Leptomeningeal metastases

Changed by Ammar Haouimi, 1 Oct 2018

Updates to Article Attributes

Body was changed:

Leptomeningeal metastases, also know as carcinomatous meningitis, refers to the spread of malignant cells through the CSF space. These cells can be originated both in primary CNS tumours (e.g. drop-metastases), as well as from distant tumours that have metastasised via haematogenous spread.

This article has a focus on subarachnoid space involvement. Please refer on intradural extramedullary metastases for a particular discussion related to the leptomeningeal metastases in the spine. For other intracranial metastatic locations, please refer to the main article on intracranial metastases

Epidemiology

The demographics follow those of the underlying malignancy.

Clinical presentation

Clinical presentation is varied, but most commonly includes a headache, spine or radicular limb pain or sensory abnormalities, nausea and vomiting, and focal neurological deficits 3. Meningism is only present in a minority of patients (13% 3).

Pathology

The primary intracerebral malignancies that may cause metastases to the subarachnoid space are:

The vast majority of leptomeningeal metastases originates from a widespread metastatic disease (haematogenous spread). The most common primary sites are: 

Radiographic features

MRI
  • T1: usually normal
  • T1 C+ (Gd): leptomeningeal enhancement is the primary mode of diagnosis, often scattered over the brain in a 'sugar coated' manner
  • T2: usually normal
  • FLAIR
    • abnormally elevated signal within sulci 2
    • can be performed both non-contrast and post-contrast, but is slightly less specific if performed post-contrast 1

Treatment and prognosis

Untreated leptomeningeal metastases have a poor prognosis with patients usually succumbing within a few months, whereas, with treatment, that time may be extended up to 6-10 months 2,3. Treatment can consist of 3:

  • intrathecal chemotherapy
  • radiotherapy

Differential diagnosis

  • leptomeningeal inflammation: leptomeningitis
  • slow flow in vessels
  • propofol, high oxygen tension, subarachnoid blood can all elevate sulcal FLAIR signal
  • -<li> <a title="carcinoma of the uterine cervix" href="/articles/carcinoma-of-the-uterine-cervix">carcinoma of the uterine cervix </a><sup>10</sup>
  • +<li> <a href="/articles/carcinoma-of-the-uterine-cervix">carcinoma of the uterine cervix </a><sup>10</sup>
  • +</li>
  • +<li> <a title="Adrenal cortical carcinoma" href="/articles/adrenal-cortical-carcinoma-1">adrenal cortical carcinoma</a> <sup>11</sup>

References changed:

  • 11. Capone G, Della Pepa G, Sabatino G et al. A Rare Bone-Leptomeningeal Metastasis from an Adrenal Cortical Carcinoma. J Clin Neurosci. 2009;16(7):977-80. <a href="https://doi.org/10.1016/j.jocn.2008.10.013">doi:10.1016/j.jocn.2008.10.013</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/19375918">Pubmed</a>

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