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Published on: March 7, 2017
Desmoplastic infantile ganglioglioma: a questionably benign tumour
A Taranath1, A Lam, C K F Wong
1Department of Medical Imaging, The Children's Hospital at Westmead, Sydney, New South Wales, Australia. ajay_deepa@yahoo.com
Insights
Desmoplastic infantile gangliogliomas (DIGs) are rare childhood brain tumors. This report details two pediatric cases with multiple cerebrospinal metastases, a rare presentation for these low-grade neoplasms.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
- Neurosurgery
Background:
- Desmoplastic infantile ganglioglioma (DIG) is a rare intracranial tumor affecting childhood cerebral cortex and leptomeninges.
- DIGs are characterized by glial and ganglionic differentiation with a pronounced desmoplastic stromal reaction.
- These neoplasms are histologically classified as low-grade but exhibit questionable benignity.
Observation:
- This report presents two pediatric cases of desmoplastic infantile ganglioglioma.
- Both patients exhibited multiple cerebrospinal metastases.
- This metastatic pattern is exceptionally rare, with only two similar cases previously documented in literature.
Findings:
- The study highlights an unusual presentation of desmoplastic infantile ganglioglioma with extensive leptomeningeal spread.
- The two cases underscore the potential for aggressive behavior and widespread dissemination in these rare pediatric tumors.
- Pathological analysis confirms the characteristic dual glial and neuronal differentiation alongside a significant desmoplastic stroma.
Implications:
- The findings suggest that desmoplastic infantile ganglioglioma, despite its low-grade classification, can possess metastatic potential.
- This case series emphasizes the importance of thorough staging and monitoring for leptomeningeal disease in affected children.
- Further research into the biological behavior and prognostic factors of DIGs is warranted to optimize treatment strategies.
Abstract:
Desmoplastic infantile ganglioglioma is a rare intracranial tumour of childhood that involves the cerebral cortex and the leptomeninges. We report two patients with desmoplastic infantile gangliogliomas and multiple cerebrospinal metastases. To our knowledge, only two similar cases have been reported in the published literature. Pathologically, this rare intracranial tumour shows glial and ganglionic differentiation, accompanied by an extreme desmoplastic reaction. These are low-grade neoplasms that are questionably benign.
