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 rarity of malignant insulinoma limits reports on therapeutic strategies and outcome. The treatment and follow-up of 10 patients, all presenting an insulinoma with metastatic disease of the liver and newly diagnosed between 1992 and 2002, is reported. Pancreatic surgery with successful removal of the primary tumor preferentially located in the tail was performed in 7 women and 3 men, median age 55 years (range 36-82 years). If appropriate, 5 patients underwent additional hepatic surgery and lymph node resections. Liver metastases as the major cause of postoperatively persistent hypoglycemia were subsequently treated by repeated transarterial hepatic chemoembolization and chemoperfusion protocols using high-dose transhepatic streptozocin perfusions (3-4 g per session). The current median survival time for all 10 patients is 2.6 years (range: 1.6-9.7 years). Six patients are currently alive with a median survival of 3.7 years (1.7-9.7 years), five of them with stable disease and free of hypoglycemia. Four patients died after a median survival of 1.8 years (range: 1.6-7.5 years) from complications of unmanageable hypoglycemia. It is concluded that the necessity to treat debiliating and life-threatening hypoglycemia in metastatic malignant insulinoma warrants the option of radical endocrine surgery in combination with extended and repeated postoperative chemoembolization of liver metastases.
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PMID:Malignant metastatic insulinoma-postoperative treatment and follow-up. 1588 Feb 79

An eight-year-old, female springer spaniel was treated for metastatic insulinoma with a single intravenous dose of 500 mg/m2 streptozotocin (SZN), and pre- and post-treatment diuresis. A tapering dose of corticosteroids was also administered over a 28 day period. SZN and corticosteroid administration resulted in resolution of hypoglycaemia and subsequent development of diabetes mellitus. Further metastases caused cervical spinal pain and the dog was euthanased 118 days after SZN administration. SZN can be safely used for the treatment of canine insulinoma, but, when compared with other published cases, a marked variation in clinical response to this drug exists and further study is warranted.
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PMID:Treatment of insulinoma in a springer spaniel with streptozotocin. 1590 49

We can define paraneoplastic syndromes as a combination of effects occurring far from the original location of the tumour and independently from the local repercussion of its metastases. Paraneoplastic hormonal syndromes depend on the secretion of hormonal peptides or their precursors, cytokines and, more rarely, thyroidal hormones and Vitamin D, which act in an endocrine, paracrine or autocrine way. Sometimes, paraneoplastic syndromes can be more serious than the consequences of the primary tumour itself and can precede, develop in parallel, or follow the manifestations of this tumour. It is important to recognise a paraneoplastic hormonal syndrome for several reasons, amongst which we would draw attention to three: 1) It can lead to the diagnosis of a previously undetected, underlying malign or benign neoplasia; 2) It can dominate the clinical picture and thus lead to errors with respect to the origin and type of primary tumour; and 3) It can follow the clinical course of the underlying tumour and thus be useful for monitoring its evolution. The molecular mechanisms responsible for the development of these syndromes are not well-known, but it is believed that they might be inherent to the mutations responsible for the primary tumour or depend on epigenetic factors such as methylation. In this review, we consider the following paraneoplastic hormonal syndromes: malign hypercalcaemia, hyponatraemia (inappropiate secretion of the antidiuretic hormone), ectopic Cushing's syndrome, ectopic acromegaly, hypoglycaemia due to tumours different from those of the islet cells and paraneoplastic gynaecomastia; we make a brief final reference to other hormones (calcitonin, somatostatin, and VIP).
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PMID:[Paraneoplastic hormonal syndromes]. 1615 18

The surgical management of pancreatic endocrine tumors in patients with multiple endocrine neoplasia type 1 remains controversial. Gastrinoma and insulinoma are the 2 most common functional pancreatic neuroendocrine tumors in patients with multiple endocrine neoplasia type 1. Gastrinomas cause gastric acid hypersecretion and peptic ulcer disease that are best managed using proton pump inhibitors. Surgery to remove the gastrinoma in patients with multiple endocrine neoplasia type 1 is seldom curative unless a more extensive Whipple pancreaticoduodenectomy is performed. Because the prognosis is excellent, aggressive resections such as a Whipple procedure are only indicated for large, locally metastatic, advanced tumors. Furthermore, surgery to remove imageable tumors that are 2 cm in diameter is associated with excellent outcomes and decreased probability of liver metastases. Because gastrinomas are commonly multiple and most originate in the duodenum and develop lymph node metastases, the duodenum should be opened and all tumors and lymph nodes excised. Insulinomas cause hypoglycemia that results in neuroglycopenic symptoms. Medical management of the hypoglycemia is less effective than that of the gastric acid hypersecretion. Fortunately, the insulinoma is usually clearly identified using routine pancreatic imaging studies. There is a high likelihood of cure when the insulinoma is excised surgically. However, recurrent hypoglycemia may occur, and careful follow-up is indicated.
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PMID:Surgery for gastrinoma and insulinoma in multiple endocrine neoplasia type 1. 1645 71

Insulinoma is the most common pancreatic endocrine tumor, accounting for 40% of all pancreatic functional neoplasm, and is characterized by hypersecretion of insulin and hypoglycemia. Elective treatment for insulinomas is surgical enucleation. Medical therapy with diazoxide, followed by somatostatin analogues in some cases, may be necessary to treat the hypoglycemic symptoms. We report a case of a patient affected by metastatic insulinoma with severe hypoglycemia. After surgery, histopathology confirmed the presence of a malignant insulinoma with multiple metastases in the liver. Due to the persistence of hypoglycemia, the patient was started on octreotide LAR treatment, which determined a complete clinical remission with regression of the metastatic lesions in the liver after one year. Repeated CT scans 2 and 3 years after surgery confirmed the remission. To our knowledge, the complete regression of the disease in insulinomas treated with long-standing somatostatin analogue therapy has never been reported. Immunohistochemical analysis in tissue specimens showed a strong membrane immunoreactivity for somatostatin receptors type 2 (SSTR2) in both the primary nodule and the metastases. The capacity of somatostatin analogues to negatively regulate cell proliferation through indirect and direct mechanisms has been experimentally demonstrated. Furthermore, SSTR2 activation may exert pro-apoptotic effects in neoplastic cells. Thus, both mechanisms may have been responsible of the remission of the disease in this patient. This case underlies the potential impact of the treatment of pancreatic insulinomas with somatostatin analogues, and, if confirmed, the usefulness of SSTR determination in these neoplastic specimens.
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PMID:Complete clinical remission and disappearance of liver metastases after treatment with somatostatin analogue in a 40-year-old woman with a malignant insulinoma positive for somatostatin receptors type 2. 1647 42

Pancreatic tumors constitute a rare surgical problem in infancy and childhood. Insulinomas are rare in all age groups with an estimated incidence of one per 250,000 person-years and even rarer in childhood. We report a 10 year-old girl with malignant insulinoma. The presenting symptom was hypoglycemic attacks. Laboratory investigation demonstrated that the hypoglycemia was due to hyperinsulinism. MRI of the abdomen revealed a mass at the tail of the pancreas. Distal pancreatectomy with splenectomy was performed. Histological examination showed malignant insulinoma with peripancreatic lymph node metastases. One month later abdominal MRI revealed the existence of multiple small metastatic foci in the liver, which were confirmed by In111 octreoscan. Treatment with octreotide was started and the disease is stable after 12 months of therapy.
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PMID:Malignant insulinoma in childhood. 1678 43

We wanted to assess the effectiveness and safety of glargine in the treatment of patients with type 2 diabetes mellitus in secondary failure and/or with severe comorbidities ("T2DM group"), and patients with secondary diabetes after corticosteroid and/or anticancer treatment ("secondary DM group"). We reviewed the records of patients on glargine from 1 August 2004 to 30 July 2005. The after-minus-before change in HbA1c was the main outcome measure. At baseline, the 18 "T2DM" patients had a mean (+/-SD) age of 66.7+/-9.5 years and a diabetes duration of 13.6+/-10.3 years; 52.9% were male. Their fasting plasma glucose (FPG) decreased from 228.6+/-76.6 to 134.6+/-37.5, two-hour post-prandial glycaemia (2hPPG) from 268.2+/-10.4 to 140.6+/-30.8 and HbA1c from 10.4+/-2.3 to 7.9+/-1.6%. Mean daily insulin dosage was 12.0+/-4.8 UI for glargine alone and 37.4+/-22.6 UI for basal-bolus scheme. The daily cost was Euro 0.75 (range Euro 0.31-1.15). The 24 "secondary DM" patients had a mean age of 67.0+/-11.0 years and a diabetes duration of 3.7+/-6.5 years; 54.2% were male and 91.7% had a metastatic cancer. Their FPG decreased from 222.3+/-108.6 to 121.5+/-28.7 mg/dl, 2hPPG from 259.4+/-108.6 to 133.0+/-35.0 mg/dl and HbA1c from 10.1+/-2.5 to 7.6+/-1.3%. Mean daily insulin dosage was 12.5+/-6.1 UI for glargine alone and 27.2+/-9.1 UI for basal-bolus scheme. Mean daily cost was Euro 0.70 (range Euro 0.31-1.38). One (4.2%) cancer patient withdrew from glargine because of nausea. Nine (37.5%) cancer patients had an increase in appetite after glargine therapy, including 3 end-of-life patients. No severe hypoglycaemia occurred. Insulin glargine was safe and effective in improving glycaemic control both in severe "T2DM" and in "secondary DM" patients.
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PMID:Effectiveness and safety of insulin glargine in the therapy of complicated or secondary diabetes: clinical audit. 1686 31

A 14-year-old domestic shorthair cat was presented with hypoglycaemia and seizures of several weeks duration. Bloodwork revealed hypoglycaemia (1.83 mmol/l; reference range 4.22-8.05 mmol/l) with concurrent normal insulin levels (171 pmol/l; reference range 72-583 pmol/l). An insulinoma was suspected and medical and dietary management were attempted with minimal success. An exploratory laparotomy was performed and a single, well-defined mass was found within the left lobe of the pancreas. The mass was removed and histologically classified as an islet cell carcinoma, consistent with an insulinoma. The cat had an episode of presumed postoperative pancreatitits, but recovered with appropriate treatment. The cat has had no clinical signs of recurrence of greater than 32 months postsurgery. There are only four cases of insulinoma in cats reported in the literature. All prior insulionomas reported were in older cats and were malignant in character, which is similar to the reports in the dog. This case is unique because of the apparent lack of local recurrence and development of metastatic disease, leading to the prolonged survival.
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PMID:Insulinoma in a cat. 1778 34

Sarcomas of the liver are rare. We report a case of intractable hypoglycemia secondary to a solitary fibrous tumor that underwent malignant transformation into a fibrosarcoma. A 70-year-old man presented with a hepatic mass and tumor-associated hypoglycemia which was resistant to medical management. Blood tests were remarkable only for elevated serum insulin-like growth factor (IGF)-2. The hypoglycemia resolved following resection of a solitary fibrous tumor surrounded by a high-grade fibrosarcoma. Real time reverse transcriptase polymerase chain reaction (RT-PCR) measured elevated levels of IGF2 mRNA in both the solitary fibrous tumor and the fibrosarcoma. Immunoblotting demonstrated a series of bands in the size range of pro-IGF2. Unfortunately, disseminated metastases developed 1 year later, concurrent with a recurrence of hypoglycemia, marked again by elevation of serum IGF2. Solitary fibrous tumors of the liver have a real risk of malignant transformation. The severity of the tumor-associated hypoglycemia may parallel the tumor burden and activity. The syndrome is the systemic effect of IGF2 secreted by the tumor. Surgery can treat the hypoglycemia syndrome and the underlying malignancy.
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PMID:Malignant transformation of a solitary fibrous tumor of the liver and intractable hypoglycemia. 1804 Jun 28

This review focuses on the tumour types and symptoms associated with non-islet cell tumour-induced hypoglycaemia (NICTH) as well as the pathogenesis, diagnosis and treatment of this rare paraneoplastic phenomenon. In addition, we report two illustrative cases of patients suffering from NICTH caused by a solid fibrous tumour and a haemangiopericytoma respectively. In the first case, NICTH resolved following complete resection of the tumour, but in the second case the patient needed long-term treatment aimed at controlling hypoglycaemia because of non-resectable metastases. Many tumour types have been associated with NICTH. The crucial event in the development of NICTH seems to be overexpression of the IGF-II gene by the tumour. NICTH is characterised by recurrent fasting hypoglycaemia and is associated with the secretion of incompletely processed precursors of IGF-II ('big'-IGF-II) by the tumour. This induces dramatic secondary changes in the circulating levels of insulin, GH, IGF-I and IGF-binding proteins, resulting in an insulin-like hypoglycaemic activity of 'big'-IGF-II.
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PMID:Non-islet cell tumour-induced hypoglycaemia: a review of the literature including two new cases. 1804 50


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