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Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case

Saritha Aryan1, Nandita Ghosal, Zarina A Aziz

  • 1Department of Pathology and Transfusion Medicine, Sri Sathya Sai Institute of Higher Medical Sciences (SSSIHMS), EPIP Area, Whitefield, Bangalore, 560066, India.

Brain Tumor Pathology
|March 21, 2013
PubMed

Insights

This is the first reported case of a holocord ependymoma in an infant. Early spinal cord screening is crucial for infantile hydrocephalus to detect rare tumors like this one.

Area of Science:

  • Pediatric Neurosurgery
  • Neuro-oncology
  • Developmental Neurology

Background:

  • Infantile hydrocephalus often requires cerebrospinal fluid diversion, such as ventriculoperitoneal shunts.
  • Spinal cord tumors in infants are rare but can present with significant morbidity.
  • Communicating hydrocephalus can occasionally be associated with underlying spinal abnormalities.

Observation:

  • A 4-month-old boy with a history of ventriculoperitoneal shunt for communicating hydrocephalus presented with shunt infection.
  • Magnetic resonance imaging revealed a diffuse, holocord, intramedullary lesion with cervical syringomyelia.
  • The lesion was T2-hyperintense and T1-hypointense, characteristic of certain tumor types.

Findings:

  • The case represents the first documented instance of infantile holocord ependymoma.
  • The tumor extended throughout the entire length of the spinal cord.
  • Associated syringomyelia at the cervical level was noted.

Implications:

  • This case highlights the importance of considering spinal cord pathology in infants with hydrocephalus, even without clear intracranial abnormalities.
  • A high index of suspicion for spinal tumors is warranted in this population.
  • Complete spinal cord screening should be considered in infants with hydrocephalus and unexplained neurological signs or symptoms.

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