PRRX1-Rearranged Mesenchymal Tumor: A Case Report and Review of the Literature

PRRX1-rearranged mesenchymal/fibroblastic tumor is a recently proposed, molecularly defined soft tissue tumor, which has not yet been included in the WHO's most recent edition of soft tissue tumors. This entity has previously been reported in a wide age range (20-76 years). However, we report the first pediatric case in an 11-year-old patient presenting with 5.5 cm mass in the head and neck region. Our case is also the second reported case of a PRRX1 (exon 1)::NCOA1 (exon 15) fusion transcript to date.