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Familial tumours of the nervous system v2.14 Arina Puzriakova Panel types changed to GMS Rare Disease Virtual; GMS Rare Disease; GMS signed-off
Familial tumours of the nervous system v2.13 SUFU Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIMs #155255 & #607174) and the OMIM records were last accessed on 29 December 2025.
Familial tumours of the nervous system v2.13 SUFU Achchuthan Shanmugasundram Phenotypes for gene: SUFU were changed from to {Medulloblastoma}, OMIM:155255; {Meningioma, familial, susceptibility to}, OMIM:607174
Familial tumours of the nervous system v2.12 SMARCE1 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIM #607174) and the OMIM record was last accessed on 29 December 2025.
Familial tumours of the nervous system v2.12 SMARCE1 Achchuthan Shanmugasundram Phenotypes for gene: SMARCE1 were changed from to {Meningioma, familial, susceptibility to}, OMIM:607174
Familial tumours of the nervous system v2.11 SMARCB1 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIMs #162091 & #609322) and the OMIM records were last accessed on 29 December 2025.
Familial tumours of the nervous system v2.11 SMARCB1 Achchuthan Shanmugasundram Phenotypes for gene: SMARCB1 were changed from to {Schwannomatosis-1, susceptibility to}, OMIM:162091; {Rhabdoid tumor predisposition syndrome 1}, OMIM:609322; rhabdoid tumor predisposition syndrome 1, MONDO:0012252; SMARCB1-related schwannomatosis, MONDO:0024517
Familial tumours of the nervous system v2.10 NF2 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with MIM #101000 in OMIM and the OMIM record was last accessed on 29 December 2025.
Familial tumours of the nervous system v2.10 NF2 Achchuthan Shanmugasundram Phenotypes for gene: NF2 were changed from to Schwannomatosis, vestibular, OMIM:101000; NF2-related schwannomatosis, MONDO:0007039
Familial tumours of the nervous system v2.9 LZTR1 Achchuthan Shanmugasundram changed review comment from: Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIM #615670) and the OMIM record was last accessed on 29 December 2025.; to: Comment on phenotypes: This gene has been associated with MIM #615670 in OMIM and the OMIM record was last accessed on 29 December 2025.
Familial tumours of the nervous system v2.9 LZTR1 Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIM #615670) and the OMIM record was last accessed on 29 December 2025.
Familial tumours of the nervous system v2.9 LZTR1 Achchuthan Shanmugasundram Phenotypes for gene: LZTR1 were changed from to {Schwannomatosis-2, susceptibility to}, OMIM:615670; LZTR1-related schwannomatosis, MONDO:0014299
Familial tumours of the nervous system v2.8 CDKN2A Achchuthan Shanmugasundram Added comment: Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIMs #155601, #155755 & #606719) and the OMIM records were last accessed on 29 December 2025.
Familial tumours of the nervous system v2.8 CDKN2A Achchuthan Shanmugasundram Phenotypes for gene: CDKN2A were changed from {Melanoma and neural system tumor syndrome}, OMIM:155755; Astrocytoma; Glioblastoma; Schwannoma; Neurofibroma; Meningioma; Malignant peripheral nerve sheath tumours to {Melanoma, cutaneous malignant, 2}, OMIM:155601; {Melanoma and neural system tumor syndrome}, OMIM:155755; {Melanoma-pancreatic cancer syndrome}, OMIM:606719; melanoma, cutaneous malignant, susceptibility to, 2, MONDO:0007964; melanoma and neural system tumor syndrome, MONDO:0007967; melanoma-pancreatic cancer syndrome, MONDO:0011713
Familial tumours of the nervous system v2.7 CDKN2A Arina Puzriakova Phenotypes for gene: CDKN2A were changed from {Melanoma and neural system tumor syndrome}, OMIM:155755; MELANOMA; PANCREATIC CANCER; ASTROCYTOMA; GLIOBLASTOMA; SCHWANNOMA; NEUROFIBROMA; MENINGIOMA; MALIGNANT PERIPHERAL NERVE SHEATH TUMOURS to {Melanoma and neural system tumor syndrome}, OMIM:155755; Astrocytoma; Glioblastoma; Schwannoma; Neurofibroma; Meningioma; Malignant peripheral nerve sheath tumours
Familial tumours of the nervous system v2.6 CDKN2A Arina Puzriakova Publications for gene: CDKN2A were set to PMID 24884915; 8317504; 9622062; 10797439; 17440112; 26794401; 29263814; 28699883; 28754699; 35422439; 38936911
Familial tumours of the nervous system v2.5 CDKN2A Arina Puzriakova Tag Q2_25_ promote_green was removed from gene: CDKN2A.
Tag Q2_25_ NHS_review was removed from gene: CDKN2A.
Familial tumours of the nervous system v2.5 CDKN2A Arina Puzriakova edited their review of gene: CDKN2A: Added comment: The rating of this gene has been updated to green and the mode of inheritance set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted following NHS Genomic Medicine Service approval.; Changed rating: GREEN
Familial tumours of the nervous system v2.4 CDKN2A Arina Puzriakova Source NHS GMS was added to CDKN2A.
Source Expert Review Green was added to CDKN2A.
Rating Changed from Amber List (moderate evidence) to Green List (high evidence)
Familial tumours of the nervous system v2.3 CDKN2A Arina Puzriakova Classified gene: CDKN2A as Amber List (moderate evidence)
Familial tumours of the nervous system v2.3 CDKN2A Arina Puzriakova Added comment: Comment on list classification: This gene was reviewed by the cancer expert group and the evidence was determined as sufficient for inclusion on this panel. Therefore, it should be updated to Green at the next GMS panel update.

Melanoma and neural system tumor syndrome (OMIM:155755) associated with heterozygous variants in the CDKN2A gene is characterised by a dual predisposition to melanoma and neural system tumors, commonly astrocytoma.
Familial tumours of the nervous system v2.3 CDKN2A Arina Puzriakova Gene: cdkn2a has been classified as Amber List (Moderate Evidence).
Familial tumours of the nervous system v2.2 CDKN2A Arina Puzriakova Tag Q2_25_ promote_green tag was added to gene: CDKN2A.
Tag Q2_25_ NHS_review tag was added to gene: CDKN2A.
Familial tumours of the nervous system v2.2 CDKN2A Arina Puzriakova Phenotypes for gene: CDKN2A were changed from MELANOMA; PANCREATIC CANCER; ASTROCYTOMA; GLIOBLASTOMA; SCHWANNOMA; NEUROFIBROMA; MENINGIOMA; MALIGNANT PERIPHERAL NERVE SHEATH TUMOURS to {Melanoma and neural system tumor syndrome}, OMIM:155755; MELANOMA; PANCREATIC CANCER; ASTROCYTOMA; GLIOBLASTOMA; SCHWANNOMA; NEUROFIBROMA; MENINGIOMA; MALIGNANT PERIPHERAL NERVE SHEATH TUMOURS
Familial tumours of the nervous system v2.1 CDKN2A Terri McVeigh gene: CDKN2A was added
gene: CDKN2A was added to Familial tumours of the nervous system. Sources: Expert Review,Literature
Mode of inheritance for gene: CDKN2A was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for gene: CDKN2A were set to PMID 24884915; 8317504; 9622062; 10797439; 17440112; 26794401; 29263814; 28699883; 28754699; 35422439; 38936911
Phenotypes for gene: CDKN2A were set to MELANOMA; PANCREATIC CANCER; ASTROCYTOMA; GLIOBLASTOMA; SCHWANNOMA; NEUROFIBROMA; MENINGIOMA; MALIGNANT PERIPHERAL NERVE SHEATH TUMOURS
Penetrance for gene: CDKN2A were set to Incomplete
Review for gene: CDKN2A was set to GREEN
Added comment: Phenotype including neural tumours discussed at Joint Dutch/UKCGG Cancer Case Meeting 2025
Sources: Expert Review, Literature
Familial tumours of the nervous system v2.1 Achchuthan Shanmugasundram Panel version 2.0 has been signed off on 2023-12-20
Familial tumours of the nervous system v2.0 Achchuthan Shanmugasundram promoted panel to version 2.0
Familial tumours of the nervous system v1.3 LZTFL1 Eleanor Williams Tag curated_removed tag was added to gene: LZTFL1.
Familial tumours of the nervous system v1.3 LZTR1 Eleanor Williams reviewed gene: LZTR1: Rating: GREEN; Mode of pathogenicity: ; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v1.3 LZTFL1 Eleanor Williams commented on gene: LZTFL1
Familial tumours of the nervous system v1.2 LZTR1 Eleanor Williams gene: LZTR1 was added
gene: LZTR1 was added to Familial tumours of the nervous system. Sources: Expert Review Green,NHS GMS
Mode of inheritance for gene: LZTR1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v1.2 LZTFL1 Eleanor Williams Source Expert Review Removed was added to LZTFL1.
Rating Changed from Green List (high evidence) to No List (delete)
Familial tumours of the nervous system v1.1 Eleanor Williams Panel version 1.0 has been signed off on 2023-09-14
Familial tumours of the nervous system v1.0 Eleanor Williams promoted panel to version 1.0
Familial tumours of the nervous system v0.3 Eleanor Williams Panel types changed to GMS Rare Disease; GMS signed-off
Familial tumours of the nervous system v0.2 Achchuthan Shanmugasundram Panel status changed from internal to public
Familial tumours of the nervous system v0.1 DGCR8 Eleanor Williams commented on gene: DGCR8
Familial tumours of the nervous system v0.1 DGCR8 Eleanor Williams Tag gene-checked tag was added to gene: DGCR8.
Familial tumours of the nervous system v0.1 SUFU Achchuthan Shanmugasundram reviewed gene: SUFU: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 SMARCE1 Achchuthan Shanmugasundram reviewed gene: SMARCE1: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 SMARCB1 Achchuthan Shanmugasundram reviewed gene: SMARCB1: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 NF2 Achchuthan Shanmugasundram reviewed gene: NF2: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 LZTFL1 Achchuthan Shanmugasundram reviewed gene: LZTFL1: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 DGCR8 Achchuthan Shanmugasundram reviewed gene: DGCR8: Rating: GREEN; Mode of pathogenicity: None; Publications: ; Phenotypes: ; Mode of inheritance: MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 SUFU Achchuthan Shanmugasundram gene: SUFU was added
gene: SUFU was added to Familial tumours of the nervous system. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: SUFU was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 SMARCE1 Achchuthan Shanmugasundram gene: SMARCE1 was added
gene: SMARCE1 was added to Familial tumours of the nervous system. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: SMARCE1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 SMARCB1 Achchuthan Shanmugasundram gene: SMARCB1 was added
gene: SMARCB1 was added to Familial tumours of the nervous system. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: SMARCB1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 NF2 Achchuthan Shanmugasundram gene: NF2 was added
gene: NF2 was added to Familial tumours of the nervous system. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: NF2 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 LZTFL1 Achchuthan Shanmugasundram gene: LZTFL1 was added
gene: LZTFL1 was added to Familial tumours of the nervous system. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: LZTFL1 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.1 DGCR8 Achchuthan Shanmugasundram gene: DGCR8 was added
gene: DGCR8 was added to Familial tumours of the nervous system. Sources: NHS GMS,Expert Review Green
Mode of inheritance for gene: DGCR8 was set to MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Familial tumours of the nervous system v0.0 Achchuthan Shanmugasundram Added Panel Familial tumours of the nervous system
Set list of related panels to R221
Set panel types to: GMS Rare Disease