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)

The pancreatic somatostatinoma belongs to the type of rare endocrine tumors of the pancreas. We report the observation of a 54 year old woman. Previously she was suffering from diabetes mellitus. An abdominal ultrasonography revealed an endocrine tumor of the pancreatic tail. There was no specific symptomatology, with the exception for the hyperglycaemia. The diagnosis of somatostatinoma was certified post operatively by the immunocytochemistry of the tumor. Then, the patient developed a hypercalcaemia associated with an increase of parathyroid hormone. The surgery of the neck revealed three hyperplastic parathyroids, inducing this association as a multiple endocrine neoplasia type 1 (MEN 1). The patient did not develop pituitary tumor. Afterwards, scintigraphy with 111 Indium- octreotide showed a residual tumor at the head of pancreas. Basal levels of somatostatine and calcium, pentagastrine test, computed tomography scan, arteriography were negative. The presence of a second somatostatinoma was confirmed by surgery and immunohistology. One year after the surgery, the patient remains clinically well. The pancreatic localization of the somatostatinoma in a MEN 1 is poorly documented. Its malignant nature can only be assured by the presence of metastases. The genetic detection of the MEN 1 becomes possible. Above all, the treatment is based on surgery and/or chemotherapy (Fluoro-Uracile; Streptozotocine). In our case, 111 In-octreotide scintigraphy was the only method demonstrating a residual focus, suggesting it could be an element of reference for the diagnosis and survey of somatostatinoma the watch of patients having a treatment for somatostatinoma.
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PMID:[Pancreatic somatostatinoma and MEN 1. Apropos of a case. Review of the literature]. 873 92

Somatostatinomas are rare endocrine tumors that are located primarily in the pancreas. Metastases are seen most frequently in the liver and lymph nodes. The authors present the case of a 63-year-old man who had a malignant somatostatinoma of pancreatic tail origin that metastasized to the brain 10 years after diagnosis of the primary tumor. The metastatic brain lesions were totally removed and the patient is alive without tumor recurrence 12.3 years after the initial diagnosis. To our knowledge, this case represents the first documentation of brain metastasis from a malignant somatostatinoma, as well as the longest survival time of a patient with a somatostatinoma.
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PMID:Brain metastasis from malignant pancreatic somatostatinoma. Case report. 881 75

Islet cell tumors are rare pancreatic or peripancreatic neoplasms that produce and secrete hormones to a variable degree. These tumors are best divided on clinical grounds into those that produce a recognizable, clinically evident endocrine syndrome (ie, functioning) and those that exhibit no clinical evidence of hormone production (ie, clinically silent). Clinically silent tumors produce symptoms due to mass effect because of their large size. They are often partially cystic or necrotic. Functioning islet cell tumors usually manifest earlier in the course of the disease because of the distinctive signs and symptoms of the associated endocrine syndrome. Clinically silent and functioning tumors cannot be histologically distinguished reliably even with the use of immunohistochemical stains. Insulinoma and gastrinoma, the two most common functioning lesions, are typically small homogeneous masses. Other functioning islet cell tumors include glucagonoma, somatostatinoma, vipoma, and adrenocorticotropic hormone-producing tumor. Larger tumors are associated with calcification, cystic degeneration and necrosis, and a more aggressive behavior (local and vascular invasion as well as distant metastases). There are many different techniques for detection and characterization of these lesions that are usually chosen according to the radiologist's experience and preference. Treatment and prognosis of these lesions depend on the hormone produced, their size, and their behavior.
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PMID:Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization. 908 84

Somatostatinoma are uncommon pancreatic endocrine tumors. We review the epidemiological, pathological, clinical and biological characteristics. The diagnosis of a somatostatinoma may be suggested clinically but is confirmed by histology of the resected tumor and specific immunohistochemistry marking. Malignancy is diagnosed on the presence metastases. Surgery is required with excision of the tumor, lymph nodes and metastases.
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PMID:[Somatostatinoma. Apropos of a case]. 929 93

A 57 year-old Japanese man with a carcinoid somatostatinoma of the papilla of Vater is presented. He was found to have cholecystolithiasis without any symptoms. Physical examination showed no abnormal findings. Routine laboratory data gave normal results, except for glucose intolerance and an elevated somatostatin concentration. A yellowish papillary tumor was found at the papilla of Vater, and histological examination suggested the diagnosis of carcinoid. He underwent a pancreatoduodenectomy in March 1992. The gallbladder contained a single pure cholesterol stone. Histological, immunohistochemical, and electron microscopic studies resulted in the diagnosis of a carcinoid somatostatinoma of the papilla of Vater, without regional lymph node metastases. Post-operative pancreatic juice output from the total pancreatic duct drainage increased to more than 1000 mL/day. Although an anastomotic leakage of the pancreatojejunostomy was noted, the pancreatic fistula closed 8 weeks later. His postoperative somatostatin value was normal. He has been well for 54 months following surgery, without any signs of recurrence.
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PMID:Carcinoid somatostatinoma of the papilla of Vater: a case report. 963 24

Somatostatin is known to inhibit the secretory release of other peptide hormones. Somatostatinomas associated either with or without somatostatinoma (inhibitory) syndrome are rare neoplasms among gut-pancreatic endocrinomas. Collected from international literature, this study aimed to perform a statistical analysis on 173 patients with somatostatinoma/inhibitory syndrome. The evaluation further attempted to provide investigators in this particular field of research with extensive and precise information on the present situation of somatostatinoma. The 173 patients consisted of 81 with pancreatic somatostatinomas and 92 with extrapancreatic somatostatinomas. Most of the latter were found to have originated in the duodenum and may be termed as carcinoid somatostatinoma. Where data were considered to be adequate, a comparative study was carried out between two groups, pancreatic and duodenal, each consisting of 81 patients. A statistically significant difference between these two groups was found in the incidence of inhibitory syndrome (18.5% versus 2.5%) and von Recklinghausen's disease (1.2% versus 43.2%), large size of tumor (>20 mm) (85.5% versus 41.4%), multisecretory activities (33.3% versus 16.3%), and presence of psammoma bodies (2.5% versus 49.4%). There was no statistically significant difference in the rate of metastases and malignancy between the two groups. The average postoperative 5-year survival rate was 75.2% in 90 patients overall, 59.9% in 44 with metastases and 100.0% in 46 without metastases. Compared with the other pancreatic endocrinomas, including PPomas, glucagonomas, vipomas, gastrinomas, and insulinomas, somatostatinomas were characterized by the low rate of the relevant syndrome and multiple endocrine neoplasia syndrome type 1. There was a low rate of multiplicity, and a high incidence of psammoma bodies in the duodenal group particularly with von Recklinghausen's disease. A high rate of malignancy was recorded, resulting in a low postoperative survival rate of patients with metastases. In conclusion, somatostatinomas exhibited characteristic features quite different from those of the other pancreatic endocrinomas regarding multiple points.
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PMID:Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas. 1037 71

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

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

Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. We report a case of somatostatinoma in a 42-year-old male with neither neurofibromatosis nor somatostatinoma syndrome. A large tumor in the descending duodenum had given rise to multiple lymph node metastases. An additional 31 duodenal somatostatinoma cases were also reviewed. Most originated in the descending part of the duodenum, with the ampulla and peri-ampullary area as the most common sites (60%). Frequent manifestations were abdominal pain (25%), jaundice (25%), or cholelithiasis (19%), the latter two reflecting obstruction of the bile duct by tumors. Only two cases showed a possible somatostatinoma syndrome (6%). The tumors with metastases, lymph nodes (10) and liver (2), were significantly larger than average than those without (2.91 +/- 1.49 cm vs 1.36 +/- 0.71 cm, P < 0.05). With a cut-off point of 2.0 cm, diagnostic accuracy for metastasis was 77.78% with 87.50% specificity and 63.64% sensitivity. The smallest tumor with metastases was 0.8 cm and the largest without metastases was 3.0 cm. These results indicate that duodenal somatostatinomas are malignant by nature and the risk of metastasis significantly increases with tumors larger than 2.0 cm.
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PMID:Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis. 1110 73

A new concept of classifying neuroendocrine pancreatic tumors based on clinicopathologic patterns was summarized recently. To evaluate the clinical reliability and prognostic specificity of this classification system, 100 neuroendocrine pancreatic tumors were retrospectively categorized as "benign," "uncertain," and "malignant" based on tumor risk factors (size, local invasion and angioinvasion, cell atypia, metastases) and were followed for disease recurrence and progression. Altogether, 71 functioning tumors (insulinoma, gastrinoma, glucagonoma, enterochromaffin-like (ECL)oma, somatostatinoma) and 29 nonfunctioning neuroendocrine pancreatic tumors (NETs) were studied. NETs had an increased risk of malignancy (p < 0.05). Tumor size, gross invasion, and metastases correlated significantly with tumor behavior and allowed us to distinguish between "benign" and "malignant" tumors. About 89% of the tumors < or = 20 mm were "benign," whereas 71% > 20 mm were "malignant" (p < 0.05). In patients with "benign" and "uncertain" neuroendocrine pancreatic tumors, neither recurrence nor progression of disease was seen. About 41% of the patients with "malignant" tumors died of the disease. The 5-year estimated cumulative survival of those with "benign" and "uncertain" tumors was 100% and 52 +/- 10% for those with "malignant" tumors (p < 0.05). Histomorphologic details classifying the behavior of an "uncertain" tumor are known only after initial treatment and definitive histopathologic investigation. Thus this information is of limited clinical help for treatment strategies.
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PMID:Is the new classification of neuroendocrine pancreatic tumors of clinical help? 1103 99


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