Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Of the rare pancreatic endocrinomas, glu-cagonomas, either with or without diabetico-dermatogenic syndrome (DDS), are probably third in frequency after insulinomas and gastrinomas. This study was carried out to evaluate the present status of glucagonoma/DDS in a statistically reliable number of cases and to provide precise information to investigators actively working in this particular field of research. A total of 407 cases of glucagonoma were collected from the international literature and evaluated according to characteristic clinicopathologic features. Findings were: (1) The incidence of DDS was 57.2% (233/407). (2) The tail of the pancreas was predominantly involved, in 53.7% (213/397). (3) One-third of the tumors (80 of 276 for whom size was recorded; 29.0%) measured 20 mm or less. (4) Metastases occurred in 51.4% (209/407) and malignant tumors in 60.7% (247/407). (5) Multiplicity occurred in 11.8% (48/407), and associated multiple endocrine neoplasia type 1 in 13. 0% (53/407). (6) In the patients with DDS, the rates of hyperglucagonemia, necrolytic migratory erythema, diabetes mellitus, loss of weight, hypo-aminoacidemia, or anemia, as representative constituents of DDS, were all higher than rates in the overall series (P < 0.01). (7) The 10-year survival rate in the 233 patients with DDS was 51.6% in those with metastases and 64.3% in those without metastases (P < 0.001).
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PMID:Glucagonomas/diabetico-dermatogenic syndrome (DDS): a statistical evaluation of 407 reported cases. 988 Jul 81

A 59-yr-old man with multiple pancreatic tumors is presented. Previously, he had undergone left adrenalectomy for primary hyperaldosteronism and left nephrectomy for renal cell carcinoma at the ages of 39 and 55 yr, respectively. This time, 3 yr after removal of renal cancer, two solid lesions in the pancreas associated with hyperglucagonemia were detected. Under a diagnosis of pancreatic metastasis from renal cell carcinoma or islet cell tumor of the pancreas, distal pancreatectomy with splenectomy and enucleation of the tumor in the pancreas head were performed. Microscopically, a glucagonoma, measuring 2.3 mm in diameter, was detected among five pancreatic metastases from renal cell carcinoma. Four years after surgery, the patient remains well, without signs of recurrence despite multiple pancreatic metastases. This is the first report of such a rare combination consisting of aldosterone-secreting adrenal adenoma and glucagonoma.
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PMID:A rare combination consisting of primary hyperaldosteronism and glucagonoma. 1023 26

Glucagonoma, a rare neuroendocrine pancreatic tumour, is frequently malignant and often accompanied by hepatic metastases. Our aim was to consider the different treatments of metastatic glucagonoma to the liver and their results. A case of glucagonoma with metachronous, small, multiple and bilobar liver metastases is reported. Combined treatment with octreotide and hepatic arterial chemoembolization was applied with good results in terms of symptom relief, plasma glucagon levels and regression of hepatic metastases. Survival rates were also improved. Based on our experience, glucagonoma with metachronous, multiple, diffuse and bilobar hepatic metastases should be treated with octreotide plus hepatic arterial chemoembolization with improved outcome and prognosis.
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PMID:Treatment of metastatic glucagonoma to the liver: case report and literature review. 1042 77

Glucagonoma and somatostatinoma are tumors which produce the respective hormone. When these peptides are also secreted into the circulation the clinical syndromes are characterized by the signs and symptoms due to hormone overproduction. In case of the glucagonoma-syndrome diabetes and typical skin lesions are dominating while patients with the somatostatinoma syndrome have diabetes frequently associated with steatorrhea. Surgical resection of the tumor and its metastases as far as possible is the therapy of choice. For symptomatic relief and inhibition of the growth of the metastases interferon-a and somatostatin analogues can be employed.
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PMID:[Glucagonoma--somatostatinoma]. 1044 13

Glucagon-producing neuroendocrine tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema, non-insulin-dependent diabetes, weight loss, anemia, glossitis, and an increased thrombotic tendency. Most glucagonomas are solid and arise in the body or tail of the pancreas. We report two cases of cystic glucagonoma, one found incidentally in an asymptomatic patient and one in a patient with weight loss and diabetes but no rash. In the first patient, distal pancreatectomy and splenectomy were curative, whereas the second patient continued to exhibit elevated serum glucagon levels and symptoms of glucose intolerance in the absence of demonstrable metastases. Cystic glucagonoma is a unique variant of classic glucagonoma and should be considered in the differential diagnosis of cystic pancreatic neoplasms.
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PMID:Cystic glucagonoma: A rare variant of an uncommon neuroendocrine pancreas tumor. 1045 11

In patients with gastro-enteropancreatic neuroendocrine tumours the localization of all the neoplastic lesions and an accurate staging of the diseases have important therapeutic implications. Somatostatin receptor scintigraphy with In-111 pentatreotide has proved to be useful in detecting gastro-enteropancreatic tumours; however, the role of abdominal single photon emission computed tomography has not yet been definitively established. In a series of 52 patients with gastro-enteropancreatic tumours (9 non-functioning islet cell carcinomas, 4 insulinomas, 3 somatostatinomas, 2 VIPomas, 1 glucagonoma and 33 carcinoids) we compared somatostatin receptor scintigraphy with the results of computed tomography and magnetic resonance imaging performed within one month. Four and 24-hour total body planar images and 4-hour abdominal single photon emission computed tomography were acquired after the i.v. injection of approximately 250 MBq of In-111 pentatreotide. Only abdominal localizations were considered: planar scans detected 16 extrahepatic lesions in 13 patients and 54 liver sites in 21 patients; single photon emission computed tomography visualized 31 extrahepatic lesions and 89 liver metastases in 27 and 28 patients, respectively; computed tomography and magnetic resonance imaging detected 11 extrahepatic lesions in 10 patients and 73 liver sites in 21 patients. In-111 pentatreotide single photon emission computed tomography was the only imaging method able to localize tumoural lesions in 13 patients; all these localizations were then histologically verified. The scintigraphic positivity did not depend on the site or on the presence of hormonal hypersecretions. In conclusion, our results indicate that single photon emission computed tomography is more sensitive than planar images and computed tomography/magnetic resonance imaging in detecting abdominal gastro-enteropancreatic tumours and their metastases; it is able to increase both the number of visualized lesions and that of patients with positive findings. Single photon emission computed tomography is particularly useful in patients in whom tumoural lesions have not been already localized; it should be the first imaging modality in patients with gastro-enteropancreatic tumours: its initial use will result in more information and proper management.
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PMID:Single photon emission computed tomography procedure improves accuracy of somatostatin receptor scintigraphy in gastro-entero pancreatic tumours. 1060 27

Duodeno-pancreatic biochemically polyfunctional endocrine tumour is a well known entity. Usually, only one hormone is responsible for the clinical features. We report a case of aggressive combined glucagonoma and gastrinoma tumour without metastases, causing respectively diabetic ketoacidosis and fulminant peptic ulcer, and death. Occasional patients can present with clinical features of both glucagonoma and gastrinoma. Diabetic patients exhibiting migratory skin lesions should be suspected of glucagonoma. In addition, a multidisciplinary approach to such patients including dermatologists, surgeons, radiologists and endoscopists is mandatory.
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PMID:A particularly aggressive combined glucagonoma and gastrinoma syndrome. 1065 4

We reviewed our 8.5 year experience with magnetic resonance imaging (MRI) in the demonstration of neuroendocrine tumors of the pancreas using precontrast fat-suppressed T1-weighted, fat-suppressed T2-weighted, and serial post-gadolinium T1-weighted images, to describe the spectrum of appearances of these tumors. All MR examinations of patients with histologically proven neuroendocrine tumors were retrospectively reviewed. Histological type, tumor location, tumor diameter, signal intensity on precontrast images, enhancement patterns, and presence and appearance of metastases were determined. Twenty-two patients had histologically proved neuroendocrine tumors detected by MRI over the 8.5 year period. Histological types were gastrinoma (n = 8), insulinoma (n = 3), glucagonoma (n = 2), somatostatinoma (n = 1), VIPoma (n = 1), ACTHoma (n = 1), carcinoid (n = 1), and five untyped tumors. Primary tumors ranged in diameter from 1 to 6.2 cm. There was one histopathology-proven false-positive neuroendocrine tumor. The positive predictive value for MRI in the detection of these tumors was 96%. The most common appearance on precontrast images was low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, which was observed in tumors in 18 of 22 patients. Moderate or intense early enhancement of all or portions of the primary tumors was observed in tumors in 19 of 22 patients either as uniform homogeneous, ring, or diffuse heterogeneous enhancement. Enhancement was minimal on these images in the other three patients. Gastrinomas enhanced in a ring pattern in 7 of 8 patients whereas the majority (9 of 11 patients) of noninsulinoma-nongastrinoma and untyped tumors enhanced in a diffuse heterogeneous fashion. Liver metastases were present in 13/22 patients including 3/8 with gastrinoma and 9/11 with noninsulinoma-nongastrinoma tumors. Most neuroendocrine tumors of the pancreas are low signal intensity on fat-suppressed T1-weighted images and moderately high in signal intensity on fat-suppressed T2-weighted images, although variations do exist. Tumors most often enhance in an early moderately intense fashion. Gastrinomas are often different in appearance than other neuroendocrine tumors in that they usually enhance in a ring fashion whereas nongastrinoma-noninsulinoma tumors usually enhance in a heterogeneous fashion.
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PMID:Neuroendocrine tumors of the pancreas: spectrum of appearances on MRI. 1071 46

Two years after resection of a pancreatic glucagonoma, scintigraphy with 111indium-labeled octreotide revealed hepatic metastases in a 48-yr-old man. Hepatic metastases were also visualized by CT, whereas an additional lesion in the chest was seen only by scintigraphy. A total of 11 follow-up examinations over 46 months proved somatostatin receptor scintigraphy to monitor reliably somatostatin receptor expression, growth and dissemination of glucagonoma metastases, and to indicate therapeutic readjustment if necessary. The survival time of the patient is now >75 months, in comparison with a mean survival time of 59 months reported for metastatic glucagonoma.
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PMID:Scintigraphic long-term follow-up of a patient with metastatic glucagonoma. 1092 91

A case of 40-year-old patient with glucagonoma associated with neurological and consciousness disturbances is reported. The diagnosis of the tumour was based on clinical manifestations (diabetes mellitus, anaemia, weight loss, distant metastases), visualisation (USG, CT of the abdomen) and immunohistochemical staining of the biopsy of tumour metastatis to the liver. During the progress of disease paraplegia, other neurological symptoms and three episodes of coma were observed. This should be associated with the neurologic paraneoplastic syndrome and hepatic failure due to diffuse metastases to the liver, especially when no metastases to the central nervous system were found in CT. Although patient was treated with chemotherapy, disseminated neoplasmatic process was the cause of the fatal outcome.
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PMID:[Neurological disturbances in the course of glucagonoma: a case report]. 1094 3


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