Code System Concept

Code System Concept

Code System Concept Code	725049005
Code System Concept Name	Aggressive fibromatosis (disorder)
Code System Preferred Concept Name	Aggressive fibromatosis (disorder)
Concept Status	Published
Concept Status Date	09/01/2024
Code System Name	SNOMED-CT

Concept Details

Code System Code	PH_SNOMED-CT
Code System OID	2.16.840.1.113883.6.96
HL7 Table 0396 Code	SCT
Superseded By Concept
SDO Concept Status	Active
SDO Concept Status Date	07/31/2017
SDO Date Created	07/31/2017
SDO Date Revised	07/31/2017
Description	Desmoid tumour can occur in any part of the body: extra-abdominally (neck, shoulders, upper limbs, gluteal region), abdominally (originating from muscle fascia or the abdominal/chest wall), and more rarely intra-abdominally in the mesentery or retroperitoneum. Depending on the location of the tumour, symptoms may include pain, fever and functional impairment or loss of function of the organ involved. Desmoid tumour results from the proliferation of well-differentiated myofibroblasts. The exact aetiopathogenetic mechanism is still unknown, but they seem to have a multi-factorial origin with hormonal and genetic factors being involved. Somatic mutations in the CTNNB1 gene (3q21) encoding beta-catenin have been found in about 85% of sporadic cases.
Sequence

Extended Properties

No extended properties.

Concept Alternate Designations

SDO Designation ID	Concept Designation Description	Status	Designation Type	PHIN Preferred Term Flag	Code Flag
3437474012	Aggressive fibromatosis	Active	Synonym	false	false
3437475013	Desmoid type fibromatosis	Active	Synonym	false	false
3437476014	Desmoid tumor	Active	Synonym	false	false
3437477017	Desmoid tumour	Active	Synonym	false	false
3773008016	Desmoid fibromatosis	Active	Synonym	false	false
3773009012	Deep fibromatosis	Active	Synonym	false	false

Associated Value Sets

Parent Concepts

Parent Concepts

Parent/Child (Relationship Type)

Other Relationships

No other relationships present.

Child Concepts

Aggressive fibromatosis (disorder) {725049005 , SNOMED-CT }

Parent/Child (Relationship Type)