Cerebrospinal fluid-disseminated meningioma

Background: Intracranial meningiomas are common and comprise 20% of all primary brain tumors. Meningiomas infrequently metastasize; however, to the authors' knowledge there are limited data regarding the spread of disease through cerebrospinal fluid (CSF).

Methods: Eight of 200 consecutive patients (4%) with meningiomas manifested CSF dissemination. CSF cytology was positive in all patients, and neuroradiographic studies were consistent with CSF dissemination in eight patients. The patients (6 women and 2 men) ranged in age from 24-87 years (mean age, 52 years). All patients had undergone prior surgery (range, one to five surgeries; median, two surgeries), radiotherapy (involved-field radiotherapy in seven patients and stereotactic radiotherapy in six patients), and chemotherapy (hydroxyurea in eight patients). Multiple sites of metastases were seen in all patients and were both within the nervous system (subarachnoid or ventricular tumor: intracranial in eight patients, spinal cord in four patients) and extraneural (subcutaneous, cervical lymph nodes, orbit, or pulmonary in five patients). Treatment utilized both systemic chemotherapy (temozolomide in four patients, irinotecan in three patients, hydroxyurea in three patients, interferon-alpha in two patients, and doxorubicin plus ifosfamide in one patient) and intraventricular chemotherapy (liposomal cytosine arabinoside in seven patients, thiotepa in one patient, and busulfan in one patient).

Results: Treatment-related toxicity was seen in eight patients, including chemical meningitis in eight patients (Grade 2), neutropenia in five patients (Grade 2 in four patients and Grade 3 in one patient), fatigue in one patient (Grade 2), and gastrointestinal toxicity in one patient (Grade 2). The best response was stable disease in seven patients and progressive disease in one patient. The response duration ranged from 2-31 months (median, 3.5 months). The median survival was 5.5 months, and 3 patients were alive with disease at the time of last follow-up.

Conclusions: The treatment of CSF-disseminated meningioma, although feasible and comparatively nontoxic, was associated with modest outcomes despite combined systemic and intraventricular chemotherapy.