Leptomeningeal disease: current diagnostic and therapeutic strategies

// Gautam Nayar 1, 2, 3 , Tiffany Ejikeme 1, 2, 3 , Pakawat Chongsathidkiet 1, 2, 6 , Aladine A. Elsamadicy 1, 2, 3 , Kimberly L. Blackwell 4 , Jeffrey M. Clarke 5 , Shivanand P. Lad 3 and Peter E. Fecci 1, 2, 3, 6 1 Duke Brain Tumor Immunotherapy Program, Department of Neurosurgery, Duke University Medical Center, Durham, NC, USA 2 The Preston Robert Tisch Brain Tumor Center, Duke University Medical Center, Durham, NC, USA 3 Department of Neurosurgery, Duke University Medical Center, Durham, NC, USA 4 Department of Radiation Oncology, Duke University Medical Center, Durham, NC, USA 5 Division of Medical Oncology, Duke University Medical Center, Durham, NC, USA 6 Department of Pathology, Duke University Medical Center, Durham, NC, USA Correspondence to: Peter E. Fecci, email: Peter.fecci@duke.edu Keywords: leptomeningeal disease, neoplastic meningitis, leptomeningeal carcinomatosis, intrathecal chemotherapy, radiation therapy Received: May 10, 2017      Accepted: July 20, 2017      Published: August 16, 2017 ABSTRACT Leptomeningeal disease has become increasingly prevalent as novel therapeutic interventions extend the survival of cancer patients. Although a majority of leptomeningeal spread occurs secondary to breast cancer, lung cancer, and melanoma, a wide variety of malignancies have been reported as primary sources. Symptoms on presentation are equally diverse, often involving a combination of neurological deficits with the possibility of obstructive hydrocephalus. Diagnosis is definitively made via cerebrospinal fluid cytology for malignant cells, but neuro-imaging with high quality T1-weighted magnetic resonance imaging can aid diagnosis and localization. While leptomeningeal disease is still a terminal, late-stage complication, a variety of treatment modalities, such as intrathecal chemotherapeutics and radiation therapy, have improved median survival from 4–6 weeks to 3–6 months. Positive prognosticative factors for survival include younger age, high performance scores, and controlled systemic disease. In looking to the future, diagnostics that improve early detection and chemotherapeutics tailored to the primary malignancy will likely be the most significant advances in improving survival.