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000299586 1001_ $$00000-0002-0598-7102$$aPerrino, Melissa R$$b0
000299586 245__ $$aUpdate on Cancer and Central Nervous System Tumor Surveillance in Pediatric NF2-, SMARCB1-, and LZTR1-Related Schwannomatosis.
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000299586 500__ $$a2025 Apr 14;31(8):1400-1406 / Perspective
000299586 520__ $$aSchwannomatoses (SWN) are distinct cancer predisposition syndromes caused by germline pathogenic variants in the genes NF2, SMARCB1, or LZTR1. There is significant clinical overlap between these syndromes with the hallmark of increased risk for cranial, spinal and peripheral schwannomas. Neurofibromatosis type 2 was recently renamed as NF2-related SWN and is the most common SWN syndrome with increased risk for bilateral vestibular schwannomas, intradermal schwannomas, meningiomas and less commonly ependymoma. SMARCB1-related SWN is a familial SWN-syndrome associated with peripheral and spinal schwannomas and an increased risk for meningiomas and malignant peripheral nerve sheath tumors, even in the absence of radiation. These individuals do not develop bilateral vestibular schwannomas. Finally, patients with LZTR1-related SWN typically present with peripheral schwannomas, and unilateral vestibular schwannomas have been reported. The following perspective is intended to highlight the clinical presentation and international tumor surveillance recommendations across these SWN-syndromes.
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000299586 7001_ $$00000-0001-6572-4374$$aJongmans, Marjolijn C J$$b1
000299586 7001_ $$00000-0003-2579-1833$$aTomlinson, Gail E$$b2
000299586 7001_ $$00000-0001-6159-5472$$aGreer, Mary-Louise C$$b3
000299586 7001_ $$00000-0003-2017-0245$$aScollon, Sarah R$$b4
000299586 7001_ $$00000-0003-1656-2799$$aMitchell, Sarah G$$b5
000299586 7001_ $$00000-0001-7733-383X$$aHansford, Jordan R$$b6
000299586 7001_ $$00000-0002-1788-5832$$aSchultz, Kris Ann P$$b7
000299586 7001_ $$00000-0002-9134-9640$$aKohlmann, Wendy K$$b8
000299586 7001_ $$00000-0003-1500-9713$$aKalish, Jennifer M$$b9
000299586 7001_ $$00000-0001-5894-6403$$aMacFarland, Suzanne P$$b10
000299586 7001_ $$00000-0001-7653-9529$$aDas, Anirban$$b11
000299586 7001_ $$00000-0001-8192-4202$$aMaxwell, Kara N$$b12
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000299586 7001_ $$00000-0002-6501-4150$$aWeksberg, Rosanna$$b14
000299586 7001_ $$00000-0002-6097-6457$$aMichaeli, Orli$$b15
000299586 7001_ $$00000-0002-5019-2683$$aTabori, Uri$$b16
000299586 7001_ $$00000-0002-6530-780X$$aNey, Gina M$$b17
000299586 7001_ $$00000-0003-0978-5863$$aLupo, Philip J$$b18
000299586 7001_ $$00000-0002-9475-0077$$aBrzezinski, Jack J$$b19
000299586 7001_ $$00000-0001-8193-1488$$aStewart, Douglas R$$b20
000299586 7001_ $$00000-0002-6297-2855$$aWoodward, Emma R$$b21
000299586 7001_ $$00000-0003-4120-5873$$aKratz, Christian P$$b22
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