Clinical Presentation, Diagnosis, and Radiological Findings of Neoplastic Meningitis

Georgios Rigakos, MD; Chrysoula I. Liakou, MD; Naillid Felipe, Dennis Orkoulas-Razis, MHS; Evangelia Razis, MD, PhD

Disclosures

Cancer Control. 2017;24(1):9-21. 

In This Article

Abstract and Introduction

Abstract

Background. Neoplastic meningitis is a complication of solid and hematological malignancies. It consists of the spread of malignant cells to the leptomeninges and subarachnoid space and their dissemination within the cerebrospinal fluid.

Methods. A literature review was conducted to summarize the clinical presentation, differential diagnosis, laboratory values, and imaging findings of neoplastic meningitis.

Results. Neoplastic meningitis is an event in the course of cancer with a variable clinical presentation and a wide differential diagnosis. In general, characteristic findings on gadolinium-enhanced magnetic resonance imaging and the presence of malignant cells in the cerebrospinal fluid remain the cornerstones of diagnosis.

However, both modalities do not always confirm the diagnosis of neoplastic meningitis despite a typical clinical picture.

Conclusions. Clinicians treating patients with cancer should be aware of the possibility of neoplastic meningitis, especially when multilevel neurological symptoms are present. Neoplastic meningitis can be an elusive diagnosis, so clinician awareness is important so that this malignant manifestation is recognized in a timely manner.

Introduction

Neoplastic meningitis is a rare, late, and frequently terminal event in the course of malignancy. It occurs in 4% to 15% of solid tumors and up to 20% of lymphomas and leukemias, and it is associated with significant morbidity and short survival rates (range, several weeks to 8 months).[1,2]

Neoplastic meningitis is characterized by the diffuse involvement of the leptomeninges (pia and arachnoid), the subarachnoid space, and the cerebrospinal fluid (CSF) by malignant cells, and it occurs through the hematogenous invasion of the subarachnoid space and ventricles or through direct extension from bone and brain lesions, or, in some cases, with local spread through the dura along perineural and perivascular spaces.[3] The most frequent primary solid tumors associated with neoplastic meningitis are breast cancer, non–small-cell lung cancer, and malignant melanoma.[4] Neoplastic meningitis is typically a late event (70%) and is rarely (15%) a presenting finding in an undiagnosed malignancy.[5] However, neoplastic meningitis is expected to occur more frequently in the future because longer cancer survival times allow tumor cells the time needed to penetrate so-called central nervous system (CNS) sanctuary sites.[6] Molecular therapeutic agents, particularly monoclonal antibodies, generally do not penetrate the CNS, and their use could lead to increasing rates of neoplastic meningitis in patients receiving such agents. Use of improved neuroimaging techniques might also increase the diagnostic rate of neoplastic meningitis.

A diagnosis of neoplastic meningitis is generally suggestive of advanced disease, and the overall prognosis is affected by controlling leptomeningeal disease. However the prognosis, choice of therapy and patient outcomes depend on the state and extent of the systemic disease. Furthermore, a patient's general performance status is a core determinant of outcome, possibly because this value reflects the entire stage of the disease and its severity.

The dissemination of leptomeningeal cancer is a metastatic complication whose impact in clinical oncology is growing. Treatment advances have been hampered by difficulties in diagnosis and response assessment, as well as possible frustration from clinicians because of the poor prognosis of the disease, even with aggressive treatment.[5] However, advances in therapeutic management have been achieved.[6] In select patients, survival and time to neurological progression can be improved with therapy, making early diagnosis and a high index of suspicion very important.[7]

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