2B31.3

ICD-11 MMS 2026-01

Langerhans cell sarcoma

Description

A neoplastic proliferation of Langerhans cells with overtly malignant cytologic features. It can be considered a higher grade variant of Langerhans cell histiocytosis (LCH) and it can present de novo or progress from antecedent LCH.

Index terms

  • Langerhans cell sarcoma

Exclusions from above levels

  • 2E03 – Malignant neoplasm metastasis in bone or bone marrow
  • http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
  • http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

  • 2B31.0 – Juvenile xanthogranuloma
  • 2B31.1 – Histiocytic sarcoma
  • 2B31.2 – Langerhans cell histiocytosis
  • 2B31.4 – Interdigitating dendritic cell sarcoma
  • 2B31.5 – Follicular dendritic cell sarcoma
  • 2B31.6 – Indeterminate cell histiocytosis
  • 2B31.7 – Fibroblastic reticular cell tumour
  • 2B31.Y – Other specified histiocytic or dendritic cell neoplasms
  • 2B31.Z – Histiocytic or dendritic cell neoplasms, unspecified

Foundation entity

Langerhans cell sarcoma – http://id.who.int/icd/entity/588958190

Allowed postcoordination axes

Part of: 2B31: Histiocytic or dendritic cell neoplasms › 02: Neoplasms