2B54.0

ICD-11 MMS 2026-01

Undifferentiated pleomorphic sarcoma of skin

Description

A rare malignant neoplasm arising from the skin. It is characterised by the presence of spindle cells in a storiform pattern and histiocytes with abundant cytoplasm.

Coding note from above levels

For use of this category, reference should be made to the mortality coding rules and guidelines in the Reference Guide.

Index terms

  • Undifferentiated pleomorphic sarcoma of skin
  • malignant fibrous histiocytoma of skin
  • malignant fibrous histiocytoma of unspecified site

Inclusions

  • malignant fibrous histiocytoma of skin

Exclusions from above levels

  • 2A01.1 – Mesenchymal tumour of meninges
  • 2A02.1 – Tumours of cranial or paraspinal nerves
  • http://id.who.int/icd/entity/879154182 – Neoplasms of central nervous system or related structures
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  • http://id.who.int/icd/entity/944754984 – Neoplasms of haematopoietic or lymphoid tissues

Sibling codes

  • 2B54.1 – Undifferentiated pleomorphic sarcoma of retroperitoneum or peritoneum
  • 2B54.Y – Undifferentiated pleomorphic sarcoma, other specified primary site
  • 2B54.Z – Undifferentiated pleomorphic sarcoma, unspecified primary site

Foundation entity

Undifferentiated pleomorphic sarcoma of skin – http://id.who.int/icd/entity/361875781

Allowed postcoordination axes

Part of: 2B54: Undifferentiated pleomorphic sarcoma, primary site › 02: Neoplasms