La Revue du praticien
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La Revue du praticien · Feb 2002
Review[Definition and histologic characterization of neuroendocrine tumors].
Neuroendocrine tumours are defined by a common phenotype, which is not supported by a common embryologic origin. This common phenotype is characterized by the expression of general neuroendocrine markers, and sometimes by cell specific hormonal products. Neuroendocrine tumours are ubiquitous, but the major localizations are the digestive tract. ⋯ Size, degree of invasion, major secretion, and proliferation rate are the main criteria of this classification. Most neuroendocrine tumours are sporadic. A few cases occur in the context of a multiple endocrine neoplasia type 1.
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The somatostatin analogues have an excellent symptomatic therapeutic effect (60-90% responses in carcinoid syndrome) which permits an amelioration in the quality of life, particularly in patients with metastatic tumours. The availability of long-acting somatostatin analogues assuring stable plasma concentrations over several weeks (thanks to progressive liberation) further facilitates the use of these medications by allowing a reduction in the number of injections. Interferon also has an anti-secretory effect, albeit inferior to that of the somatostatin analogues, but potentially of interest in the case of resistance to these products.
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Therapeutic strategy of neuroendocrine tumours is complex, due to their heterogeneity and to the fact that although generally slow growing, a significant proportion demonstrates aggressive tumour growth. Symptomatic carcinoid syndrome and various pancreatic endocrine tumours with symptomatic syndromes are well controlled with somatostatin analogues. Surgery remains the mainstay of treatment if the tumour can be resected. ⋯ In indolent cases, somatostatin analogues are the best treatment, in case of aggressive tumours chemoembolisation should be preferred when the disease is located or predominant in the liver. Poorly differentiated tumours are treated by combination chemotherapy with etoposide and cisplatin, and surgery has no indication. Gastrinoma and other pancreatic tumours arising in the context of multiple endocrine neoplasia type I disease need a specific therapeutic strategy.
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Recurrent fevers in children are common, mainly due to viral (particularly in day care centers), or to bacterial (urinary tract upper and lower respiratory) infections. The diagnosis of recurrent hereditary fever is now possible on the basis of clinical features, biochemical and genetic tests. Familial Mediterranean Fever (FMF) remains the most frequent disorder of this group, which includes now three other entities: TNF receptor associated periodic syndrome (TRAPS), the hyperIgD syndrome(HIDS) and the Muckle-Wells syndrome.