Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P42345 (mTOR)
 26,049 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Insulinoma is the most common hormonally active neuroendocrine tumor of the pancreas. Low blood sugar, caused by excessive secretion of insulin from the tumor cells, leads to a variety of symptoms (e.g. signs of neuroglycopaenia), that may be mistaken for diseases of the central nervous system, psychiatric disturbances or cardiovascular conditions. That is why a lot of attention should be brought to the diagnostic tests towards insulinoma. The development of imaging techniques in the past years has lead to the possibility of locating an insulinoma of a few milimetres in size, which is helpful to avoid the need of a blind resection and preserves the pancreatic parenchyma. 90% of insulinomas are benign and surgery is the treatment of choice. However, as in any neuroendocrine tumor, a malignant form may occur--resection of the metastases is the only curative method, but radiofrequency ablation, selective internal radiation therapy, hepatic artery embolization or chemoembolization, can also have positive therapeutic applications. Systemic therapies regarding malignant insulinoma are: chemotherapy, somatostatin analogs, radiolabelled somatostatin analogs and the newly developed biological targeted therapies such as everolimus--oral inhibitor of the mammalian target of rapamycin, and sunitinib--the tyrosine kinase inhibitor.
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PMID:[The newest perspectives on diagnostic methods and treatment of insulinoma]. 2576 82

Insulinoma is the most common neuroendocrine tumor of the pancreas. Surgical management of insulinomas is considered to be the only curative method. However, effective glycemic control preoperatively and in unresectable insulinomas remains a significant issue. Hyperinsulinism, occurring as a result of the hormone-secreting tumor, leads to life-threatening hypoglycemia episodes which require urgent medical treatment. This article discusses current management of hypoglycemia in insulinoma patients, including: education and lifestyle modifications, pharmacotherapy (diazoxide, somatostatin analogs, mTOR inhibitor - everolimus), cytoreductive methods and continuous glucose monitoring systems.
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PMID:Glycemic control in patients with insulinoma. 2822 4

Insulinoma is a rare neuroendocrine pancreatic islet cell tumor of which the majority are benign and solitary. Its estimated incidence is 2 to 4 cases per 1 million person-years. We report the case of an 82-year-old female who presented with metastatic insulinoma to the liver and retroperitoneal lymph nodes. It was diagnosed based on positive Whipple's triad, elevated insulin, elevated C-peptide, and negative insulin auto-antibody. Her disease was initially managed with diazoxide 100 mg orally every 8 hours, octreotide LAR 30 mg intramuscularly every month, and sunitinib 12.5 mg orally three times a day. However, patient had recurrent symptoms and imaging consistent with worsened metastatic insulinoma; and thus, treatment was changed to everolimus 10 mg PO daily. Over the subsequent 10 months, the patient developed progressive shortness of breath and hypoxemia with and oxygen saturation (SpO2) of 89% on room air. Computerized tomography (CT) and lung biopsy were consistent with cryptogenic organizing pneumonia (COP) temporally associated with the initiation of everolimus. She was started on prednisone 1 mg/kg/day and within 48 hours, her symptoms and hypoxemia improved to SpO2 of 98-99% at room air and her repeat CT chest showed marked disease improvement. Given her good response with everolimus, it was continued in conjunction with the prednisone and to this day, patient has had a significant therapeutic response with normoglycemia and stable, well-controlled symptoms. This case is unique because to our knowledge, it is the first reported case of a patient with metastatic insulinoma complicated by the development of cryptogenic organizing pneumonia. It reaffirms the causal association between everolimus and cryptogenic organizing pneumonia that has been reported numerous times in literature. However, there has been no reported cases showing that the COP can be managed with prednisone concurrently with everolimus for the metastatic insulinoma without diminished clinical benefit. While pulmonary complications have been cited as reasons for discontinuation of everolimus therapy, our case report highlights the use of steroids as a viable therapeutic strategy that allows successful therapy with everolimus to be continued. In addition to presenting this case, we will also do a thorough review of the literature surrounding the available therapeutic options of metastatic insulinoma. This will include surgery, somatostatin analogs, antimicrobials, potassium channel activators, VEGF-A inhibitors, alkylating agents and mTOR inhibitors to provide a more in-depth picture of how we treat metastatic insulinoma.
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PMID:Refractory Metastatic Insulinoma Treated with Everolimus, Complicated by Cryptogenic Organizing Pneumonia. 3141 42