How Many Times Can One Go Back to the Drawing Board before the Accurate Diagnosis and Surgical Treatment of Glucagonoma?
Carmen Sorina Martin,
Ovidiu Dumitru Parfeni,
Liliana Gabriela Popa,
Mara Madalina Mihai,
Dana Terzea,
Vlad Herlea,
Mirela Gherghe,
Razvan Adam,
Osama Alnuaimi,
Valentin Calu,
Adrian Miron,
Silvius Negoita,
Cornelia Nitipir,
Simona Fica
Affiliations
Carmen Sorina Martin
Department of Endocrinology, Diabetes Mellitus, Nutrition and Metabolic Disorders, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Ovidiu Dumitru Parfeni
Department of Endocrinology, Diabetes Mellitus, Nutrition and Metabolic Disorders, Elias Emergency University Hospital, 011461 Bucharest, Romania
Liliana Gabriela Popa
Department of Oncological Dermatology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Mara Madalina Mihai
Department of Oncological Dermatology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Dana Terzea
OncoTeam Diagnostic, 030138 Bucharest, Romania
Vlad Herlea
Department of Pathology, Fundeni Clinical Institute, 022328 Bucharest, Romania
Mirela Gherghe
Department of Radiology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Razvan Adam
Department of Firs Aid and Disaster Medicine, Titu Maiorescu University, 040051 Bucharest, Romania
Osama Alnuaimi
Department of Radiology, Elias Emergency University Hospital, 011461 Bucharest, Romania
Valentin Calu
Department of Surgery, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Adrian Miron
Department of Surgery, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Silvius Negoita
Department of Anaesthesia and Intensive Care, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Cornelia Nitipir
Department of Oncology, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Simona Fica
Department of Endocrinology, Diabetes Mellitus, Nutrition and Metabolic Disorders, Carol Davila University of Medicine and Pharmacy, 020021 Bucharest, Romania
Glucagonomas are neuroendocrine tumors (NETs) that arise from the alpha cells of the pancreatic islets. They are typically slow-growing tumors associated with abnormal glucagon secretion, resulting in one or more non-specific clinical features, such as necrolytic migratory erythema (NME), diabetes, diarrhea, deep vein thrombosis, weight loss, and depression. Here, we report the case of a 44-year-old male with a history of diabetes mellitus, presenting with a pruritic and painful disseminated cutaneous eruption of erythematous plaques, with scales and peripheral pustules, misdiagnosed as disseminated pustular psoriasis and treated for 2 years with oral retinoid and glucocorticoids. During this period, the patient complained of weight loss of 32 kg and diarrhea and developed deep vein thrombosis. These symptoms, together with an inadequate response to therapy of the skin lesions, led to the reassessment of the initial diagnosis. Laboratory tests confirmed elevated plasma glucagon levels (>1000 pg/mL) and computed tomography (CT) scans revealed a 35/44 mm tumor in the pancreatic tail. Due to considerable disease complications and the COVID-19 pandemic, the surgical removal of the tumor was delayed for nearly 2 years. During this time, somatostatin analogue therapy efficiently controlled the glucagonoma syndrome and likely prevented tumor progression. As in other functional pancreatic NETs, the early clinical recognition of hormonal hypersecretion syndrome and the multidisciplinary approach are the keys for best patient management.