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Joseph Junewick, MD FACR
over 6 years ago
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Leptomeningeal PNET

Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

10 year old male with seizure.


Case Images


Diagnosis

Leptomeningeal PNET

Findings

MR – Diffuse nodular leptomeningeal enhancement; no parenchymal lesion.

Discussion

Extracerebellar PNETs are uncommon central nervous system tumors affecting children and young adults. Typically these tumors are found in the supratentorial parenchyma and rarely the spinal cord with only a few cases reported to involve the leptomeninges.

PNETs arising outside of the central nervous system most frequently involve the deep soft tissues of the trunk and lower extremities. While morphologically similar to CNS PNETs, these tumors are immunohistochemically and cytogenetically distinct exhibiting high amounts of the MIC2 antigen (CD99) and translocation of chromosomes 11 and 22 (q24;q12).

PNET of the dura represents a form of peripheral PNET. Dural PNET may be focal or diffuse. Overall, peripheral type PNETs have better prognosis than CNS PNETs, especially if they are completely resected.

Reference

Dedeurwaerdere F, Giannini C, Sciot R, Rubin BP, et al. Primary peripheral PNET/Ewing’s Sarcoma of the dura: a clinicopathological entity distinct from central PNET. Mod Pathol 2002; 15(6):673-678.



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