Item # Length Source of Standard Year Implemented Version Implemented Year Retired Version Retired
1193 1 NAACCR/SEER 2025 25

NAACCR XML: Tumor.foxo1GeneRearrangements

Alternate Names
   FKHR-PAX3
   FKHR-PAX7
Description

FOXO1 gene rearrangement fusions are found to be positive in about 85% of alveolar rhabdomyosarcoma patients, while they are generally negative for embryonal rhabdomyosarcomas. The presence of these fusions indicates a poor prognosis. Identifying these fusions will also provide new therapeutic opportunities for the treatment of fusion positive rhabdomyosarcomas (FP-RMS).

Rationale

This is part of the National Childhood Cancer Registry (NCCR) project to collect more specific information on pediatric patients, specifically for Rhabdomyosarcoma. Registries part of the NCCR will start collection on specific pediatric data items with 2024+ diagnoses.

Source documents: molecular pathology report (may be addendum to original pathology report)

Codes
0No gene rearrangements (fusions) identified
1FOXO1-PAX3 gene rearrangement (fusion) present
2FOXO1-PAX7 gene rearrangement (fusion) present
3FOXO1-PAX3 and FOXO1-PAX7 gene rearrangements (fusions) present
4FOXO1 gene rearrangement present, fusion partner not known
7Test ordered, results not in chart
8Not applicable: Information not collected for this case (If this information is required by your standard setter, use of code 8 may result in an edit error.)
9Not documented in medical record Cannot be determined by pathologist FOXO1 gene rearrangement not assessed or unknown if assessed

Note 1: Effective years

Note 2: Physician statement

Note 3: Applicable histologies