rep
December 2024
Department of Gastrointestinal and General Surgery, Kathmandu Medical College and Teaching Hospital, Kathmandu, Nepal.
Introduction And Importance: Insulinomas are rare pancreatic neuroendocrine neoplasms with an incidence of one to four cases per million annually and a 5 % to 10 % association with hereditary multiple endocrine neoplasia type-1. While most insulinomas are benign and well-encapsulated, approximately 6 % may have malignant potential. Intraoperative localization remains a vital component of treatment, often facilitated by modern imaging techniques like intraoperative ultrasound and fluorescence modalities.
View Article and Find Full Text PDFEndocr Oncol
January 2024
Department of Molecular Medicine and Pathology, School of Medical Sciences, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.
Although the gene has a long-standing association with cancer, its mechanisms of action remain incompletely understood, acting both as a tumour suppressor in neuroendocrine tumours and as an oncogene in leukaemia. The best-characterised isoform of the encoded protein, MENIN, is the 610-amino acid MENIN isoform 2. We hypothesise that some of the complexity of biology can be attributed to a currently unappreciated contribution of different MENIN isoforms.
View Article and Find Full Text PDFPancreatology
November 2024
Pancreatobiliary Cancer Center, Yonsei Cancer Center, Severance Hospital, Seoul, South Korea; Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Yonsei University College of Medicine, Seoul, South Korea. Electronic address:
Background: Pancreatic neuroendocrine tumors (PNETs) are the leading cause of death related to multiple endocrine neoplasia type 1 (MEN1). Previous studies have linked certain mutations in the MEN1 gene and loss of interactions with MENIN's functional partners to the mortality or aggressiveness of PNETs. This study aimed to evaluate the genotype-phenotype correlations of MEN1-related PNETs in Korean patients and to summarize the treatment outcomes comprehensively.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!