Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children. One of the primary histological subtypes is the alveolar RMS (ARMS)1.
The majority (55%) of ARMS are associated with the t(2;13)(q36.1;q14) translocation, and less commonly (22%) with t(1;13)(p36;q14), leading to the fusion of transcription factor FOXO1 (Forkhead Box protein 1) to the transcription factors PAX3 (Paired Box protein 3) and PAX7, respectively2. The distinction between the t(1;13) and t(2;13) in ARMS is important as patients with the t(2;13) have a more adverse outcome than those with the t(1;13)3.
In ARMS cases that are negative for translocations involving FOXO1, there are two other variant PAX3 translocations that could be present - t(X;2)(q13;q36.1) PAX3-FOXO44 and t(2;2)(q36.1;p23) PAX3-NCOA15.
1. Kodet R et al., Am J Surg Pathol 1993;17(5):443-53
2. Ahn et al., Oncol Rep. 2013 August; 30(2): 968–978
3. Sorensen et al., J Clin Oncol 2002;20:2672-9
4. Barr et al., Cancer Res 2002;62:4704-10
5. Wachtel et al., Cancer Res 2004:64(16):5539-45
- Area of Interest*
This product is intended to be used on formalin-fixed paraffin-embedded (FFPE) tissues.
*Disease information supported by the literature and is not a reflection of the intended purpose of this product.