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Purpose:Primary intracranial rhabdomyosarcoma (RMS) is a rare tumor in childhood that is found in various locations in the central nervous system.This report presents such a case.Method: This 6 year old boy presented with severe headache, emesis, and double vision.On examination, left 6th and 7th cranial nerve palsy was noted.MRI revealed a temporal mass extending to frontal lobe, basal ganglia, sylvian fissure and obliterating the left ventricle.The patient underwent a left frontotemporal craniotomy with gross total tumor resection.The pathological diagnosis was embryonal RMS.Postoperatif MRI revealed left hipocampal residual nodule.Computerized tomography (CT) of chest showed metastatic nodules in both lungs.Radiation therapy with a dose of 54 Gy was applied to whole brain.He concurrently received chemotherapy consisting of ifosfamide, carboplatin and etoposide.At the end of induction chemotherapy, CT revealed considerable resolution of the lung metastasis and also MRI revealed resolution of the hipocampal residual nodule.After 6 months of remission period, cranial MRI revealed recurrence of the hipocampal nodul.Total reexcision of tumor was performed and histopathological examination revealed embryonal RMS.Post-operative MRI revealed a new periventricular lesion.MRI-Spectroscopy also supported this finding.A regimen of chemotherapy including intravenous vincristine, cyclophosphamide, adriamycin and cisplatin and intrathecal methotrexate, cytosine arabinoside and prednisolon was administered.Under chemotherapy, cervical lymph node metastasis was detected.Patient's family rejected further treatment and he is still alive with progresive disease after 30 months of diagnosis.Results: Primary RMS of the central nervous system (CNS) is rare in children.The outcome in the majority of cases is poor and many cases are associated with early mortality as in our case Conclusion: In spite of advances in the treatment of RMS, primary brain localization has still very poor prognosis.Novel approaches for treatment of special localizations are required.
Poele et al. (Mon,) studied this question.