Bajcsay A

Erdheim-Chester's disease of the heart: a diagnostic conundrum and collision with the same mass in the orbit.

Erdheim-Chesters disease is a rare multisystem xanthogranulomatosis, afflicting the skeletal system with the occasional involvement of soft tissues. We delineate an unusual case of a cardiac variant of Erdheim-Chesters disease presenting with pericardial effusion and as a collision with a synchronous orbital manifestation. We describe our diagnostic pathway and propose a novel treatment option involving nonsteroidal anti-inflammatory drugs. The role of cyclo-oxygenase in the disease process and inhibition thereof by NSAIDs is hypothesized and discussed.

Az ismételt frakcionált külsô sugárkezelés (reirradiáció) elsôdleges központi idegrendszeri daganatok kiújulásának kezelésére: az elsô hazai tapasztalatok

INTRODUCTION The practice of image-based three dimensional treatment planning and conformal radiotherapy techniques offer the opportunity to elaborate novel treatment forms, e.g. repeat irradiation techniques for primary brain tumours. AIM The authors analysed the effect on survival and toxicity of fractionated external beam repeat irradiation in brain tumour patients. METHODS At the National Institute of Oncology, between 2002 and 2006, fractionated external beam repeat irradiation was performed in eleven patients with recurrent primary brain tumour, with total of 50-54 Gy or near total of 34-40 Gy doses. All patients were previously treated with total radiotherapy doses of 50-64 Gy. The intervals between radiotherapy courses were in the range of 7-30 years. All the treatments were carried out with 3D image-based conformal methods, the fractionation was conventional, with 1,8-2,0 Gy daily fractions in all cases. RESULTS The repeat irradiation was tolerated well in the material. No grade 3-4 acute toxicity was detected, and serious, grade 3 mental deterioration, not related tumour progression was observed in only one case. In one case reoperation was necessary due to histologically verified radio-necrosis with mass-effect, and we believe that late neurotoxicity caused serious functional inabilities in one case. The median progression free survival was 8 (2-33) months, the median survival was 13 (4,5-33) months. Three of our patients were alive at the end of the study. CONCLUSIONS Based on this experience and current knowledge, in absence of other treatment possibilities, the fractionated external beam repeat irradiation with near total doses could be a therapeutic choice in case of recurrent primary brain tumours, if having appropriate background. To define the optimal treatment strategy and regimens, further clinical trials should be carried out.