Leptomeningeal dissemination of anaplastic glioma: prolonged survival in two patients treated with temozolomide

Abstract

Leptomeningeal disease (LMD) occurs when tumor cells access the cerebrospinal fluid (CSF) pathways, travel to distant sites, settle and grow. The diagnosis depends upon clinical presentation, with confirmation through neuroimaging or CSF cytology. Findings consistent with LMD include contrast enhancement of the leptomeninges, subependyma, cranial and spinal nerves, and communicating hydrocephalus. Unfortunately, cytology—the diagnostic gold standard—may be negative even when disease is present. Recently, symptomatic LMD was reported in 8% of glioma patients, none of whom had positive cytology [1]. Increased LMD incidence may result from longer survivals and neuroimaging improvements. In gliomatous LMD, no established chemotherapy regimen exists, although general treatment guidelines include intrathecal chemotherapy with methotrexate, thiotepa and cytarabine. Temozolomide (TMZ) is an alkylating agent with good brain penetration and low toxicity, with a CSF:plasma drug ratio of 0.20 [2]. It is the standard of care in parenchymal gliomas, but little published experience exists for LMD. We report two anaplastic glioma patients enjoying durable responses of LMD to Temozolomide.