Endocrine Abstracts

Introduction: Glucagonomas are rare neuroendocrine tumours (NET). Glucagonoma syndrome is often misdiagnosed as other skin lesions by clinicians due to typical clinical sign of necrolytic migratory erythema (NME). In this article, we report a metastatic glucagonoma case diagnosed at adult age with distinct features of the disease.

Observations: We present a case of a 28-year-old female patient. The initial rash appeared in early 2019 and was mostly on lower extremities by the ankles, which were red patches with irregular borders, intact and ruptured vesicles, and crust formation. The patient was prescribed several topical glucocorticoids and antimicrobials, but the rash was persistent and spread over her proximal lower extremities, back, chest, face and thus became generalized. By this 15 month interval the patient also developed symptoms of weight loss of 10 kg, nausea-vomiting, night sweats and abdominal-back pain. A skin biopsy was performed in 2020 and necrolytic migratory erythema was diagnosed. The patient then admitted to investigate glucagonoma. Admission laboratory measurements demonstrated no hyperglycemia but elevated blood glucagon levels (>700 ng/ml). Initial screening demonstrated a distal pancreatic lesion and overspread metastatic liver involvement. A liver biopsy was performed and the diagnosis of glucagonoma was established. The patient was initiated lanreotide 90 mg q28d and the dose was uptitrated to 120 mg q28d after 1 year. In this time interval generalized skin rash disappeared completely, and other symptoms improved. Then, she had recieved six doses of peptide receptor radionuclide therapy. Control imaging demonstrated that partial metabolic response. And, she underwent distal pancreatectomy and liver metastasectomy. Pathology report demonstated metastatic glucagonoma. Treatment of lanreotide 120 mg per/28 day was continued postoperatively. Imaging after 3 months showed multiple metastatic lesions over the liver. A chemotherapy regimen of capecitabine, temozolomide and bevacizumab was initiated. She is still receiving treatment and is in good performance condition.

Discussion: Clinicians should consider glucagonoma diagnosis based on the typical initial symptoms. An early diagnosis is critical for a better prognosis. Treatment options include somatostatin analogs, peptide receptor radioligand therapy, cytotoxic chemotherapy and surgery. In patients with unresectable metastatic cancers, a multidisciplinary approach is effective.