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Published on: February 22, 2015
Long-term survival of an infant with gliomatosis cerebelli
Lucy B Rorke-Adams1, Harold Portnoy
1Department of Pathology and Laboratory Medicine, Division of Neuropathology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA. Rorke@email.chop.edu
Insights
Gliomatosis cerebri, a rare brain tumor, can occur in children. This case study details a young boy
Area of Science:
- Neuro-oncology
- Pediatric neurosurgery
Background:
- Gliomatosis cerebri is a rare central nervous system neoplasm primarily affecting adults.
- It typically originates in the cerebrum but can spread to other brain regions and the spinal cord.
Observation:
- This report details a rare pediatric case of gliomatosis cerebri originating in the cerebellum of a 13-month-old boy.
- The tumor extended to the thalamus, with growth cessation observed at 3 years and 10 months.
Findings:
- The patient underwent two partial tumor resections.
- No radiotherapy or chemotherapy was administered.
- The patient achieved independent function and is now 21 years old.
Implications:
- This case highlights the potential for long-term survival and independent function in pediatric gliomatosis cerebri patients with limited surgical intervention.
- It suggests that aggressive adjuvant therapies may not always be necessary for favorable outcomes in select pediatric cases.
- Further research into the natural history and prognostic factors of pediatric gliomatosis cerebri is warranted.
Abstract:
Gliomatosis cerebri is an uncommon but well-established central nervous system neoplasm that occurs primarily in adults. Although the neoplastic process typically arises in the cerebrum, it often spreads to brainstem, cerebellum, or even the spinal cord. In this report the authors document the surgical treatment of a 13-month-old boy whose tumor arose in the cerebellum and over time extended to the thalamus where its growth halted at age 3 years and 10 months. Aside from 2 partial resections the patient underwent neither radiotherapy nor chemotherapy. He is now 21 years old and functions independently.
