Abstract
Multiple endocrine neoplasia (MEN) syndrome type I is a rare genetic syndrome characterized by the growth of endocrine tumors in different organs, mostly endocrine glands. Zollinger-Ellison and organic hyperinsulinism can be a part of this syndrome but it is very rare when they can be observed together in one patient. Clinical case of MEN I was described. Symptoms, presented by hypoglycemia and multiple ulcers, occurred in a 42-year-old woman. She suffered for 9 years, the diagnosis was multiple neuroendocrine tumors of the pancreas and duodenum, organic hyperinsulinism, Zollinger-Ellison syndrome. Robotic total duodenumpancreatectomy, splenectomy, distal gastrectomy with D2 lymphadenectomy was performed. Postoperative recovery was uneventful; patient was discharged after less than 2 weeks. Long-term survival showed no recurrence of the disease. In reviewed literature, we found no other cases of synchronous hypoglycemic and Zollinger-Ellison syndromes managed by total robotic duodenumpancreatectomy. Robotic technology can become a feasible and preferable method of treatment even in such difficult cases.
Similar content being viewed by others
Data Availability
The data are not available for public access because of patient privacy concerns, but are available from the corresponding author on reasonable request.
Abbreviations
- CT:
-
computed tomography
- IC unit:
-
intensive care unit
- HPF:
-
high presentative field
- MEN I:
-
multiple endocrine neoplasia type I
- NET:
-
neuroendocrine tumor
- WHO:
-
World Health Organization
References
Mason TH, Keigher HA. Intramedullary spinal neurilemmoma: case report. J Neurosurg. 1968;29(4):414–6. https://doi.org/10.3171/jns.1968.29.4.0414.
Yoshino T, Yoneda K. Laparoscopic resection of a retroperitoneal ancient schwannoma: a case report and review of the literature. Anticancer Res. 2008;28(5B):2889–91.
Propp JM, McCarthy BJ, Davis FG, Preston-Martin S. Descriptive epidemiology of vestibular schwannomas. Neuro-Oncology. 2006;8(1):1–11. https://doi.org/10.1215/S1522851704001097.
Jayaraj SM, Levine T, Frosh AC, Almeyda JS. Ancient schwannoma masquerading as parotid pleomorphic adenoma. J Laryngol Otol. 1997;111(11):1088–90.
Conti P, Pansini G, Mouchaty H, Capuano C, Conti R. Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature. Surg Neurol. 2004;61(1):34–44. https://doi.org/10.1016/S0090-3019(03)00537-8.
Li Q, Gao C, Juzi JT, Hao X. Analysis of 82 cases of retroperitoneal schwannoma. ANZ J Surg. 2007;77(4):237–40. https://doi.org/10.1111/j.1445-2197.2007.04025.x.
Chan PT, Tripathi S, Low SE, Robinson LQ. Case report–ancient schwannoma of the scrotum. BMC Urol. 2007;7:1. https://doi.org/10.1186/1471-2490-7-1.
Jindal T, Mukherjee S, Kamal MR, Sharma RK, Ghosh N, Mandal SN, et al. Cystic schwannoma of the pelvis. Ann R Coll Surg Engl. 2013;95(1):1–2. https://doi.org/10.1308/003588413X13511609956697.
Author information
Authors and Affiliations
Contributions
AGK and DSG designed the surgical concept and performed the surgery. SVB, ARK, RSD, and VIP carried out the acquisition of data and drafted the manuscript. OVP is responsible for the morphological part. AGK, DSG, SVB, ARK, and VIP revised the article. All authors read and approved the final manuscript.
Corresponding author
Ethics declarations
Ethics Approval and Consent to Participate
The present study was conducted in accordance with the ethical standards of our institution.
Consent for Publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Competing Interests
The authors declare that they have no competing interests.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
This article is part of the Topical Collection on Surgery
Rights and permissions
About this article
Cite this article
Kriger, A.G., Gorin, D.S., Berelavichus, S.V. et al. Multiple Functional Neuroendocrine Tumors of Pancreas and Duodenum Managed by Robotic Totally Duodenumpancreatectomy. SN Compr. Clin. Med. 2, 675–680 (2020). https://doi.org/10.1007/s42399-020-00272-6
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s42399-020-00272-6