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

• Published in: Brain tumor pathology Vol. 31; no. 1; pp. 47 - 50
• Main Authors: Aryan, Saritha, Ghosal, Nandita, Aziz, Zarina A., Hegde, A. S., Dadlani, Ravi
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 2014; Springer Nature B.V
• Subjects: Brain cancer; Brain tumors; Calcification; Cancer Research; Case Report; Case reports; Cysts; Ependymoma - complications; Ependymoma - diagnosis; Ependymoma - pathology; Fatal Outcome; Humans; Hydrocephalus; Hydrocephalus - etiology; Hydrocephalus - therapy; Infant; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Neurology; Neurosurgery; Oncology; Pathology; Pediatrics; Pneumonia; Spinal cord; Spinal Cord Neoplasms - complications; Spinal Cord Neoplasms - diagnosis; Spinal Cord Neoplasms - pathology; Syringomyelia - diagnosis; Syringomyelia - etiology; Syringomyelia - pathology; Tumors; Ventriculoperitoneal Shunt - adverse effects; Congenital hydrocephalus; Infant; Holocord; Ependymoma; Communicating hydrocephalus
• ISSN: 1433-7398; 1861-387X
• DOI: 10.1007/s10014-013-0145-1

We present a case of infantile holocord ependymoma in a 4-month-old boy who presented with infection of ventriculoperitoneal shunt done elsewhere for a communicating hydrocephalus. On magnetic resonance imaging, a diffuse holocord T2-hyperintense, T1-hypointense intramedullary bulky lesion with syringomyelia in the cervical level was seen. To the best of our knowledge, this is the first case of infantile holocord ependymoma. As the extent of morbidity associated with a spinal cord tumor is high, an increased level of suspicion and the need for a complete spinal cord screening in a case of infantile hydrocephalus without obvious clinical and radiological evidence of intracranial abnormality is emphasized.