Infantile Fibrosarcoma

Preferred Label : Infantile Fibrosarcoma;

NCIt synonyms : Congenital Fibrosarcoma; IFS;

NCIt related terms : Infantile fibrosarcoma (congenital fibrosarcoma);

NCIt definition : A fibrosarcoma that occurs in infants. It shares identical morphologic features with adult fibrosarcoma but carries the t(12;15)(p13;q25) translocation that results in ETV6-NTRK3 gene fusion. It usually affects the superficial and deep soft tissues of the extremities. The prognosis is generally much more favorable than for adult fibrosarcoma, and it rarely metastasizes.;

Alternative definition : NICHD: A fibrosarcoma typically congenital, and occurring most commonly within the first three months of life, sharing identical morphologic features with adult fibrosarcoma but rarely metastatic. It usually affects the superficial and deep soft tissues of the extremities. The majority of infantile fibrosarcomas carry the t(12;15)(p13;q25) translocation.;

Neoplastic status : Malignant;

ICD-O code : 8814/3;

Codes from synonyms : 10065859;

Détails

Origin ID

C4244;

UMLS CUI

C0334459;

CISMeF manual mappings
- congenital infantile fibrosarcoma [Disease (Nov.)]
Currated CISMeF NLP mapping
- Congenital fibrosarcoma [MedDRA Preferred Term]
- Infantile fibrosarcoma [ICD-11 Extension code]
- Infantile fibrosarcoma [MedDRA LLT]
- Infantile fibrosarcoma [ICD-O code]
- Infantile fibrosarcoma (disorder) [SNOMED CT concept]
- Infantile fibrosarcoma (morphologic abnormality) [SNOMED CT concept]
- congenital fibrosarcoma [DO Concept]
- infantile fibrosarcoma [SNOMED Notion]
DO Cross reference
- congenital fibrosarcoma [DO Concept]
Disease excludes abnormal cell
- Malignant Epithelial Cell [NCIt concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
Disease may have findings
- Favorable Clinical Outcome [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Congenital fibrosarcoma [MedDRA Preferred Term]
- Infantile fibrosarcoma [ICD-O code]
- Infantile fibrosarcoma [MedDRA LLT]
- Infantile fibrosarcoma (disorder) [SNOMED CT concept]
- Infantile fibrosarcoma (morphologic abnormality) [SNOMED CT concept]
- congenital fibrosarcoma [DO Concept]
- congenital infantile fibrosarcoma [Disease (Nov.)]
- infantile fibrosarcoma [SNOMED Notion]
concept_is_in_subset
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology Terminology [NCIt concept]
- Mapped ICDO3.1 Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCI CTEP SDC Non-Rhabdomyosarcoma Soft Tissue Sarcoma Sub-Category Terminology [NCIt concept]
- NCI CTEP Simplified Disease Classification Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Hematology-Oncology Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Fibroblast [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Cell [NCIt concept]
- Neoplastic Fibroblast [NCIt concept]
disease_has_finding
- Childhood Lesion [NCIt concept]
- Infantile Lesion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
disease_has_normal_cell_origin
- Connective and Soft Tissue Cell [NCIt concept]
- Fibroblast [NCIt concept]
disease_has_primary_anatomic_site
- Connective and Soft Tissue [NCIt concept]
disease_mapped_to_gene
- ETV6/NTRK3 Fusion Gene [NCIt concept]
- NTRK3 Gene [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Trisomy 11 [NCIt concept]
- Trisomy 17 [NCIt concept]
- Trisomy 20 [NCIt concept]
- Trisomy 8 [NCIt concept]
pathogenesis_of_disease_involves_gene
- ETV6/NTRK3 Fusion Gene [NCIt concept]
- NTRK3 Gene [NCIt concept]
- NTRK3 wt Allele [NCIt concept]

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