Atypical Meningioma in a Young Child: Case Report and Management

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What are the key histological features that differentiate atypical meningiomas from typical meningiomas, adn how do these features influence treatment decisions in pediatric cases?

Understanding Pediatric Meningiomas

Meningiomas are typically considered tumors of adulthood, arising from the meninges – the membranes surrounding the brain and spinal cord. Their occurrence in children, notably atypical meningiomas, is exceptionally rare, representing less than 1% of all pediatric brain tumors. This rarity frequently enough leads to diagnostic delays and challenges in management. The term “atypical” signifies histological features suggesting a more aggressive behavior than typical meningiomas. Keywords: pediatric meningioma, childhood brain tumor, atypical meningioma, rare pediatric tumor.

Case Presentation: A 7-Year-Old with Progressive Symptoms

We present the case of a 7-year-old female who initially presented with a six-month history of progressively worsening headaches, accompanied by intermittent nausea and vomiting. Neurological examination revealed mild left-sided weakness. Initial imaging with MRI demonstrated a well-defined extra-axial mass in the right frontal lobe, enhancing with contrast. Differential diagnoses included meningioma, hemangiopericytoma, and other pediatric brain tumors.

Initial MRI Findings: 3.5 cm mass,homogenous enhancement,mild surrounding edema.

Patient History: No family history of neurofibromatosis or other relevant genetic syndromes.

Pre-operative Assessment: Complete blood count,metabolic panel,and coagulation studies were within normal limits.

Histopathological Diagnosis & Atypical Features

Following surgical resection, histopathological examination confirmed the diagnosis of atypical meningioma, WHO grade II.Key atypical features observed included:

1. Increased Mitotic Activity: More than 15 mitoses per 10 high-power fields.
2. Nuclear Atypia: Presence of enlarged, irregular nuclei.
3. Microvascular Proliferation: Clusters of small, abnormal blood vessels.
4. Necrosis: Areas of cell death within the tumor.

These features distinguish atypical meningiomas from benign meningiomas and indicate a higher risk of recurrence and aggressive behavior. Keywords: meningioma histology, WHO grade II meningioma, atypical features, mitotic count, nuclear atypia.

Surgical Management: Resection Challenges

Gross total resection (GTR) is the primary goal of surgical intervention for meningiomas, even in atypical cases. Though, in children, achieving GTR can be challenging due to the tumor’s location, proximity to critical structures, and the developing brain. In this case, a right frontal craniotomy was performed.

Surgical Considerations: Minimizing brain retraction, preserving eloquent cortex, and careful dissection from surrounding vessels were paramount.

Extent of Resection: Near-total resection (95%) was achieved, with residual tumor adhering to the superior sagittal sinus.

Post-operative Neurological Status: The patient experienced transient worsening of left-sided weakness, which gradually improved over several weeks with physical therapy.

Adjuvant Therapy: Radiation and Chemotherapy Considerations

Given the atypical histology and incomplete resection, adjuvant therapy was considered. The decision-making process involved a multidisciplinary team including neuro-oncologists, radiation oncologists, and neurosurgeons.

Radiation Therapy: Stereotactic radiosurgery (SRS) or fractionated radiotherapy were options. SRS was favored due to its ability to deliver a high dose of radiation to a precisely defined target,minimizing damage to surrounding normal brain tissue. Keywords: pediatric radiation oncology, stereotactic radiosurgery, fractionated radiotherapy.

Chemotherapy: The role of chemotherapy in atypical meningiomas remains controversial.Though,given the potential for recurrence,chemotherapy with temozolomide was discussed and ultimately initiated as a precautionary measure.

* Treatment Protocol: The patient received 60 Gy of fractionated radiation therapy over 30 fractions, followed by adjuvant temozolomide for six months.

Follow-Up and Long-Term Outcomes

the patient has been followed clinically and radiologically every three months for the past two years. MRI scans demonstrate stable residual disease. Neurological