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Optic pathway tumor in children: Toward a new classification for neurosurgical use
I Stella1, M Helleringer1, A Joud1
1Pediatric Neurosurgical Unit, Nancy Regional University Hospital, University of Lorraine, France.
Object:
Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification.
Methods:
We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification.
Results:
Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests).
Conclusion:
Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system.
Insights
A new classification system for pediatric optic pathway tumors (OPT) aids surgical decision-making. This system helps identify cases where surgery is beneficial, particularly for managing intracranial hypertension (IH) and improving outcomes.
Area of Science:
- Pediatric neurosurgery
- Oncology
- Ophthalmology
Background:
- Optic pathway tumors (OPT) pose significant challenges in pediatric neurosurgery.
- The role of surgery in OPT management is debated due to risks, with chemotherapy often prioritized.
- Surgery remains crucial for diagnosis, managing intracranial hypertension (IH), and complementing therapies for tumor relapse.
Purpose of the Study:
- To propose a simple, practical, and reproducible classification system for pediatric optic pathway tumors.
- To standardize the selection criteria for surgical intervention in OPT cases.
- To correlate tumor characteristics with treatment decisions and outcomes.
Main Methods:
- Retrospective analysis of 38 pediatric patients with OPT treated between 1990 and 2018.
- Development of a new classification system based on MRI analysis, categorizing tumors into four groups based on location and extension.
- Evaluation of patient data, including treatments and outcomes, in relation to the proposed classification.
Main Results:
- Twenty pediatric patients underwent surgery for OPT.
- Permanent clinical impairment occurred in 25% of operated patients; visual improvement was noted in one patient.
- Groups 3-4 (hypothalamo-chiasmatic lesions) showed significantly higher prevalence of IH and were more likely to receive first-line surgical treatment compared to groups 1-2 (P<0.001).
Conclusions:
- Surgery can be a valuable adjunct therapy for OPT, with manageable risks of iatrogenic damage.
- Surgical intervention is essential for OPT cases presenting with intracranial hypertension (IH), particularly those in groups 3 and 4.
- The proposed classification system can effectively guide the selection of eligible patients for surgical treatment of OPT.
