Neurology· Topic 10.9 Central nervous system tumors· 10.9.7

Leptomeningeal carcinomatosis (carcinomatous meningitis)

Leptomeningeal carcinomatosis is diffuse metastatic seeding of the leptomeninges, most often by breast or lung cancer, melanoma, or leukemia/lymphoma, producing simultaneous cerebral, cranial nerve, and spinal symptoms. Diagnosis rests on CSF cytology, often requiring repeat lumbar puncture, plus contrast MRI of the brain and spine, and outcomes remain poor despite intrathecal chemotherapy and radiation.

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What this article covers

  • Overview

    Leptomeningeal carcinomatosis (carcinomatous meningitis) is diffuse metastatic seeding of the pia mater and arachnoid by a solid tumor or a hematologic malignancy, distinct from a single parenchymal…

  • Epidemiology

    Leptomeningeal metastases occur most frequently in lung and breast cancer, melanoma, leukemia, and lymphoma.

  • Risk factors

    Known metastatic breast or lung cancer, or cutaneous melanoma, the leading solid-tumor sources.

  • Pathophysiology

    Tumor cells reach the leptomeninges by hematogenous spread, by direct extension from an adjacent parenchymal, dural, or vertebral/skull-base metastasis, or by perineural spread along cranial or spinal nerves.

  • Clinical features

    The hallmark is simultaneous, multifocal involvement at more than one level of the neuraxis (cerebral, cranial nerve, spinal) occurring together, rather than a single localized deficit.

  • Differential diagnosis

    Bacterial meningitis: acute onset over hours, fever and nuchal rigidity, neutrophilic CSF pleocytosis with a positive Gram stain/culture; exclude or cover empirically first given its rapid lethality.

  • Diagnosis

    The diagnosis is confirmed by positive CSF cytology (with flow cytometry added when leukemia or lymphoma is suspected), the gold-standard basis for diagnosis.

  • Management

    First, if there is symptomatic mass effect or elevated intracranial pressure, treat with dexamethasone (10 mg IV/PO once, then 4 mg every 6 hours, tapered over 1-2 weeks as definitive therapy takes hold).

  • Complications

    Communicating hydrocephalus and sustained elevated intracranial pressure from impaired CSF resorption, which can require CSF diversion when refractory.

  • Prognosis

    Overall prognosis is generally poor; leptomeningeal carcinomatosis is a rare, high-suspicion diagnosis, and even with treatment cure is uncommon.

  • Prevention

    In acute lymphoblastic leukemia, essentially all patients receive prophylactic intrathecal chemotherapy (for example intrathecal methotrexate 12 mg) starting with the first induction cycle…

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