Coexistence of neurofibromatosis type 1 with multiple malignant neoplasia.

Neurofibromatosis type 1 (NF1, von Recklinghausen disease) is inherited in autosomal dominant way genetic disorder, with an incidence at birth 1:3000. It is one of the most common congenital disorders. It is characterized by café-au-lait spots, neurofibromas, and less common MPTST and gliomas of the optic nerve. It is caused by germline mutations of the NF1 gene, which acts as tumor suppressor. Inactivation of the gene leads to increased activation of the kinase pathways, and in consequence, uncontrolled proliferation of cells. The disease predisposes to the development of both benign and malignant tumors. Malignant tumors, but not related to the nervous system occur in neurofibromatosis quite rare. The aim of the study is a literature review of NF1, with presentation of a patient with NF1 and coexisting numerous tumors: synchronous somatostatinoma and gastrointestinal stromal tumor with metachronous prostate adenocarcinoma and non-small cell lung carcinoma. And attempt to answer the question if there is a common pathway for oncogenesis of these four tumors.

[Cardio-vascular risk factors control in elderly patients with peripheral artery disease]. / Kontrola czynników ryzyka sercowo-naczyniowego u starszych pacjentów z choroba tetnic obwodowych.

OBJECTIVE: The assessment of control of modifiable risk factors among elderly patients with peripheral artery disease (PAD) admitted to the hospital angiology ward. METHODS: The results of treatment of dyslipidemia (DL), hypertension (HT), diabetes mellitus (DM) and prevalence of cigarette smoking were assessed among older (>65 years old, group I) and younger patients (group II) with PAD in a admission day to hospital. RESULTS: The study population included 154 patients (I--92 and II--65 subjects) aged 67.4 ± 9.4 years, 69.5% men. The study groups presented similar grades of PAD classification by Rutherford. Group I was older (73.5 ± 6.4 vs 58.3 ± 4.7 yrs, p < 0.001), had lower frequency of current smokers (21.7 vs 48.4%, P < 0.001) than group II. Diagnosis of DL, HT and DM were equally frequent in both groups. However, coronary heart disease was diagnosed more frequently in group I than II (52.2 vs 29.0%, p = 0.004). Both groups were similar according to systolic blood pressure (BP) values and levels of glucose and HDL cholesterol. Group I had lower diastolic BP than group II (69.5 ± 11.1 vs 74.0 ± 9.9 mmHg, p = 0.02), but control of HT was similar in both groups (71.7 vs 67.7%). LDL cholesterol levels were lower in a group I than II (2.2 ± 1.0 vs 2.5 ± 1.1 mmol/l, p = 0.04), but LDL cholesterol values < 1.8 mmol/l were observed with similar frequency in both groups (40.2 vs 27.4%). CONCLUSIONS: Patient's age seems to have no important impact on the control of atherosclerosis risk factors in patients with PAD.