Bulletin du cancer
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Review
[Cowden disease and the PTEN gene: a successfully clinical and biological combined approach].
Cowden disease is an autosomal dominant inherited cancer syndrome characterized by the occurrence of multiple hamartomas, tumors or hyperplastic lesions that may develop in any organ. The disease is related to germline mutation of the PTEN gene, a recently cloned tumor suppressor gene involved in the pathogenesis of sporadic glioblastoma and endometrial carcinoma. It has been shown that the PTEN gene product was a phosphatase able for dephosphorylating a lipid substrate: the phosphatidylinositol (3,4,5)-triphosphate (PIP3). So PTEN appears to negatively control the PI3K-AKT signaling pathway implicated in regulation of cell growth and survival.
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The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the National Federation of Comprehensive Cancer Centres (FNCLCC), the 20 French Cancer Centers and specialists from French Public Universities, General Hospitals and Private Clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery. ⋯ The main recommendations for nutritional support in adult patients with advanced or terminal cancer are: 1) Palliative care has been defined in a consensual way and is governed by the law (standard). Nutritional support is a palliative care which aim is to maintain and restore the "well-being" of the patient (standard). 2) Digestive symptoms and nutritional troubles are frequently noted in patients with advanced or terminal cancer (standard, level of evidence B2). Karnofsky index (KPS) and performance status (PS) are functional scores with a prognostic value and have to be used (standard, level of evidence B2). 3) Anorexia has a bad predictive value in patients with advanced or terminal cancer (standard, level of evidence B2). 4) In France, patients with advanced or terminal cancer are referred to medical institutions, palliative care units or remained at home (standard). Patients need a multidisciplinary follow-up (standard). An active participation of patients and/or their family circle is very important and physicians have to pay attention for their opinions (standard). 5) Dietetic counseling can help patients to improve their alimentation and its drawbacks (standard). 6) Palliative nutritional care often includes medicinal treatments (standard). 7) Artificial nutrition can slow down nutritional degradation, avoid dehydration and improve quality of life in patients with advanced stage cancer (especially head and neck cancer for enteral nutrition and digestive occlusions for parenteral nutrition) and unable to eat adapted meals (standard, level of evidence C). 8) When life expectancy is below 3 months with a KPS 3/4 50% (or PS > 2), artificial nutrition is not recommended (recommendation, expert agreement). 9) The assessment of nutritional care in patients with advanced or terminal cancer has to include functional scores measurement, quality of life and satisfaction degree of the patient and/or their family (standard, expert agreement).
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During the past ten years, the improvements of our understanding of cellular signal transduction pathways provide new targets for drug therapies. Chronic myeloid leukemia (CML), a malignant hematopoietic stem cell disorder, is characterised by an acquired genetic abnormality: the Philadelphia chromosome (Ph) and its molecular counterpart, the oncogene BCR-ABL. The latter, which is translated in an active BCR-ABL protein, exhibited a deregulated tyrosine kinase activity inducing malignant transformation. ⋯ It suppresses the proliferation of the majority of BCR-ABL positive cell lines. The phases I-II clinical trials in CML have demonstrated promising results, especially in the chronic phase of the disease. STI571 is an original therapeutic approach which may be used as a model for the development of other drugs in cancer.