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Sjogren-Larrson syndrome
Joseph Junewick, MD FACR
over 11 years ago
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Leptomeningeal Carcinomatosis

Case Detail

Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 11 years ago
Updated: over 11 years ago
Tags: PEDS
Modality/Study Types: MR
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6 year old male with progressive bulbar palsies, lethargy and paresis.

Case Images


Primary Primitive Neuroectodermal Tumor of the Leptomeninges

Clinical Notes

Leptomeningeal biopsy revealed primitive neuroectodermal type tumor infiltration.


MR – Post-gadolinium axial T1 images demonstrate diffusely enhancing and thickened leptomeninges with no parenchymal lesion.


Primitive neuroectodermal tumor (PNET) is rare and primary leptomenigeal involvement is even more rare.

Leptomeningeal carcinomatosis occurs in ~5% of patients with cancer; the increased frequency of diagnosis is related to improved cancer survival and imaging. Leptomeningeal carcinomatosis is usually secondary to hematogenous or direct extension, most often related to breast cancer, lung cancer and melanoma.

Symptoms of leptomeningeal carcinomatosis include cranial neuropathies, focal neurologic deficits related to direct tumor invasion of the brain or spinal cord, disturbed vascular supply, or general brain dysfunction. Diagnosis is made by CSF cytology. Imaging may show discrete extra-axial masses, diffuse leptomeningeal enhancement and/or hydrocephalus.


Begemenm M et al. Primary Leptomeningeal Primitive Neuroectodermal Tumor. J Neuro-oncology (2003); 63(3):299-303.

Grossman SA and Kraback MJ. Leptomeningeal Carcinomatosis. Cancer Treat Rev (1999); 25(2):103-119.

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