Lymphangiomatosis - very rare disease of the lymphatic vessels.

Lymphangiomatosis is rare disease, we can find this entity in differential diagnosis of osteolytic leasions of bones of unknown origin. Typical sign for lymphangiomatosis is proliferation of lymphatic tissue with production of lymphangiomas in various organs and systems. Clinical manifestation of disease is variable, involvement of lungs and bone is typical. In our article we present recent classification of lymphatic tissue neoplasias, their clinical symptoms and treatment possibilities.

RADIATION EXPOSURE OF PAEDIATRIC GENERAL RADIOGRAPHY, FLUOROSCOPY AND CT PROCEDURES IN THE CZECH REPUBLIC-PILOT STUDY.

The pilot dose survey on paediatric general radiography, fluoroscopy and CT procedures was performed in four university hospitals. The analysis of data was focused on the radiography and CT imaging of head, chest, abdomen, pelvis and spine and fluoroscopic procedures of gastrointestinal and urinary tracts. The survey was conducted by the National Radiation Protection Institute. Two hospitals exported data from the patient dose management system, while the others collected the data manually. The methodology of diagnostic reference levels assessment was proposed and tested. Local diagnostic reference levels were calculated in terms of air kerma-area product $P_{KA}$, CT air kerma-length product $P_{KL,CT}$ and volumetric CT air kerma index $C_{VOL}$. The lack of procedure standardisation, e.g. in tube voltage setting irrespective of patient's weight, was revealed at one hospital. Dose and exposure parameters distributions with respect to patient's anatomical constitution are presented in this article. In future, this pilot study will be a base for national survey of paediatric diagnostic reference levels.

Comprehensive Molecular Profiling for Relapsed/Refractory Pediatric Burkitt Lymphomas-Retrospective Analysis of Three Real-Life Clinical Cases-Addressing Issues on Randomization and Customization at the Bedside.

In order to identify reasons for treatment failures when using targeted therapies, we have analyzed the comprehensive molecular profiles of three relapsed, poor-prognosis Burkitt lymphoma cases. All three cases had resembling clinical presentation and histology and all three patients relapsed, but their outcomes differed significantly. The samples of their tumor tissue were analyzed using whole-exome sequencing, gene expression profiling, phosphoproteomic assays, and single-cell phosphoflow cytometry. These results explain different treatment responses of the three histologically identical but molecularly different tumors. Our findings support a personalized approach for patient with high risk, refractory, and rare diseases and may contribute to personalized and customized treatment efforts for patients with limited treatment options like relapsed/refractory Burkitt lymphoma. SUMMARY: The main aim of this study is to analyze three relapsed Burkitt lymphoma patients using a comprehensive molecular profiling, in order to explain their different outcomes and to propose a biomarker-based targeted treatment. In cases 1 and 3, the tumor tissue and the host were analyzed prospectively and appropriate target for the treatment was successfully implemented; however, in case 2, analyses become available only retrospectively and his empirically based rescue treatment did not hit the right target of his disease.