Leptomeningeal metastases

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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~~originate from 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~~Refer to intradural extramedullary metastases for a ~~particular~~ discussion ~~related to the~~of 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~~occur in the context of a widespread metastatic disease ~~(haematogenous~~, likely by haematogenous spread. Over 50% of cases have concurrent brain (parenchymal) metastases ¹³. The most common primary sites are:

breast cancer~~: most common~~ (particularly infiltrating lobular carcinoma)
lung cancer~~: most common~~
melanoma
~~lymphoma~~
~~leukaemia~~
gastrointestinal (gastric carcinoma^4,5, colorectal cancer⁸)
hematologic: lymphoma / leukaemia

Less common, but reported primary sites include:

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~~Leptomeningeal metastases have a poor prognosis with patients usually succumbing within a few months~~, whereas, with~~ (median overall survival 2.4 months ¹³). With treatment, that time may be extended up to 6-10 months ^2,3. Treatment can consist of ³:

intrathecal chemotherapy
radiotherapy

Resection is usually inappropriate due to the presence of widespread metastases.

Differential diagnosis

leptomeningeal inflammation: leptomeningitis
slow flow in vessels
propofol, high oxygen tension, subarachnoid blood can all elevate sulcal FLAIR signal

-<p><strong>Leptomeningeal metastases</strong>, also know as <strong>carcinomatous meningitis</strong>, refers to the spread of malignant cells through the CSF space. These cells can be originated both in primary CNS tumours (e.g. <a href="/articles/leptomeningeal-drop-metastases">drop-metastases</a>), as well as from distant tumours that have metastasised via haematogenous spread.</p><p>This article has a focus on subarachnoid space involvement. Please refer on <a href="/articles/intradural-extramedullary-metastases">intradural extramedullary metastases</a> for a particular discussion related to the leptomeningeal metastases in the spine. For other intracranial metastatic locations, please refer to the main article on <a href="/articles/intracranial-metastases">intracranial metastases</a>. </p><h4>Epidemiology</h4><p>The demographics follow those of the underlying malignancy.</p><h4>Clinical presentation</h4><p>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 <sup>3</sup>. Meningism is only present in a minority of patients (13% <sup>3</sup>).</p><h4>Pathology</h4><p>The primary intracerebral malignancies that may cause metastases to the subarachnoid space are:</p><ul>
+<p><strong>Leptomeningeal metastases</strong>, also know as <strong>carcinomatous meningitis</strong>, refers to the spread of malignant cells through the CSF space. These cells can originate from primary CNS tumours (e.g. <a href="/articles/leptomeningeal-drop-metastases">drop-metastases</a>), as well as from distant tumours that have metastasised via haematogenous spread.</p><p>This article has a focus on subarachnoid space involvement. Refer to <a href="/articles/intradural-extramedullary-metastases">intradural extramedullary metastases</a> for a discussion of leptomeningeal metastases in the spine. For other intracranial metastatic locations, please refer to the main article on <a href="/articles/intracranial-metastases">intracranial metastases</a>. </p><h4>Epidemiology</h4><p>The demographics follow those of the underlying malignancy.</p><h4>Clinical presentation</h4><p>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 <sup>3</sup>. Meningism is only present in a minority of patients (13% <sup>3</sup>).</p><h4>Pathology</h4><p>The primary intracerebral malignancies that may cause metastases to the subarachnoid space are:</p><ul>
~~-</ul><p>The vast majority of leptomeningeal metastases originates from a widespread metastatic disease (haematogenous spread). The most common primary sites are: </p><ul>~~
+</ul><p>The vast majority of leptomeningeal metastases occur in the context of a widespread metastatic disease, likely by haematogenous spread. Over 50% of cases have concurrent brain (parenchymal) metastases <sup>13</sup>. The most common primary sites are: </p><ul>
~~-<a href="/articles/breast-cancer">breast cancer</a>: most common</li>~~
~~-<li>~~
~~-<a href="/articles/lung-cancer-3">lung cancer</a>: most common</li>~~
+<a href="/articles/breast-cancer">breast cancer</a> (particularly <a title="Infiltrating lobular carcinoma" href="/articles/invasive-lobular-carcinoma-of-the-breast">infiltrating lobular carcinoma</a>)</li>
+<li><a href="/articles/lung-cancer-3">lung cancer</a></li>
~~-<li><a href="/articles/lymphoma">lymphoma</a></li>~~
~~-<li><a href="/articles/leukaemia">leukaemia</a></li>~~
~~-<li>~~
~~-<a href="/articles/gastric-adenocarcinoma">gastric carcinoma</a> <sup>4,5</sup>~~
+<li>gastrointestinal (<a href="/articles/gastric-adenocarcinoma">gastric carcinoma</a> <sup>4,5</sup>, <a href="/articles/colorectal-carcinoma">colorectal cancer</a> <sup>8</sup>)</li>
+<li>hematologic: <a href="/articles/lymphoma">lymphoma</a> / <a href="/articles/leukaemia">leukaemia</a>
+</ul><p>Less common, but reported primary sites include:</p><ul>
~~-<a href="/articles/colorectal-carcinoma">colorectal cancer</a> <sup>8</sup>~~
~~-</li>~~
~~-<li>~~
~~-<li> <a 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> <a href="/articles/adrenal-cortical-carcinoma-1">adrenal cortical carcinoma</a> <sup>11</sup>~~
+<li>
+<a href="/articles/adrenal-cortical-carcinoma-1">adrenal cortical carcinoma</a> <sup>11</sup>
~~-<li> <a title="Oesophageal cancer" href="/articles/oesophageal-carcinoma-1">oesophageal cancer</a> <sup>12</sup>~~
+<li>
+<a href="/articles/oesophageal-carcinoma-1">oesophageal cancer</a> <sup>12</sup>
-</ul><h4>Treatment and prognosis</h4><p>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 <sup>2,3</sup>. Treatment can consist of <sup>3</sup>:</p><ul>
+</ul><h4>Treatment and prognosis</h4><p>Leptomeningeal metastases have a poor prognosis with patients usually succumbing within a few months (median overall survival 2.4 months <sup>13</sup>). With treatment, that time may be extended up to 6-10 months <sup>2,3</sup>. Treatment can consist of <sup>3</sup>:</p><ul>
~~-</ul><h4>Differential diagnosis</h4><ul>~~
+</ul><p>Resection is usually inappropriate due to the presence of widespread metastases.</p><h4>Differential diagnosis</h4><ul>

References changed:

13. Clarke J, Perez H, Jacks L, Panageas K, Deangelis L. Leptomeningeal Metastases in the MRI Era. Neurology. 2010;74(18):1449-54. <a href="https://doi.org/10.1212/WNL.0b013e3181dc1a69">doi:10.1212/WNL.0b013e3181dc1a69</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/20439847">Pubmed</a>

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