A 42-year-old Filipino female was admitted due to weight loss and recurrent abdominal pain over the past ten years. In 2010, she was diagnosed to have disseminated PTB associated with a liver mass. After 1 year of anti-TB treatment, lung condition was treated but her liver mass has enlarged. She also developed diarrhea, diabetes, and skin lesions with biopsy results showing Necrolytic Migratory Erythema (NME). CT scan showed liver and pancreatic tumors that were biopsied revealing a neuroendocrine tumor. Blood glucagon level was elevated. She was treated as a case of glucagon-secreting tumor with liver metastases with Everolimus and Octreotide. After 3 months of treatment, she gained weight, the skin lesions improved, and the liver mass decreased in size. Many of the initial symptoms of glucagonoma are nonspecific and subtle. As a result, glucagonoma is often diagnosed relatively late in the course of the disease. NME, the characteristic skin lesion of the glucagonoma syndrome, is often the clue that leads to the correct diagnosis.
Glucagonoma ; Necrolytic Migratory Erythema

A 58-year-old Malay female with underlying diabetes mellitus, presented with chronic skin lesions, associated with weight loss and anemia. There were erosive, scaling skin lesions over the extremities, gluteal region and perioral area. Skin biopsy histopathological examination revealed Necrolytic Migratory Erythema (NME). A CT scan of the abdomen revealed a pancreatic neck and body tumor with possible liver metastases. She was successfully treated with subcutaneous somatostatin and underwent distal pancreatectomy with wedge resection of liver nodule.
Glucagonoma ; Necrolytic Migratory Erythema ; Glucagon ; Somatostatin

Necrolytic migratory erythema (NME) is a typical cutaneous manifestation of glucagonoma syndrome. The entire syndrome consists of NME, glucose intolerance, weight loss, anemia, glossitis, diarrhea, and increased glucagon levels. We herein report a patient with glucagonoma syndrome who was diagnosed as having NME. A 48-year-old male presented with a 2-month history of painful erythematous, desquamative, erosive papules and plaques on both lower extremities. Histological examination revealed an intraepidermal cleft, the presence of vacuolated, pale epidermal cells, and necrosis in the upper epidermis. His glucagon level was 2650 pg/ml, with the upper limit of a normal range being 250 pg/ml. The patient was treated with octreotide, and showed an improvement of the skin eruption with normalization of the glucagon level within 4 weeks.
Anemia ; Diarrhea ; Epidermis ; Glossitis ; Glucagon ; Glucagonoma* ; Glucose Intolerance ; Humans ; Lower Extremity ; Male ; Middle Aged ; Necrolytic Migratory Erythema* ; Necrosis ; Octreotide ; Reference Values ; Skin ; Weight Loss

Necrolytic migratory erythema is a cutaneous reaction pattern with specific histopathologic features that is typically associated with a functioning pancreatic islet cell neoplasm such as a glucagonoma. However, cases without. glucagonoma have also been reported, such as, liver cirrhosis, chronic pancreatitis, celiac sprue etc. Other clinical features include anemia, glossitis and weight loss. We report a case of neccrolytic migratory erythema induced by a pancreatic insufficiency without glucagonoma. A 43-year-old male was seen at our department because of rnultiple erythematous, desquamative and erosive patches on the whole body for 1 month. He also had weight loss(25Kg) and stomatitis. Seven years previously he had a Whipple's procedure for a pancreatic head rupture. The laboratory data showed a low protein and zinc level, and high glucagon level. Histopathologic findings of the case showed epidermal edema and pallor, and superficial epiderrnal necrosis. The patient improved progressively after intravenous infusion of amino acids with a pancreatic enzyme supplementation.
Adult ; Amino Acids ; Anemia ; Celiac Disease ; Edema ; Erythema ; Exocrine Pancreatic Insufficiency* ; Glossitis ; Glucagon ; Glucagonoma ; Head ; Humans ; Infusions, Intravenous ; Islets of Langerhans ; Liver Cirrhosis ; Male ; Necrolytic Migratory Erythema* ; Necrosis ; Pallor ; Pancreatitis, Chronic ; Rupture ; Stomatitis ; Weight Loss ; Zinc

Pancreatic neuroendocrine tumors (PNET) are rare, with approximately 2.2 in 1,000,000 people affected annually. In the classification of neuroendocrine tumors, glucagonomas are a functional PNET and comprise 1.6% of PNET. Glucagonoma syndrome is a paraneoplastic syndrome that is characterized by necrolytic migratory erythema, weight loss, anemia, and diabetes mellitus. Metastatic disease at presentation is common, but is often limited to the liver and regional lymph nodes. Sunitinib malate improves the progression-free and overall survival of PNET. This report presents a 45-year-old Asian woman with prolonged neutropenia after sunitinib treatment of a glucagonoma with multiple hepatic metastases. The severity of the neutropenia after the sunitinib treatment fluctuated from grade 1 to 4 repeatedly, with a non-febrile pattern. Ultimately, the patient did not recover from the neutropenia, even after stopping the sunitinib.
Anemia ; Asian Continental Ancestry Group ; Diabetes Mellitus ; Female ; Glucagonoma ; Humans ; Indoles ; Liver ; Lymph Nodes ; Necrolytic Migratory Erythema ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors ; Neutropenia ; Pancreas ; Paraneoplastic Syndromes ; Pyrroles ; Weight Loss

Necrolytic migratory erythema is a rare, distinctive, necrotizing cutaneous eruption usually associated with glucagonoma, an alpha-cell tumor of the pancreas. Other clinical features include ane- mia, diabetes mellitus and weight loss. However, it also occurrs without the pancreatic glucagonoma. So this rare condition is called pseudoglucagonoma syndrome, and has been described in association with chronic hepatic diseases, malabsorption with villous atrophy, chronic pancreatitis, celiac sprue, hypoproteinemia and odontogenic infection. We report a case of pseudoglucagonoma syndrome induced by malnutrition without glucagonoma. A 28-year-old Korean female was referred to our department complaining of multiple erythematous, desquamative, crusted and erosive patches on the extremities, and diffuse scalp alopecia for 5 years. She also had perianal erosion, angular cheilitis without glossitis, amenorrhea, axillary and pubic hair loss, and weight loss. Six years previously she had had Whipples operation for a pancreatic solid and papillary neoplasm. The laboratory data showed hypoalbuminemia, essential fatty acid deficiency, diabetes mellitus and slightly high levels of zinc and glucagon. A skin biopsy showed irregular acanthosis, epidermal edema and pallor, dyskeratosis, areas of superficial epidermal necrosis and vascular dilatation of the papillary dermis. The patient improved dramatically after intravenous hyperalimentation and oral intake of mediurn chain triglyceride. For 2.5 years she has taken medium-chain triglycerides without any other treatment, and no side effects on recurrence has been observed.
Adult ; Alopecia ; Amenorrhea ; Atrophy ; Biopsy ; Celiac Disease ; Cheilitis ; Dermis ; Diabetes Mellitus ; Dilatation ; Edema ; Extremities ; Female ; Glossitis ; Glucagon ; Glucagonoma ; Hair ; Humans ; Hypoalbuminemia ; Hypoproteinemia ; Malnutrition ; Necrolytic Migratory Erythema* ; Necrosis ; Pallor ; Pancreas ; Pancreatitis, Chronic ; Parenteral Nutrition, Total ; Recurrence ; Scalp ; Skin ; Triglycerides* ; Weight Loss ; Zinc

Glucagonomas are rare pancreatic tumors of islet ahpha-2 cells. Less than 430 cases have been reported worldwide and 210 cases are malignant tumors. In generally, the tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema of the skin, weight loss, non-insulin-dependent diabetes mellitus, anemia, cheliosis, stomatitis, and an increased thrombotic tendency. Since pancreatic glucagonomas are predominantly located in the tail and findings of radiographic or sonographic examination can remain unspecific, patients often present already metastasis when diagnosis is first established, and can be difficult to differentiate from the other pancreatic tumors. We report the case of a 59-year-old woman with an malignant glucagonoma of the pancreas infiltrating already the spleen and presenting metastatic lesion in perirenal lymph nodes, and that the tumor was not assocated with the characteristic skin rash. The pateint with a past history of a diabetes mellitus and hypertension for 9 years was admitted with cramp-like left lower abdominal pain, watery diarrhea, and nausea. A solid tumor of tail of the pancreas revealed by ultrasonography and abdominal computed tomography and distal pancreatectomy, radical nephrectomy, and splenectomy were performed. Immunohistochemial examination of the tumor did show glucagon-reactive tissue and electron microscopy revealed many secretory granules, 180 to 300 nm in diameter in granulated cells. After pancreatic tumor resection, the patient had normalization of plasma glucagon and blood sugar.
Abdominal Pain ; Adenoma, Islet Cell ; Anemia ; Blood Glucose ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Diagnosis ; Diarrhea ; Exanthema ; Female ; Glucagon ; Glucagonoma* ; Humans ; Hypertension ; Lymph Nodes ; Microscopy, Electron ; Middle Aged ; Nausea ; Necrolytic Migratory Erythema ; Neoplasm Metastasis ; Nephrectomy ; Pancreas* ; Pancreatectomy ; Plasma ; Secretory Vesicles ; Skin ; Spleen ; Splenectomy ; Stomatitis ; Tail ; Ultrasonography ; Weight Loss