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)

Neuroendocrine tumors of the pancreas and peripancreatic area are rare entities with a wide spectrum of clinical presentation. This study retrospectively reviews the patients who underwent surgery for these tumors at The Johns Hopkins Hospital from 1949 to 1996, inclusive. There were 125 patients (65 males and 60 females) whose mean age was 51 +/- 1 years. Fifty-eight patients (48%) had nonfunctional tumors, whereas 64 (52%) had functional tumors: 35 (55%) insulinomas, 23 (36%) gastrinomas, three (5%) VIP-omas, two (3%) glucagonomas, and one (1%) ACTHoma. All patients with functional tumors presented with appropriate signs and symptoms of hormonal excess; 86% of patients with nonfunctional tumors presented with weight loss, abdominal pain, or jaundice. Preoperative computed tomography (CT) correctly localized the tumor in 66 (76%) of 87 patients; angiography in 45 (58%) of 78 patients; and CT plus angiography in 54 (79%) of 68 patients. Tumors were benign in 60 patients (48%), malignant in 65 patients (52%), and were located in the head, neck, or uncinate process of the pancreas in 54, body in 14, tail in 18, and duodenum in eight. The most common operative procedures performed were 50 pancreaticoduodenectomies (40%), 39 distal pancreatectomies (31%), and 21 tumor enucleations (17%). Nine synchronous hepatic resections were performed for metastases. Of the evaluable patients, 46 (43%) had postoperative complications, the most common of which were pancreatic fistula (16%), wound infection (15%), and delayed gastric emptying (8%). There were three in-hospital deaths (2.8%). With a mean follow-up of 55 +/- 6 months, there have been 30 additional deaths, 23 of which were related to disease progression. The overall 2-, 5-, and 10-year actuarial survival rates were 82%, 65%, and 47%, respectively. The 5-year survival for patients with functional tumors was 77% compared to 52% for those with nonfunctional tumors (P = 0.025); the 5-year survival for patients with benign tumors was 91% compared to 49% for those with malignant tumors (P=0.0004). By univariate analysis the most powerful predictor of poor outcome for patients with malignant tumors (n = 60) was positive surgical margins (P=0.006). This single-institution experience documents low mortality and moderate morbidity for patients treated operatively for pancreatic and peripancreatic neuroendocrine tumors. The most favorable outcomes are observed in patients with benign functional tumors and in those with completely resected malignant tumors.
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PMID:Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: review of 125 patients. 984 8

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

All pancreatic neuroendocrine tumors derive from Langerhans islet cells and have a low incidence. Half of them are functioning tumors that produce diverse hormones and occasionally cause serious clinical endocrine syndromes. They may be malignant, but they have a better survival, if compared to pancreatic ductal adenocarcinoma. Insulinoma, gastrinoma, glucagonoma, VIPoma (VIP=vasoactive intestinal peptide), somatostatinoma and ACTHoma are functioning tumors and they may also be part of Multiple Endocrine Neoplasia type I (MEN 1) syndrome and of von Hippel-Lindau disease. Diagnosis of non-functioning tumors is usually late, when they reach a big size and have even developed nodal and hepatic metastases. Nowadays, there are effective medical treatments for the medical problems secondary to excessive hormone production. For example, the hypergastrinemia typical of the Zollinger-Ellison syndrome in gastrinoma, can be adequately managed. Surgical resection is the most advisable therapy for pancreatic endocrine tumors, especially when they are small, when long time survival is better. Pre and intra operative imagenology is a great aid to locate these tumors. There are several surgical alternatives, according to the tumor size and location within the pancreas. Furthermore, palliative therapy can be used in disseminated disease. Treatment success is the result of a multidisciplinary medical team work of endocrinologists, surgeons, gastroenterologists, pathologists and geneticists.
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PMID:[Pancreatic neuroendocrine tumors]. 1527 51

Pituitary carcinomas are very rare tumors that in most cases produce prolactin and adrenocorticotropic hormone (ACTH). It is a challenge to diagnosis of a pituitary carcinoma before disclosed symptomatic metastasis. We report the case of a female patient with Cushing's disease who underwent three transsphenoidal surgeries, with pathological findings of common ACTH pituitary adenoma including Ki-67 expression <3%. She achieved hypocortisolism after the 3rd surgery although ACTH levels remained slightly elevated. The patient returned some time later with fast worsening of hypercortisolism. Magnetic resonance imaging showed clivus invasion, which led to a fourth surgery and radiation. This time, immunohistochemistry revealed strong Ki-67 (10% to 15%) and p53 expression. Liver and lumbar spine metastases were found on workup. The patient died after few months due to lung infection. Pituitary carcinomas are rare, and the transformation of an ACTH-secreting pituitary adenoma into a carcinoma is exceptional. The difficulty of defining markers for the diagnosis of carcinoma, before metastasis diagnosis, in order to change the management of the disease, is a challenge.
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PMID:Progression of an Invasive ACTH Pituitary Macroadenoma with Cushing's Disease to Pituitary Carcinoma. 2636 11