With the incidence of pancreatic diseases increasing year by year, pancreatic hyperglycemia, as one of the common complications, is gradually gaining attention for its impact on the skin health of patients.

This was the case of an elderly female with clinical manifestations of necrolytic migratory erythema, "three more and one less," diabetes mellitus, hypertension, anemia, hypoproteinemia, and other syndromes, which had been misdiagnosed as eczema. Abdominal computed tomography showed a pancreatic caudal space-occupying lesion, and the magnetic resonance scanning of the epigastric region with dynamic enhancement and diffusion-weighted imaging suggested a tumor of the pancreatic tail, which was considered to be a neuroendocrine tumor or cystadenoma. The patient was referred to a more equipped hospital for laparoscopic pancreatic tail resection. Post-surgery diagnosis revealed a neuroendocrine tumor in the tail of the pancreas. To date, the patient's general condition is good, and she is still under close follow-up.

Necrolytic migratory erythema can be induced by endocrine system tumors or endocrine metabolic abnormalities, with complex clinical manifestations, difficult diagnosis, and easy misdiagnosis by dermatologists. The initial treatment principles in dermatology include symptomatic supportive therapy and effective drugs to relieve skin lesions. After clarifying the etiology of glucagonoma, comprehensive treatment in collaboration with endocrinologists, general surgeons, and oncologists can help provide individualized treatment for patients and improve their prognosis.

Functioning pancreatic neuroendocrine neoplasms other than insulinomas and gastrinomas (rf-pNENs) are exceptionally rare tumours. Thus, their characteristics and long-term prognosis have not been well defined. This article aims to present data and experience from a single institution concerning this topic. Twelve of 216 (5.5%) patients with pNENs operated between 2002 and 2022 in the ENETS Centre of Excellence Marburg had rf-pNENs and their data were retrospectively analysed. We identified three vasoactive intestinal polypeptide producing pNENs, four glucagonomas and five calcitoninomas. The tumour could be visualised by preoperative imaging in all 12 patients, and six patients had distant metastases at the time of diagnosis. The tumour was located in the pancreatic tail in nine patients and the median tumour size was 82 (range 12-220) mm. Eleven patients underwent tumour resections (two robotic, nine conventional), nine of which were R0. After a median follow-up of 75 (range 1-247) months, six patients were alive, five of whom had no evidence of disease. All patients who remained disease-free had an initial R0 resection of the primary tumour and no initial liver involvement. This study sheds light on the distinct characteristics and outcomes of these exceedingly rare tumours, offering insights for improved understanding and management.

Glucagonoma is a hormonally active rare pancreatic neuroendocrine tumour causing an excess of glucagon. This is a narrative review based on a multidisciplinary approach of the tumour. Typically associated dermatosis is necrolytic migratory erythema (NME) which is most frequently seen at disease onset. Insulin-dependent diabetes mellitus, depression, diarrhoea, deep vein thrombosis are also identified, as parts of so-called 'D' syndrome. Early diagnosis is life saving due to potential aggressive profile and high risk of liver metastasis. NME as paraneoplastic syndrome may be present for months and even years until adequate recognition and therapy; it is remitted after successful pancreatic surgery. Thus the level of practitioners' awareness is essential. If surgery is not curative, debulking techniques may improve the clinical aspects and even the outcome in association with other procedures such as embolization of hepatic metastasis; ablation of radiofrequency type; medical therapy including chemotherapy, targeted therapy with mTOR inhibitors such as everolimus, PRRT (peptide receptor radiotherapy), and somatostatin analogues (including combinations of medical treatments). Increased awareness of the condition involves multidisciplinary practitioners.

Glucagonoma is a rare neuroendocrine tumor of the pancreas. We present the case of a young female patient who presented with the major clinical manifestations of glucagonoma syndrome.

The major clinical manifestations of glucagonoma syndrome are described in a 44-year-old, female patient. Beyond glucagonoma, the patient also displayed deep venous thrombosis, depression, diabetes, and necrolytic migratory erythema. We discuss the difficulty of treatment of patients with glucagonoma due to the low prevalence of the disorder, scarcity of medical evidence, lateness of diagnosis with liver metastases in most cases, and poor response to chemotherapy with high rates of relapse after surgery. In this case, pancreatectomy and hepatic lobectomy followed by somatostatin analogue therapy was the chosen treatment strategy.

The clinical findings were pancreatic and hepatic masses, proximal deep venous thrombosis, depression, diabetes, and necrolytic migratory erythema. The patient also had elevated levels of glucagon. Pancreatectomy and right hepatic lobectomy were performed and confirmed the glucagonoma.

Our case adds new knowledge about glucagonoma which is important due to the low incidence of the disease and the particular characteristics of the syndrome.

A 54-year-old Caucasian male with history of hypertension, hyperlipidemia, insulin-dependent diabetes mellitus, and chronic skin rash of 4 years presented to the emergency department with worsening rash and weight loss. Physical examination revealed diffuse erythematous rash, skin ulceration, bullae with associated paresthesia in the lower extremities, trunk, bilateral upper extremities, and palms and soles. A computed tomography (CT) scan with contrast showed a large, heterogenously enhancing pancreatic mass measuring 9.4 × 3.8 cm with surrounding low-attenuation soft tissue thickening. Blood tests showed hemoglobin A1C of 10.0%. Glucagon level was elevated to 2,178 (normal < 80 pg/dl). Endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) from the pancreatic mass was suggestive of pancreatic endocrine tumor. The tumor cells were positive for synaptophysin, chromogranin, CD56, and pan-cytokeratin with focal positivity for glucagon, suggestive of glucagonoma. The patient underwent distal pancreatectomy along with splenectomy and cholecystectomy. The glucagon level normalized to 25 pg/dl within a week of tumor resection, and during his 6-week outpatient follow up, skin rash had completely resolved.

Glucagonoma is a rare neuroendocrine tumor of the pancreas. Glucagonoma syndrome is often misdiagnosed as other skin lesions by clinicians due to a typical clinical sign of necrolytic migratory erythema (NME) with severe erythematous rash.

A 48-year-old female patient was admitted to our department because she presented with unclear recurrent severe erythematous rash. The patient was diagnosed as skin disease.

Histopathologic examination revealed a pancreatic glucagonoma. Immnohistochemical staining of tumor tissue was positive for glucagon.

The distal pancreatectomy plus splenectomy was performed in 2017.

The skin lesions disappeared after surgery. She was followed up and showed no recurrence until now.

Clinicians should consider the diagnosis of glucagonoma according to the typical initial symptoms. Early diagnosis is very important to provide a better prognosis. A multidisciplinary approach is effective in patients with unresectable metastatic tumors.