Spinal primitive neuroectodermal tumor - infant

Case contributed by Ian Bickle


Bilateral flaccid lower limb weakness. Initially thought to be Gullian Barre. No response to treatment. Cause?

Patient Data

Age: 2 months
Gender: Male

Expansile intramedullary mildly hyperintense, T1 isointense mass occupying the spinal canal from T9 down to L5. The tumor measures 2.1 cm in the axial plane and extends 7.2 cm craniocaudally. The conus medullaris is inseparable from the mass. Tiny foci of high signal centrally within the mass. Minimal contrast enhancement.

Small amount of scalloping of the posterior aspects of the L3-L5 vertebral bodies.

No proximal syrinx. No synchronous upper cord lesion.

Distended bladder in keeping with urinary retention.

The skin and subcutaneous tissues are normal. No spina bifida.

Comment: Appearances most in keeping with a primary spinal cord tumor

The differential diagnoses include: spinal ependymoma, astrocytoma, and less likely lymphoma/leukemia

Case Discussion

A surgical biopsy was performed from a medullary mass inseparable from the cord and cauda equina.

HISTOLOGY:  Primitive neuroectodermal tumor

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