PNR-20PRIMARY METASTATIC LEIOMYOSARCOMA OF THE SKULL

PNR-20. PRIMARY METASTATIC LEIOMYOSARCOMA OF THE SKULL Alexandra Paul1, Olinda Rebelo2, and Alice Carvalho1; Pediatric Oncology Department, Centro Hospitalar e Universitario de Coimbra-EPE, Coimbra, Portugal; Neuropathology Department, CentroHospitalar e Universitario de Coimbra-EPE, Coimbra, Portugal We report the case of a previous healthy 11 yearold-boy, with a one month history of worsening headache which appeared apparently in the context of trauma. He had an x-ray of the skull that showed a right fronto-parietal lytic lesion with 10 cm in size and with ill-defined borders. CT scan and MRI of the skull revealed a soft tissue mass invading the cranial cavity just beyond the lytic lesion, compressing the brain parenchyma, the upper sagittal sinus and the right ventricle. Skeletal scintigraphy with technetium 99m showed a positive uptake of the proximal left femur and the distal right femur. Chest CT scan detected multiple bilateral lesions of the lung. Blood tests were normal, HIV 1 and 2 were negative. Histopathological results of the soft tissue biopsy were compatible with an unspecified high-grade sarcoma and a second opinion was requested to Prof. Christopher Fletcher Harvard Medical School, USA. His examination was compatible with a high-grade spindle cell malignant neoplasm with extensive necrosis. Immunohistochemistry showed multifocal positivity for smoth muscle actin, numerous scattered cells were positive fordesmin andalsowith quiteextensivekeratinpositivity,while caldesmon, CD34 and epithelial membrane antigen were negative, suggesting a high grade leiomyosarcoma. A high complex karyotype was detected by genetic analysis. Despite chemotherapy and radiotherapy, the disease progressed and he died five months after the diagnosis. Neuro-Oncology 18:iii7–iii15, 2016. doi:10.1093/neuonc/now067.17 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.