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Rutkowski P.,Klinika Nowotworow Tkanek Miekkich i Kosci | Kulig J.,I Katedra Chirurgii Ogolnej I Klinika Chirurgii Gastroenterologicznej | Krzakowski M.,Klinika Nowotworow Pluca i Klatki Piersiowej | Osuch C.,I Katedra Chirurgii Ogolnej I Klinika Chirurgii Gastroenterologicznej | And 16 more authors.
Nowotwory | Year: 2011

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. Over the last years advances in the understanding of the molecular mechanisms of GIST pathogenesis have resulted in the emerging of GIST as a distinct sarcoma entity. This paper presents the guidelines for diagnostics and therapy of these tumors based on scientific research and experts' experience, These guidelines are commonly accepted and worthy of recommendation. Overexpression of the KIT receptor, as a consequence of mutation of the KITprotooncogene is highly specific for GIST and enables immunohistochemilcal detection staining (CD117) in tumor specimens. It is the most important criterion in microscopic diagnostics and for indicating treatment with small-molecule tyrosine kinase inhibitors. Sending material for molecular analysis is strongly recommended (for KIT and PDGFRA genotyping). Radical surgery is still the mainstay treatment for primary, localized, resectable GISTs, although although a significant ratio of patients after potentially curative operations develop recurrent or metastatic disease. In inoperable/metastatic lesions the treatment of choice is a tyrosine kinase inhibitor - imatynib mesylate - the first effective systemic therapy in advanced CD117(+) GIST. The recommended initial dose should be 400 mg daily (800 mg for exon 9 KIT mutants). Tretament monitoring should be based on serial computed tomography imaging of the abdominal cavity with the assessment of changes of tumor size and density. In case of disease progression the increase of imatynib dose to 800 mg daily is recommended and - if progression maintains - sunitinib in the initial dose of 50 mg daily should be introduced. Clinical trials evaluating the role of surgery combined with imatynib and the efficacy of other molecular targeted drugs in resistant cases are ongoing. Existing data indicate the beneficial role of adjuvant imatynib therapy in terms of relapse-free survival, especially in the group of patients with a significant risk of relapse. The presented recommendations for the diagnostics and therapy of GIST should be practically implemented by physicians involved in the management of GIST patients in Poland. Entering all GIST cases in the National Clinical Registry (http:// gist.coi.waw.pl) and standardising patient treatment in multidisciplinary teams with expertise in GIST therapy, as well as enrollment of new cases into prospective clinical trials, are recommended. Source

Salamacha M.,Klinika Nowotworow Tkanek Miekkich | Kosela H.,Klinika Nowotworow Tkanek Miekkich | Falkowski S.,Klinika Nowotworow Tkanek Miekkich | Cybulska-Stopa B.,Klinika Nowotworow Ukladowych I Uogolnionych | And 2 more authors.
Nowotwory | Year: 2011

Neurofibromatosis comprises a group of three disorders - schwannomatosis, type 2 neurofibromatosis, and the most common - type 1 neurofibromatosis (NF1). NF1 is inherited in an autosomal dominant fashion and is found in 1 per 3500 live births. Type 1 neurofibromatosis is caused by a mutation in the NF1 gene, the gene product being neurofibromin . This protein is a negative regulator of the Ras kinase pathway. Neurofibromin alters or weakens this protein, allowing rapid, uncontrolled growth of cells. The range of symptoms is very wide; they vary from minor cutaneus lesions to malignancies. NF1 is associated with a higher incidence of soft tissue sarcomas, such as MPNST and GIST. MPNST arise from preexisting plexiform neurofibromas, due to a somatic mutation leading to the loss of heterozygosity of the NF1 gene. It requires intensive treatment modalities, often leading to disability and the prognosis is often poor. New therapeutic modalities have been investigated recently, as a consequence of discovering cell pathways leading to this type of cancer. GIST (gastrointestinal stromal tumor) is another soft tissue sarcoma often occurring in NF1 patients. Imatinib - an inhibitor of tyrosine kinases - applied as a standard therapy in the group of sporadic GIST, can be useless for NF1 patients with GIST, because of different pathogenesis. Source

Introduction. The quality of life of cancer patients is becoming an essential element of decision-making in designing anti-cancer therapies. The method of the reconstruction of the gastrointestinal tract has a significant impact on the quality of life of patients after complete resection of the stomach Aim. The presented work examines two methods of reconstruction after total excision of the stomach: Roux-en-Y and Longmire-Henley intestinal insertion. Material and methods. The comparison was performed at least 6 months (6-52, median 14) after the completion of oncological treatment and relied on the subjective assessment of the quality of life based on the EORTC - QLQ-C30 (ver. 2-0) questionnaire, the incidence of postprandial symptoms (Supplementary Book surveys), an objective assessment of BMI, morphology and biochemistry of peripheral blood (nutritional parameters) and endoscopically confirmed esophageal reflux. 68 patients (31 women and 37 men, median age: 63 (34-77) years) were enrolled. All had given informed consent and met the inclusion criteria. Reconstruction methods were as follows: Roux-en-Y - 37 patients, and Longmire-Henley intestinal insertion - 31 patients. Results. The statistical analysis revealed no differences between the groups neither in the subjective assessment of the quality of life and postprandial symptoms nor in an objective assessment of the parameters of their nutritional status. Conclusions. The quality of life and the nutritional status do not differ between the both patient groups. Source

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