How Many Times Can One Go Back to the Drawing Board before the Accurate Diagnosis and Surgical Treatment of Glucagonoma?

Abstract

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.

An Overview of Clinical Manifestations of Dermatological Disorders in Intensive Care Units: What Should Intensivists Be Aware of?

“…It occurs in conjunction with a pancreatic islet alpha-cell tumor (glucagonoma). It can also occur in hepatic disease and malabsorption syndrome (pseudo glucagonoma syndrome) [ 119 , 120 ]. The sacrum, perineum, buttocks, intertriginous and periorificial areas, distal extremities, and face are all affected by a radially distributed, extremely erythematous rash with scaly necrotic plaques.…”

A review of functional pancreatic neuroendocrine tumors: Exploring the molecular pathogenesis, diagnosis and treatment

“…[63] Elevated circulating glucagon levels, in conjunction with other clinical and imaging findings, help to establish the diagnosis. [63] However, given the overlap of glucagon levels with other pancreatic disorders, a complementary approach using additional biomarkers, such as CgA and pancreatic polypeptide, has been explored to enhance diagnostic accuracy. [63] The key biomarker utilized in the diagnostic evaluation of somatostatinomas is somatostatin itself, whose excessive secretion contributes to the characteristic somatostatinoma syndrome, comprising diabetes mellitus, cholelithiasis, diarrhea, and weight loss.…”

“…[63] However, given the overlap of glucagon levels with other pancreatic disorders, a complementary approach using additional biomarkers, such as CgA and pancreatic polypeptide, has been explored to enhance diagnostic accuracy. [63] The key biomarker utilized in the diagnostic evaluation of somatostatinomas is somatostatin itself, whose excessive secretion contributes to the characteristic somatostatinoma syndrome, comprising diabetes mellitus, cholelithiasis, diarrhea, and weight loss. [64] Elevated circulating levels of somatostatin aid in establishing the diagnosis, although the overlap of somatostatin levels with other pancreatic disorders necessitates the integration of additional biomarkers for enhanced diagnostic precision.…”

“…[63] Elevated circulating glucagon levels, in conjunction with other clinical and imaging findings, help to establish the diagnosis. [63] However, given the overlap of glucagon levels with other pancreatic disorders, a complementary approach using additional biomarkers, such as CgA and pancreatic polypeptide, has been explored to enhance diagnostic accuracy. [63]…”

The New Radiolabeled Peptide 99mTcEDDA/HYNIC-TOC: Is It a Feasible Choice for Diagnosing Gastroenteropancreatic NETs?

“…GEP-NETs may present as hormonally functional or nonfunctional tumors and may have distinct clinical features based on their site of origin. Additionally, they present a specific tissue characteristic that can be targeted by molecular imaging and peptide receptor radionuclide therapy (PRRT), such as expression of different receptors, especially somatostatin receptors (SSTRs), particularly SSTR2 and SSTR5 [ 6 , 7 , 8 , 9 , 10 ].…”