Aggressive Multiple Endocrine Neoplasia with Multiorgan Invasion and Comprehensive Surgical Reconstruction: An Innovative Multidisciplinary Approach
DOI:
https://doi.org/10.70577/asce.v5i3.1086Keywords:
Multiple endocrine neoplasia; MEN1; MEN2; aggressive surgery; surgical reconstruction; multidisciplinary approach.Abstract
Introduction: Multiple endocrine neoplasia (MEN) syndromes constitute a group of rare hereditary disorders characterized by the development of tumors in multiple endocrine glands. MEN type 1 (MEN1), with autosomal dominant inheritance, is characterized by parathyroid, duodenopancreatic, and anterior pituitary tumors, while MEN2 is associated with medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. Aggressive forms, especially MEN2B, exhibit particularly malignant biological behavior with rapid progression and early metastases. Multiorgan invasion and liver metastases constitute the most life-threatening determinant in the evolution of these patients.
Objective: This review aims to synthesize current evidence on aggressive surgical management and comprehensive reconstruction in patients with multiple endocrine neoplasia with multiorgan invasion, analyzing innovative multidisciplinary approaches.
Methods: A systematic search was conducted in PubMed, Scopus, and Web of Science up to May 2026, following PRISMA guidelines. Original studies, systematic reviews, clinical guidelines, and expert consensus on surgical management of aggressive MEN and multiorgan reconstruction were included.
Results: Reviewed evidence supports early and aggressive surgery for duodenopancreatic neuroendocrine tumors in MEN1 patients to prevent the development of liver metastases. Resection of hepatic metastases in gastrinomas can achieve prolonged biochemical cure even in the presence of metastatic disease. Duodenopancreatic reoperations are frequently necessary and are not associated with increased perioperative morbidity in specialized centers. Distal pancreatectomy and pylorus-preserving pancreaticoduodenectomy constitute key procedures in pancreatic neuroendocrine tumor resection. Multidisciplinary management in referral centers, involving endocrinologists, surgeons, geneticists, and nuclear medicine specialists, is essential to optimize outcomes. Inferior vena cava reconstruction after oncologic resection has proven safe and durable, with 3-year patency rates exceeding 85% in high-volume centers.
Conclusion: Aggressive surgical approach and comprehensive reconstruction in patients with multiple endocrine neoplasia with multiorgan invasion require an innovative multidisciplinary approach in referral centers. Early surgery, planned reoperations, and complex vascular reconstruction can significantly improve survival and quality of life.
Downloads
References
Bartsch, D. K., Langer, P., Wild, A., Schilling, T., Celik, I., Rothmund, M., & Nies, C. (2000). Pancreaticoduodenal endocrine tumors in multiple endocrine neoplasia type 1: Surgery or
surveillance? Surgery, 128(6), 958-966.
https://doi.org/10.1067/msy.2000.109727
Bartsch, D. K., Fendrich, V., Langer, P., Celik, I., Kann, P. H., & Rothmund, M. (2005). Outcome of duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.
Annals of Surgery, 242(6), 757-764.
https://doi.org/10.1097/01.sla.0000189549.51913.d8
Cherner, J. A., & Sawyers, J. L. (1992). Benefit of resection of metastatic gastrinoma in multiple
endocrine neoplasia type I. Gastroenterology, 102(3), 1049-1053.
https://doi.org/10.1016/0016-5085(92)90196-6
Del Rivero, J., Gangi, A., Annes, J. P., Jasim, S., Keller, J., Lundholm, M. D., Silverstein, J. M.,
Vaghaiwalla, T. M., & Wermers, R. A. (2025). American Association of Clinical
Endocrinology consensus statement on management of multiple endocrine neoplasia
type 1. Endocrine Practice, 31(4), 403-418. https://doi.org/10.1016/j.eprac.2025.02.001
English, K. A., Pieterman, C. R. C., Marini, F., Lines, K. E., Cuny, T., Saulle, R., Shariq, O. A.,
Mitrova, Z., Castinetti, F., Waguespack, S. G., Brandi, M. L., Thakker, R. V., Valk, G.
D., & Minozzi, S. (2025). Multiple endocrine neoplasia type 1 (MEN1):
Recommendations and guidelines for best practice. The Lancet Diabetes &
Endocrinology, 13(8), 699-721. https://doi.org/10.1016/S2213-8587(25)00118-4
Furtado Neves, P. J., Simioni, A., Ishida, A. H., Gleisner, A. L., Mungo, B., McCarter, M. D.,
Malgor, E. A., & Malgor, R. D. (2026). Trends and outcomes of surgical reconstruction
of the inferior vena cava after oncologic resection. Journal of the American College of
Surgeons, 242(6), 1568-1578. (Sin DOI disponible)
Gaujoux, S., Pattou, F., Cadiot, G., & the AFCE and GTE Study Group. (2026). Surgical
management of Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1: An
AFCE and GTE cohort study. World Journal of Surgery. Advance online publication.
https://doi.org/10.1002/wjs.70303
Lopez, C. L., Waldmann, J., Fendrich, V., Langer, P., Kann, P. H., & Bartsch, D. K. (2011).
Long-term results of surgery for pancreatic neuroendocrine neoplasms in patients with
MEN1. Langenbeck's Archives of Surgery, 396(8), 1187-1196.
https://doi.org/10.1007/s00423-011-0828-1
Page, M. J., McKenzie, J. E., Bossuyt, P. M., Boutron, I., Hoffmann, T. C., Mulrow, C. D.,
Shamseer, L., Tetzlaff, J. M., Akl, E. A., Brennan, S. E., Chou, R., Glanville, J.,
Grimshaw, J. M., Hróbjartsson, A., Lalu, M. M., Li, T., Loder, E. W., Mayo-Wilson, E.,
McDonald, S., ... Moher, D. (2021). The PRISMA 2020 statement: An updated guideline
for reporting systematic reviews. BMJ, 372, n71. https://doi.org/10.1136/bmj.n71
Santucci, N., Caillot, J., Binquet, C., Goudet, P., Facy, O., & Gaujoux, S. (2026). Distal
pancreatectomy for neuroendocrine neoplasms in patients with multiple endocrine
neoplasia type 1. Journal of Visceral Surgery. Advance online publication.
https://doi.org/10.1016/j.jviscsurg.2026.03.001
Shimada, Y., Matsumoto, A., Takamura, K., Kobayashi, T., & Kinoshita, Y. (2025).
Laparoscopy-assisted total colectomy for progressive megacolon due to intestinal
ganglioneuromatosis in a young adult with multiple endocrine neoplasia type 2B: A case
report. International Journal of Surgery Case Reports, 136, 112028.
https://doi.org/10.1016/j.ijscr.2025.112028
Tsang, V. H. M., & the French Neuroendocrine Tumor Group (GTE). (2025). Codon 804 variants
in the RET proto-oncogene are associated with later-onset, moderately aggressive
medullary thyroid carcinoma in MEN2 patients. Journal of Investigative Medicine.
https://doi.org/10.1177/23247096251318345
Zhang, L., Ventin, M., Arya, S., et al. (2026). Surgical and oncologic outcomes after
pancreatectomy for pancreatic neuroendocrine tumor in multiple endocrine neoplasia
type 1 and von Hippel-Lindau syndrome: A large, multi-institutional, cohort study.
Journal of the American College of Surgeons, 242(6), 1602-1612. (Sin DOI disponible)
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 Paulo Sebastián Delgado Pinchao, Kevin Gabriel Correa Ortiz, Darwin Ismael Cajas Peláez, Erika Alexandra Uribe Maigua, Jaime Alejandro Cortés Morón
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Eres libre de:
- Compartir : copiar y redistribuir el material en cualquier medio o formato
- Adaptar : remezclar, transformar y desarrollar el material
- El licenciante no puede revocar estas libertades siempre y cuando usted cumpla con los términos de la licencia.
En los siguientes términos:
- Atribución : Debe otorgar el crédito correspondiente , proporcionar un enlace a la licencia e indicar si se realizaron cambios . Puede hacerlo de cualquier manera razonable, pero no de ninguna manera que sugiera que el licenciante lo respalda a usted o a su uso.
- No comercial : no puede utilizar el material con fines comerciales .
- CompartirIgual — Si remezcla, transforma o construye sobre el material, debe distribuir sus contribuciones bajo la misma licencia que el original.
- Sin restricciones adicionales : no puede aplicar términos legales ni medidas tecnológicas que restrinjan legalmente a otros hacer algo que la licencia permite.
