Abstract
Leptomeningeal disease (LMD) is an umbrella term used to refer to cancer involvement of the arachnoid and pia mater. Over the past four decades, the incidence of LMD has increased, largely as a result of new diagnostic modalities, improved therapies for systemic cancer, and increased clinical awareness. LMD can be divided into three categories: LMD resulting from solid tumors, LMD resulting from hematogenous tumors, and LMD resulting from primary brain tumors. Patients with LMD due to solid tumors usually present with a random and highly asymmetric distribution of symptoms. LMD due to hematogenous cancers is often identified during periods of remission or systemic disease inactivity and typically presents with a higher frequency of cranial nerve signs than is seen with LMD due to solid tumors. LMD arising from primary brain tumors usually occurs late in the course of the disease. The diagnostic gold standard for LMD is a positive cerebrospinal fluid cytology finding. Treatment of LMD is aimed at preventing neurologic deterioration and improving patient survival. Patients in the good-risk group may proceed to a cerebrospinal fluid flow study followed by initial intrathecal or intraventricular chemotherapy along with fractionated external-beam radiation therapy. Patients in the poor-risk group may benefit from more targeted fractionated external-beam radiation therapy delivered to symptomatic sites only, along with supportive care. Despite aggressive treatment protocols, the overall prognosis of patients with LMD remains poor; most studies report a median survival of 2–3 months.
Keywords
- Cranial Nerve
- Optic Neuropathy
- Primary Central Nervous System Lymphoma
- Primary Brain Tumor
- Leptomeningeal Metastasis
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Camoriano, G.D., Raghunath, A., Schiffman, J.S. (2010). Leptomeningeal Disease. In: Esmaeli, B. (eds) Ophthalmic Oncology. M.D. Anderson Solid Tumor Oncology Series, vol 6. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-0374-7_32
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