Abstract

Background: Primary intracranial leiomyosarcoma (LMS) is a rare tumor of the central nervous system and therefore has only been reported sporadically. Methods: The MEDLINE database was searched for relevant case reports and series published in English from 1969 to March 2012. These papers were reviewed to identify clinical and histopathological features, treatment modalities, and prognoses of patients with primary intracranial LMS. Results: We reviewed 37 patients, including our patient, with primary intracranial LMS. Of these cases, 16 (43.2%) involved immunosuppression, 4 (10.8%) involved radiation-induced LMS, 3 (8.1%) cases involved a potential leiomyosarcomatous transformation of a brain lesion, and 7 (18.9%) cases, including the presented case, had no specific medical history. The treatment for these cases included resection (33/37, 89.2%), postoperative radiotherapy (20/37, 54.1%), and chemotherapy (7/37, 18.9%). Conclusions: The best method for the treatment of LMS could not be determined due to the limited number of cases that have been reported. However, optimized surgical resection, postoperative radiotherapy, and sarcoma-based chemotherapy may improve treatment outcomes.

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