Research Information System
ENDOCRINOLOGY RESEARCH AND PRACTICE, vol.29, no.2, pp.149-152, 2025 (ESCI, TRDizin)
Glucagonomas, a rare subset of pancreatic neuroendocrine neoplasms (PNETs) originating from alpha cells of the pancreas, secrete glucagon and represent less than 10% of PNETs, with an incidence of 0.01-0.1 per million population per year. Often metastatic at diagnosis, glucagonomas commonly present with dermatological symptoms, notably necrolytic migratory erythema (NME), leading to diagnostic delays. Effective management necessitates a multidisciplinary approach. We report a case of a 26-year-old woman with itchy, coin-sized lesions, initially misdiagnosed as erythema multiforme and treated with topical corticosteroids. Recurrence of symptoms, weight loss, night sweats, nail changes, and gastrointestinal issues led to further investigation. Elevated glucagon (700 pg/mL) and chromogranin A levels (>700 ng/mL) confirmed glucagonoma. Imaging revealed multiple hepatic and pancreatic lesions. The treatment included lanreotide, peptide receptor radionuclide therapy (PRRT), distal pancreatectomy, and splenectomy. Despite 6 PRRT cycles and additional chemotherapy, recurrent liver metastases necessitated ongoing management and potential liver transplantation.This case emphasizes the importance of early NME recognition and a multidisciplinary approach involving dermatology, endocrinology, pathology, radiology, surgery, nuclear medicine, medical oncology, and interventional radiology for effective glucagonoma management.