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

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.

Necrolytic migratory erythema: an important visual cutaneous clue of glucagonoma

Objective: Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. Methods: In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi’an Jiaotong University. Results: The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23-51). The average time from onset of symptoms to diagnosis of glucagonoma was 14 months (range 2-36 months). All the patients visited dermatology firstly for necrolytic migratory erythema (NME) 7/7 (100%), other presenting symptoms included: diabetes mellitus (DM) 4/7 (57%), stomatitis 2/7 (28%), weight loss 4/7 (57%), anemia 4/7 (57%), diarrhea 1/7 (14%), DVT1/7 (14%). Plasma glucagon levels were increased in all patients (range 216.92–3155 pg/mL), and declined after surgery. Imaging studies revealed that four of seven patients had liver metastasis. Six of seven patients received surgical resection, and all of them received somatostatin analogue therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Conclusion: Our data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma. Once diagnosed, surgery and somatostatin analogues are effective for symptom relief and tumor control.

Elevated Glucagon in a Patient with Necrolytic Acral Erythema: A Case Report and Review of the Literature

Necrolytic acral erythema (NAE) is a relatively newly described dermatologic disease that is often associated with hepatitis C virus (HCV). Oral zinc therapy is a successful treatment; however, therapy is often delayed due to misdiagnosis. There are limited reports of NAE in the literature. This paper presents a case of NAE in a 68-year-old male with untreated HCV, whose NAE was diagnosed and treated as recurrent cellulitis for 12 years. He had low serum zinc and elevated serum glucagon levels. Elevated glucagon is not often reported in NAE, but the patient’s CT abdomen was negative, ruling out glucagonoma and necrolytic migratory erythema. He improved with oral zinc replacement and was referred to the hepatology department for HCV treatment. This paper additionally presents a review of the literature for NAE cases.

A case of delayed diagnosis of Necrolytic Migratory Erythema

Necrolytic Migratory Erythema (NME) is a rare cutaneous paraneoplastic manifestation of glucagonoma. We report a case of a woman with a 6-year history of delayed diagnosis of Glucagonoma. This case highlights the atypical clinical features of NME which makes the diagnosis difficult.

Third reported case of rare necrolytic migratory erythema associated with bacteraemia due to severe zinc deficiency after revisional Roux-En-Y gastric bypass: case report and literature review

Introduction Obesity is a risk factor for zinc deficiency. After bariatric surgery, non-compliance to diet/vitamin supplements, surgical complications leading to vomiting/diarrhea, poor follow-up and malabsorption can precipitate or exacerbate pre-existing zinc deficiency. Case report We report a patient with rare necrolytic migratory erythema associated with bacteraemia due to severe zinc deficiency after revisional Roux-en-Y gastric bypass (following primary laparoscopic sleeve gastrectomy). Conclusion Bariatric teams should screen patients before bariatric surgery for nutritional deficiencies and continue surveillance of their nutritional status after surgery. They should maintain a high index of suspicion for zinc deficiency in patients with skin rash after bariatric surgery. Level of evidence Level V, case report.