Exceptional Response of Necrolytic Migratory Erythema to Octreotide in a Patient with Glucagonoma: A Case Report and Scoping Review

Introduction: Glucagonomas are rare neuroendocrine neoplasms (NENs) that often present with locoregional involvement at diagnosis and are characterized by variable survival outcomes and diverse therapeutic approaches. We present a clinical case of glucagonoma associated with necrolytic migratory erythema (NME), demonstrating an exceptional response to octreotide. Additionally, a scoping review was conducted to evaluate the efficacy of somatostatin analogues (SSAs) in managing NME. Methods: Through a meticulous search on Medline, we unearthed a collection of case reports and series that shed light on patients with glucagonoma and neuroendocrine tumors (NETs) who received treatment with somatostatin analogs (SSAs). A thorough extraction and analysis of pertinent data was conducted to deepen our understanding of these cases. Case presentation: A 66-year-old woman consulted with a four-year history of migratory, pruritic, and painful vesiculobullous skin lesions consistent with NME. Laboratory tests revealed serum glucagon levels exceeding 500 pg/mL, and Magnetic Resonance Imaging identified a pancreatic mass with hepatic metastases. Octreotide therapy resulted in the complete resolution of cutaneous lesions within four days of the first dose. She chose not to undergo surgery or other treatment options, and as a result, she passed away due to the progression of her disease. Results: Out of a total of 1,712 reviewed cases, 25 met the inclusion criteria. Among these cases, 56% of the patients were female, and the mean age at diagnosis was 59.74 years. Clinical improvement in NME was observed in 80% of patients treated with SSAs, with an average time to resolution of dermatological symptoms being 1.41 months. The median overall survival for these patients was 11 months. Conclusion: Although SSAs demonstrated notable efficacy in the resolution of NME, their impact on overall survival remains limited. These results emphasize the importance of ongoing research and innovation in treating NENs to improve long-term patient outcomes.