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

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas and account for less than 5% of all primary pancreatic malignancies. Included in this group are insulinomas, gastrinomas, glucagonomas and somatostatinomas. Collectively, these neoplasms are classified as functional PETs. When a PET is not associated with a clinical syndrome due to hormone oversecretion, it is referred to as a non-functional PET. Non-functionalPETs are pancreatic tumors with endocrine differentiation but lack a clinical syndrome of hormone hypersecretion. Although a pancreatic carcinoma shows aggressive biological behavior, a cutaneous metastasis from a pancreas carcinoma is rare. We report a case of a case of a cutaneous metastasis from an endocrine pancreatic carcinoma in a 50-year-old female that clinically manifested as a painful firm nodule on the back.
Female ; Gastrinoma ; Glucagonoma ; Humans ; Insulinoma ; Middle Aged ; Neoplasm Metastasis ; Pancreas ; Pancreatic Neoplasms ; Somatostatinoma

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas accounting for less than 5% of all primary pancreatic malignancies. Insulinomas, gastrinomas, glucagonomas and somatostatinomas is included in PET. PETs are usually classified into functioning and non-functioning tumors and presents with a range of benignity or malignancy. It is very important to accurate diagnose the PET location and to predict the benignity or malignancy of PET in terms of the treatment strategy, because PET have higher respectability, better response to chemotherapy and better prognosis compared to that of pancreatic adenocarcinoma. The utility and reliability of different imaging modalities depends on the characteristics of PETs, specifically their size. Functioning PET tend to be small (less than 2 cm), well circumscribed, homogeneous, and usually shown as strong enhancement on contrast enhanced CT or MR imaging. Non-functioning PET tend to be larger (4~10 cm), heterogeneous, and may contain the cystic areas of degeneration and necrosis. In this article, we present the various imaging findings of PET according to recent WHO classification.
Accounting ; Adenocarcinoma ; Gastrinoma ; Glucagonoma ; Insulinoma ; Magnetic Resonance Imaging ; Necrosis ; Pancreas ; Prognosis ; Somatostatinoma

glucagonoma is a rare islet alpha-cell tumor. We report a case of glucagonoma in a 55-year-old male patient with such clinical findings of necrolytic migratory erythema, diabetes mellitus, body weight loss, and anemia. CT examination found a space-occupying lesion in the pancreas, and an elevated serum glucagon level indicate the diagnosis of glucagonoma, which was confirmed postoperatively by pathological examination of the tumor tissue. A definite diagnosis of glucagonoma relies on pathological report, and so far no standard treatment strategy has been available for this tumor. Surgical resection is an effective means for treatment of glucagonoma.
Glucagonoma ; diagnosis ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Pancreatectomy ; Pancreatic Neoplasms ; diagnosis ; pathology ; surgery

OBJECTIVETo study the clinical features, diagnosis, and treatment of glucagonoma syndrome (GS) for providing clues for the recognition of this disorder in clinical practice.

METHODThe clinical and laboratory findings of four confirmed patients with GS were analyzed retrospectively.

RESULTSAll four patients had typical clinical manifestations of necrolytic migratory erythema (NME), elevated glucagon level in serum and hepatic metastasis. The skin rashes disappeared gradually and serum glucagon level decreased after operation and somatostatin treatment.

CONCLUSIONSNME is the most specific clinical finding of GS. Biopsy of the lesions, glucagon level in serum and various radiological examinations should be done in order to confirm the diagnosis. Surgical resection, chemotherapy, and somatostatin are the main therapies of GS.

Adult ; Female ; Glucagonoma ; diagnosis ; therapy ; Humans ; Male ; Middle Aged ; Pancreatic Neoplasms ; diagnosis ; therapy ; Retrospective Studies ; Somatostatin ; therapeutic use

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

PABCREATIC neuroendocrine tumours are uncommon neoplasms of the pancreas. They may cause a clinical syndrome due to hormone overproduction. Glucagonoma is a rare kind of pancreatic tumors. Here we report a case of glucagonoma. Hypercalcemia occurred when the patient underwent octreotide acetate long-acting release.
Antineoplastic Agents, Hormonal ; adverse effects ; Female ; Glucagonoma ; drug therapy ; Humans ; Hypercalcemia ; chemically induced ; Middle Aged ; Octreotide ; adverse effects ; Pancreatic Neoplasms ; drug therapy

Glucagoma is rare disease and has been reported only 100 cases so far worldwidely. The experience of this disease in our country is also lacking. The associated symptoms and signs can be characterized by necrolytic migratory erythematous lesion of the skin, hypoaminoacidemia, diabetes, weight loss, anemia, diarrhea, thrombocytopenia, glossitis. Among these, necrolytic migratory erythematous lesion of the skin is particulary considered as specific in this disease entity. In some cases, glucagonoma is diagnosed after metastasis to liver or bone marrow. Our patient initially diagnosed as having multiple metastatic adenocarcinoma of the liver. But subsequent development of the dermatologic manifestation enabled us to consider this rare disease. The constitutional symptoms and skin lesion abates rapidly at receiving Octreotide, somatostatin analogue, whereas no remarkable change observed after administration of the Zinc and aminoacid.
Adenocarcinoma ; Anemia ; Bone Marrow ; Diarrhea ; Erythema* ; Glossitis ; Glucagonoma* ; Humans ; Liver ; Neoplasm Metastasis* ; Octreotide ; Rare Diseases ; Skin ; Somatostatin ; Thrombocytopenia ; Weight Loss ; Zinc

OBJECTIVETo study the diagnosis and treatment of glucagonoma.

METHODSA retrospective review of glucagonoma cases was committed between June 1993 and July 2008 in Peking Union Medical College Hospital. It was measured by sex, age, misdiagnosis, clinical symptoms, laboratory data, imaging studies, diagnosis, treatment procedures and so on.

RESULTSThe tumors of eleven cases were found in the tail, and one case was in the head of the pancreas at the same time. Ten had solitary lesion, one had multiple lesions. The average diameter of the lesions was 3.9 cm. Nine patients had the metastasis out of pancreas and all of them had the liver metastasis. One case was a member of multiple endocrine neoplasia type 1 (MEN-1) syndromes. Eight patients being treated with operation had the detailed pathological reports. The glucagon was detected by immunohistochemistry and was positive in five patients. Six patients were pathologically malignant. Multimodal treatments included tumor resection, chemoembolization, treated with somatostatin analogues and (or) radionuclides and so on were applied to all patients.

CONCLUSIONSGlucagonoma is a rare pancreatic endocrine tumor. Radical tumor surgery is used as the first choice. Multimodal approach may improve the prognosis.

Adult ; Female ; Follow-Up Studies ; Glucagonoma ; diagnosis ; therapy ; Humans ; Male ; Middle Aged ; Pancreatic Neoplasms ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Treatment Outcome