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Pediatric infratentorial gangliogliomas: a retrospective series
Bertrand Baussard1, Federico Di Rocco, Matthew R Garnett
1Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France.
Insights
Gangliogliomas in the posterior fossa require aggressive surgical resection. Complete tumor removal offers the best prognosis, while further surgery can be effective for progressive tumors.
Area of Science:
- Pediatric neurosurgery
- Pediatric oncology
- Neuroradiology
Background:
- Gangliogliomas are rare tumors, often presenting in children.
- Posterior fossa gangliogliomas can cause significant neurological symptoms.
- Understanding prognostic factors is crucial for effective treatment.
Purpose of the Study:
- To review clinical presentation, surgical roles, adjuvant therapy, and morbidity in pediatric posterior fossa gangliogliomas.
- To identify prognostic factors for improved outcomes in these children.
- To evaluate the effectiveness of different treatment modalities.
Main Methods:
- Retrospective review of 10 children treated for posterior fossa ganglioglioma (1991-2006).
- Analysis of clinical presentation, imaging findings (patchy enhancement), and surgical approaches (radical resection, biopsy, total excision).
- Assessment of adjuvant therapies (chemotherapy, radiotherapy) and treatment-related morbidity.
Main Results:
- Nine children presented with raised intracranial pressure; all showed preoperative enhancement on imaging.
- No neurological deterioration occurred post-surgery.
- Complete tumor excision resulted in no recurrence; radical resection without recurrence was observed in 3/7 patients.
- Tumor progression occurred in 6 children, necessitating further surgery, chemotherapy, or radiotherapy.
Conclusions:
- Patchy enhancement on imaging is characteristic of posterior fossa gangliogliomas.
- Radical resection, aiming to remove the enhancing tumor portion, can obviate the need for further treatment.
- Second surgical excision is an effective adjuvant therapy for progressive gangliogliomas.
Object:
The aim of this study was to retrospectively review the clinical presentation, the roles of surgery and adjuvant therapy, and the treatment-related morbidity in children with a ganglioglioma in the posterior fossa and to try and determine the prognostic factors.
Methods:
Between 1991 and 2006, 10 children were treated for a posterior fossa ganglioglioma at the authors' institution. The mean age of the children, the duration of symptoms prior to diagnosis, and the follow-up were 8.2, 2.4, and 3.9 years, respectively. Nine of the children presented with symptoms of raised intracranial pressure. Preoperative imaging showed enhancement in all patients; in eight it was in a patchy distribution. The operations consisted of radical resection (> 75%) in seven children, biopsy in two, and a total macroscopic excision in one.
Results:
The surgical procedure did not cause deterioration in the neurological condition in any of the children. There was no recurrence in the child who underwent total macroscopic excision of the tumor, and there has been no tumor progression in three children, two of whom have had no evidence of enhancement of the postoperative residual tumor. The tumor has progressed in six children, requiring further surgery in three, chemotherapy in four, and radiotherapy and second-line chemotherapy in one child to control the tumor.
Conclusions:
The imaging of gangliogliomas in the posterior fossa showed patchy enhancement. The patients in whom it was possible to achieve a radical resection, aimed at removing at least the enhancing portion of the tumor, have not required further treatment. A second excision, for progressive tumors, is an effective adjuvant therapy.
