NUTM1 (NUT midline carcinoma family member 1), located on chromosome 15q14, plays a role in the pathogenesis of multiple malignancies, the most recognized being NUT carcinoma. This highly aggressive malignant tumor exhibits squamous differentiation and primarily affects children and young adults. Other neoplasms with NUTM1 rearrangements include pediatric acute lymphoblastic leukemia, embryonal tumors of the central nervous system, poroma/porocarcinoma, and undifferentiated sarcomas.1, 2, 3

NUT-rearranged sarcomas are rare malignant mesenchymal tumors that arise in various anatomic locations; nearly all harbor a fusion involving NUTM1. First described by Dickson et al. in 2018, they have initially been reported in the somatic soft tissue, stomach, brain, and kidney. However, the renal tumors have subsequently been classified as carcinomas.4,5 Since then, additional cases have been identified in other sites. In 2021, Van Treeck et al. noted a predilection for the colorectal region in MXD4::NUTM1 tumors.6 Recently, Papke et al. reported two tumors harboring NUTM2 rearrangements (MXI1::NUTM2A and MXD4::NUTM2G).7CIC::NUTM1 sarcomas are excluded from this discussion due to their closer relationship to other CIC-rearranged round cell sarcomas.8, 9, 10, 11