Multiple endocrine neoplasia syndrome

Abstract

Background. Multiple endocrine neoplasia type 1 is a rare inherited disease that combines heterogeneous autosomal inherited disorders, the pathogenesis of which is based on hyperplasia or tumor transformation of several neuro- endocrine glands. In this pathology, treatment protocols are insufficient. Surgical treatment, suppressive or replacement therapy, radiation and chemotherapy are mainly used.
 We presented a successful experience of combined radiotherapy of multiple pancreatic insulinoma as the main clinical manifestation of the syndrome of multiple endocrine neoplasia. The combination of intra-tissue brachytherapy and megavolt remote therapy in TFD, isoeffective 50 Gy, allowed to achieve reliable control of hypoglycemic attacks within 2 years of follow-up.
 Purpose – to acquaint the medical community with successful experience of combined radiation treatment of multiple pancreatic insulinoma as a main clinical manifestation of the multiple endocrine neoplasia syndrome.
 Materials and Methods. Clinical case of patient L., 30 years old, who received combined radiation therapy for clinically significant manifestations of hormonally active multiple insulinoma of the pancreas in the Department of Radiological Oncology of State Organization «Grigoriev Institute for Medical Radiology and Oncology of the National Academy of Medical Sciences of Ukraine».
 Results. In May 2019, palliative contact brachytherapy was performed on the MULTISOURCE device. Total focal dose (TFD) for cells in the pancreas GENUS 3 Gy 5 times a week, TFD 18 Gy. At the end of brachytherapy, attacks of hypoclycemia were stopped. In March 2020, remote radiation therapy was performed on a linear accelerator CLINAC-600C (6MeV) in the mode of classical fractionation (single focal dose (SFD) 1.8 Gy 5 times a week), TFD for the entire volume of the pancreas 30.6 Gy. In May 2021, CT scan showed that the process in the pancreas was stabilized, but in connection with the resumption of hypoglycemia and the emergence of dyshormonal osteopathy, a course of remote radiation therapy for the entire pancreas, SFD 1.8 Gy, TFD 20 Gy simultaneously with the introduction of bisphosphonates.
 Conclusions. Our patient demonstrates type I syndrome of multiple neo- plasms, neuroendocrine tumor of pancreas G1 T3N0M0 IIB stage. Due to multi-stage combined radiation therapy for 2 years it is possible to achieve stabilization of the process and reliable control of hypoglycemic attacks, which indicates the effectiveness of this method as an alternative to surgery in unresectable cases.