Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0178874 (tumor progression)
 40,807 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors describe their experience with liver transplantation (OLT) for metastatic endocrine tumors (MET) in order to determine reasonable indications for OLT in patients with this disease. Removal of the primary lesion and subsequent liver transplantation were performed in two separate procedures in all patients except one. Only those patients suffering from objective tumor progression and symptoms with no evidence of extrahepatic spread after complete work-up (including endoscopic ultrasonography (US) and 123I-labeled Tyr3-octreotide body scanning) underwent liver transplantation. Fifteen patients were referred for liver transplantation. Seven patients were excluded either because of stability of liver metastases (n = 3), extrahepatic spread, general contraindication (n = 2), or feasibility of aggressive surgical resection (n = 2). Liver transplantation was undertaken in eight patients with carcinoid tumor (n = 4), gastrinoma (n = 3) and glucagonoma (n = 1). Three patients did not survive the surgical procedure itself, whereas two additional patients died from chronic rejection or from recurrent disease. Three patients who received transplants for metastatic carcinoid tumor are alive without biochemical or imaging evidence of disease recurrence at 6, 15, and 52 months. The best indication for transplantation seems to be patients with metastases restricted to the liver and unresponsive to adjuvant therapy after aggressive surgical resection including excision of the primary lesion and reduction of hepatic metastases. In such highly-selected patients, liver transplantation remains a high-risk operation, but it can yield long-term survival.
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PMID:Metastatic endocrine tumors: is there a place for liver transplantation? 934 51

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome characterized by the occurrence of multiple endocrine tumors of the parathyroid, pancreas, and anterior pituitary in patients. To study tumorigenesis related to the MEN1 syndrome, we have generated Men1 knockout mice using the gene targeting approach. Heterozygous Men1 mutant mice developed the same range of major endocrine tumors as is seen in MEN1 patients, affecting the parathyroid, pancreatic islets, pituitary and adrenal glands, as well as the thyroid, and exhibiting multistage tumor progression with metastatic potential. In particular, extrapancreatic gastrinoma, pancreatic glucagonoma, and mixed hormone-producing tumors in islets were observed. In addition, there was a high incidence of gonadal tumors of endocrine origin, i.e. Leydig cell tumors, and ovary sex-cord stromal cell tumors in heterozygous Men1 mutant mice. Hormonal disturbance, such as abnormal PTH and insulin levels, was also observed in these mice. These tumors were associated with loss of heterozygosity of the wild-type Men1 allele, suggesting that menin is involved in suppressing the development of these endocrine tumors. All of these features are reminiscent of MEN1 symptoms in humans and establish heterozygous Men1 mutant mice as a suitable model for this disease.
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PMID:Heterozygous Men1 mutant mice develop a range of endocrine tumors mimicking multiple endocrine neoplasia type 1. 1281 99

Whereas surgical resection is the only curative treatment for liver tumors, effective treatment for isolated unresectable lesions when there is tumor progression in spite of several lines of chemotherapy remains to be found. We report herein two cases of patients treated by a 1-hour Hyperthermic Isolated Liver Perfusion (HILP) with a combination of melphalan and bevacizumab leading to complete response. The first patient had liver metastases secondary to previously resected malignant glucagonoma and the second, recurrent hepatocellular carcinoma. We used bevacizumab in association with melphalan for HILP because of the additional effect of an anti-VEGF antibody in these highly vascularized tumors and its locally restricted delivery to the isolated hepatic vascular compartment despite of its classic contraindication in association with surgery. The protocol was approved by the Ethics Committee. Enhanced CT scans during follow-up showed complete tumor necrosis as early as the second postoperative day. Patients had 27 and 7 months disease-free survival and 48 and 41 months overall survival after HILP, for neuroendocrine liver metastases and HILP plus liver transplantation for HCC respectively. Under very specific conditions, bevacizumab in HILP can provide excellent tumor response in hopeless clinical cases of liver tumors.
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PMID:Hyperthermic isolated liver perfusion with melphalan and bevacizumab. 2318 50