Impaired insulin secretion and insulin resistance are the two main mechanisms leading to type 2 diabetes mellitus. Insulin exerts multiple effects upon target cells, especially skeletal muscle, liver, and adipose tissue. In general, insulin promotes storage of glucose and inhibits the breakdown of stored glycogen into glucose. The counter regulatory hormones glucagon, catecholamine, cortisol and growth hormone are released during hypoglycemia and under other stress conditions. These hormones have insulin-antagonistic effects both in the liver and in the peripheral tissues. A significant number of endocrine disorders is associated with varying degrees of glucose intolerance, with which sustained excess of these hormones is associated. Indeed, type 2 diabetes is frequently observed in patients with various hormonal diseases including acromegaly, Cushing syndrome, pheochromocytoma, hyperthyroidism, and glucagonoma. In particular, improvement of glycemic control following treatment for these hormonal diseases confirms a causal relationship between excess of these hormones and diabetes. In this review, there will be a discussion over these endocrine diseases in relation to diabetes.
Acromegaly ; Adipose Tissue ; Cushing Syndrome ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Endocrine System Diseases* ; Glucagon ; Glucagonoma ; Glucose ; Glucose Intolerance ; Glycogen ; Growth Hormone ; Humans ; Hydrocortisone ; Hyperthyroidism ; Hypoglycemia ; Insulin ; Insulin Resistance ; Liver ; Muscle, Skeletal ; Pheochromocytoma

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

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

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

Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. Most PNETs are indolent but have malignant potential. The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. Endocrine testing, imaging, and histological evidence are necessary to accurately diagnose PNETs. A 4-pronged aggressive treatment approach consisting of surgery, locoregional therapy, systemic therapy, and complication control has become popular in academic centers around the world. The optimal application of the multiple systemic therapeutic modalities is under development; efficacy, safety, availability, and cost should be considered when treating a specific patient. The clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including the novel Mahvash disease, are summarized.
Combined Modality Therapy ; Gastrinoma ; diagnosis ; therapy ; Glucagonoma ; diagnosis ; therapy ; Humans ; Insulinoma ; diagnosis ; therapy ; Multiple Endocrine Neoplasia Type 1 ; diagnosis ; therapy ; Neuroendocrine Tumors ; classification ; diagnosis ; therapy ; Pancreatic Neoplasms ; classification ; diagnosis ; therapy ; Vipoma ; diagnosis ; therapy ; von Hippel-Lindau Disease ; complications

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

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

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

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

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