LD2D.11

ICD-11 MMS 2026-01

Neurofibromatosis type 2

Description

Neurofibromatosis type 2 (NF2) is a tumour-prone disorder characterised by the development of multiple schwannomas and meningiomas.

Index terms

  • Neurofibromatosis type 2
  • Bilateral acoustic neurofibromatosis
  • Familial acoustic neuroma
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  • Familial vestibular schwannoma
  • NF2 - [Neurofibromatosis type 2] (MIM 101000)
  • Neurofibromatosis, central type

Exclusions from above levels

  • 2E83.Z – Enchondromatosis
  • 4A01.31 – Ataxia-telangiectasia
  • 8C21.1 – familial dysautonomia [Riley-Day]
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  • LA90.00 – Rendu-Osler-Weber disease
  • LD23 – Sturge-Weber syndrome
  • LD26.60 – Angio-osteohypertrophic syndrome
  • LD2C – Proteus syndrome
  • LD2F.1Y – Maffucci syndrome
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD2D.10 – Neurofibromatosis type 1
  • LD2D.12 – Neurofibromatosis type 3
  • LD2D.1Y – Other specified neurofibromatoses
  • LD2D.1Z – Neurofibromatosis, unspecified

Foundation entity

Neurofibromatosis type 2 – http://id.who.int/icd/entity/14808714

Part of: LD2D.1: Neurofibromatoses › LD2D: Phakomatoses or hamartoneoplastic syndromes › 20: Developmental anomalies