engleski

Primary intracranial pleomorphic leiomyosarcoma with rhabdoid features: a case report

Introduction: Primary intracranial leiomyosarcomas are uncommon tumors. The origin of such neoplasm could be smooth muscle cells of the vessels, pluripotent mesenchymal cells or embrionic rests from the inner layers of arachnoid and pia. They are more common in the patients who are immunosupressed and HIV infected. In this case tumor was unassociated with AIDS or immunosupression. Case report: A 58- year old male presented with 1 month history of headaches and changing mental status. MR images reveald expansive tumor mass 7 cm in diameter in right frontal lobe with characteristics of high grade glioma. Staging evaluation demostrated no other sites of disease.The patient underwent right frontal craniotomy with cranioplasty and gross total removal of tumor. Specimens sent intraoperatively to pathology were consistent with a high-grade sarcoma. Histologically tumor was composed of intersecting fascicles of elongated spindle cells with foci of anaplastic areas with collections of rhabdoid cells. Immunohistochemically tumor cells were positive for SMA, desmin and vimentin. Stains for GFAP, S-100 protein were negative. Follow up: Post-operatively the patient was neurologically intact and started radiotherapy. He is still surviving to date in stable neurogical conditon. Conclusion: This is a rare case of primary intracranial pleomorphic leiomyosarcoma with rhabdoid features in previusly healty male. Rhabdoid features are associated with aggressive biological behavior in leiomyosarcoma of external soft tissue.

primary intracranial leiomyosarcomas; AIDS; rhabdoid features

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