Does a patient with a meningioma on the brain have an increased risk of developing a meningioma on the spine?

Risk of Spinal Meningioma in Patients with Brain Meningioma

Patients with brain meningiomas should undergo baseline MRI of the spine to evaluate for concurrent spinal meningiomas, as they have an increased risk of developing meningiomas in the spine, particularly if they have an underlying cancer predisposition syndrome. 1, 2

Genetic and Clinical Associations

Meningiomas in multiple locations are frequently associated with underlying genetic conditions:

• Cancer Predisposition Syndromes (CPS): Meningiomas in children and young adults are frequently associated with cancer predisposition syndromes, including:

  • Neurofibromatosis Type-2 (NF2)
  • RTPS-1
  • NBCCS/Gorlin Syndrome
  • PTEN Hamartoma Tumor Syndrome/Cowden Syndrome
  • Werner Syndrome
  • Schwannomatosis with DGCR8 microprocessor defect 1

• Specific Genetic Mutations:

  • SMARCE1 mutations: Associated with clear cell-type meningioma (CCM) that can arise in both brain and spine, with up to 60% recurrence rates and potential for CNS dissemination 1
  • LZTR1 mutations: Associated with meningiomas that can occur in both brain and spine 1
  • BAP1 mutations: Associated with meningiomas with rhabdoid and/or papillary morphology 1

Surveillance Recommendations

For patients with brain meningioma, the following surveillance approach is recommended:

1. Initial Evaluation:

   • Complete spine MRI at the time of brain meningioma diagnosis to identify any additional schwannomas or meningiomas 2
   • Genetic evaluation for underlying cancer predisposition syndromes, particularly if the patient is young or has multiple meningiomas 1, 2

2. Ongoing Surveillance:

   • For patients with SMARCE1 mutations: Neurologic examination and MRI of brain and spine yearly until age 30, then every 2-3 years thereafter 1
   • For patients with LZTR1 mutations: Baseline MRI brain and spine at diagnosis, then every 2-3 years beginning at age 15-19 years 1, 2
   • For patients with multiple schwannomas: Regular surveillance imaging of both brain and spine 2

Risk Factors for Multiple Meningiomas

The presence of multiple factors increases the likelihood of concurrent or future spinal meningiomas:

• Young age at diagnosis of brain meningioma (particularly <30 years) 2
• Family history of meningiomas or related tumors 2
• Presence of two or more schwannomas on initial imaging 2
• Specific histological subtypes (clear cell, rhabdoid, or papillary features) 1

Clinical Implications

The presence of multiple meningiomas has important implications:

• Higher risk of recurrence and progression, particularly with certain genetic mutations 1
• Need for more intensive surveillance protocols 1, 2
• Consideration of genetic counseling for patients and family members 2
• Potential impact on surgical planning and approach 3, 4

Pitfalls and Caveats

• Spinal meningiomas can be easily missed if dedicated spine imaging is not performed 2
• Extradural spinal meningiomas (approximately 7% of all spinal meningiomas) can mimic metastatic tumors or schwannomas 5, 6
• Ossified spinal meningiomas (1-5% of spinal meningiomas) can be challenging to resect completely due to their hard consistency 3
• Ventral or ventrolateral location, large tumors, and T2 cord signal changes are associated with poor functional outcomes 4

By understanding the association between brain and spinal meningiomas and implementing appropriate surveillance, clinicians can improve early detection and optimize management strategies to reduce morbidity and mortality in affected patients.