1. Individual +/- family history fulfils clinical criteria for Neurofibromatosis Type 2
a. Bilateral vestibular schwannomas, OR
b. Unilateral vestibular schwannoma AND ≥2 NF2 associated features (meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities/cataract) OR
c. ≥ 1 of unilateral vestibular schwannoma, meningioma, schwannoma, glioma, neurofibroma, multiple meningiomas, posterior subcapsular lenticular opacities/cataract AND ≥ 1 first / second degree relative with a vestibular schwannoma OR
d. Multiple Meningiomas AND ≥2 NF2 associated features (schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities/cataract) OR
e. Unilateral Vestibular Schwannoma AND multiple meningiomas

2. Unilateral Vestibular Schwannoma AND a non-intradermal schwannoma without other NF2-features
3. Schwannomatosis:
a. Two or more non-intradermal schwannomas (at least one biopsy-confirmed) OR
b. One pathologically confirmed schwannoma, unilateral vestibular schwannoma, or intracranial
meningioma AND ≥1 FDR with Schwannomatosis
4. Schwannoma diagnosed <30years 5. ≥2 meningiomas 6. Any clear Cell Meningioma Extent of testing 1. Patients fulfilling criterion 1 should have NF2 testing only 2. Patients fulfilling criterion 2 should have testing of NF2 AND LZTR1 3. Patients fulfilling criterion 3 should have testing of NF2, LZTR1, SMARCB1 and DGCR8 4. Patients fulfilling criterion 4 should have testing of NF2, LZTR1, SMARCB1 5. Patients fulfilling criterion 5 should have testing of NF2, SMARCE1, SUFU 6. Patients fulfilling criterion 6 should have testing of SMARCE1 Note Tumour-based testing of NF2 checking for mosaicism should be offered in the following circumstances: 1. Patients fulfilling criterion 1 in whom germline NF2 testing is uninformative 2. Patients with two or more NF2-related tumours not otherwise fulfilling criteria 1-6 3. Patients fulfilling criterion 3 in whom testing of NF2, LZTR1, SMARCB1 and DGCR8 is uninformative

1. Individual +/- family history fulfils clinical criteria for Neurofibromatosis Type 2
a. Bilateral vestibular schwannomas, OR
b. Unilateral vestibular schwannoma AND ≥2 NF2 associated features (meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities/cataract) OR
c. ≥ 1 of unilateral vestibular schwannoma, meningioma, schwannoma, glioma, neurofibroma, multiple meningiomas, posterior subcapsular lenticular opacities/cataract AND ≥ 1 first / second degree relative with a vestibular schwannoma OR
d. Multiple Meningiomas AND ≥2 NF2 associated features (schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacities/cataract) OR
e. Unilateral Vestibular Schwannoma AND multiple meningiomas

2. Unilateral Vestibular Schwannoma AND a non-intradermal schwannoma without other NF2-features
3. Schwannomatosis:
a. Two or more non-intradermal schwannomas (at least one biopsy-confirmed) OR
b. One pathologically confirmed schwannoma, unilateral vestibular schwannoma, or intracranial
meningioma AND ≥1 FDR with Schwannomatosis
4. Schwannoma diagnosed <30years 5. ≥2 meningiomas 6. Any clear Cell Meningioma Extent of testing 1. Patients fulfilling criterion 1 should have NF2 testing only 2. Patients fulfilling criterion 2 should have testing of NF2 AND LZTR1 3. Patients fulfilling criterion 3 should have testing of NF2, LZTR1, SMARCB1 and DGCR8 4. Patients fulfilling criterion 4 should have testing of NF2, LZTR1, SMARCB1 5. Patients fulfilling criterion 5 should have testing of NF2, SMARCE1, SUFU 6. Patients fulfilling criterion 6 should have testing of SMARCE1 Note Tumour-based testing of NF2 checking for mosaicism should be offered in the following circumstances: 1. Patients fulfilling criterion 1 in whom germline NF2 testing is uninformative 2. Patients with two or more NF2-related tumours not otherwise fulfilling criteria 1-6 3. Patients fulfilling criterion 3 in whom testing of NF2, LZTR1, SMARCB1 and DGCR8 is uninformative