From the Guidelines
The primary treatment approach for multinodular and vascular neuronal tumors should involve surgical resection, with complete removal recommended whenever possible to offer the best chance for cure and prevent recurrence. This approach is based on the most recent guidelines and evidence, including the updated EANO guideline on rational molecular testing of gliomas, glioneuronal, and neuronal tumors in adults for targeted therapy selection-update 1 1.
Key Considerations
- Surgical resection is the primary treatment, with complete removal being the goal whenever possible.
- For tumors in accessible locations, a craniotomy may be performed to access and remove the tumor while preserving surrounding healthy brain tissue.
- In cases where complete resection isn't feasible, partial removal (subtotal resection) may be performed to reduce symptoms and tumor burden.
- Following surgery, radiation therapy, including stereotactic radiosurgery, might be used as an adjuvant treatment for residual tumor tissue or for tumors with concerning pathological features.
- Chemotherapy is generally less effective for neuronal tumors but may be considered in specific cases, especially for aggressive variants or recurrent disease.
Treatment Individualization
The treatment approach is individualized based on tumor size, location, patient's age, overall health, and the specific histological subtype of the neuronal tumor, as these factors significantly influence treatment decisions and prognosis. Regular follow-up with MRI imaging is essential to monitor for recurrence, typically every 3-6 months initially, then annually if stable. The guideline from 1 emphasizes the importance of molecular testing for targets like BRAF V600E mutations, which have proven clinical benefit, although the evidence for other targets and tumor agnostic treatments in adult brain tumors is still limited.
Multidisciplinary Approach
A multidisciplinary approach, as suggested by guidelines such as those from EANO and ESMO 1, is crucial for the management of these tumors, involving discussion at a dedicated tumor board with participation of colleagues experienced in the management of CNS tumors. This ensures that therapeutic decisions are based on a careful individualized estimation of the contributions from surgery, radiation oncology, and medical oncology.
From the Research
Multinodular and Vascular Neuronal Tumor Treatment
- Most multinodular and vacuolating neuronal tumors (MNVTs) are asymptomatic and do not require treatment, as they are often stable or non-recurrent 2
- Surgical resection is typically reserved for cases with uncontrolled seizures or atypical locations where the diagnosis is unclear 2
- Debulking surgery may be considered for certain types of tumors, but its effectiveness and safety vary depending on the specific tumor type and location 3, 4
- Radiation therapy may be used in conjunction with debulking surgery or as a standalone treatment for certain types of tumors, including spine tumors 5, 4
- Neoadjuvant therapy, including chemo(radio)therapy, may be considered for certain types of neuroendocrine neoplasms, but its efficacy and safety in MNVTs are not well established 6
Treatment Considerations
- The decision to treat MNVTs should be based on individual patient factors, including symptoms, tumor location, and overall health status 2
- A multidisciplinary approach, including surgery, radiation therapy, and medical management, may be necessary to achieve optimal outcomes for patients with MNVTs 2, 5, 4
- Further research is needed to fully understand the natural history and optimal treatment strategies for MNVTs 2, 6