Wangsiricharoen Sintawat, Meis Jeanne M, Yu Wendong
Department of Pathology and Laboratory Medicine, Oregon Health & Science University, Portland, OR 97239, USA.
Department of Anatomic Pathology, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
Semin Diagn Pathol. 2025 Sep;42(5):150941. doi: 10.1016/j.semdp.2025.150941. Epub 2025 Jun 26.
NUT-rearranged sarcoma is an emerging group of soft tissue sarcomas defined by NUT rearrangement. It is distinct from NUT carcinoma. These sarcomas show a predilection for somatic soft tissue, the thoracic region, the gastrointestinal tract, and body cavities, and affect a wide age range. Histologically, they display one or multiple growth patterns within the tumor, including fibrosarcomatous, round cell, epithelioid/rhabdoid, and hyalinized/nested patterns. Tumor cells invariably express NUT (with the exception of tumors harboring NUTM2) and may have aberrant expression of other markers. Molecularly, NUT-rearranged sarcoma typically involves genes in the MAD transcription family. Those NUT-rearranged sarcomas seem to have distinct clinical and pathologic features. While BET inhibitors are effective in NUT carcinoma, they may not be effective in NUT-rearranged sarcoma.
NUT重排肉瘤是一组新出现的软组织肉瘤，由NUT重排定义。它与NUT癌不同。这些肉瘤好发于躯体软组织、胸部、胃肠道和体腔，发病年龄范围广。组织学上，它们在肿瘤内表现出一种或多种生长模式，包括纤维肉瘤样、圆形细胞、上皮样/横纹肌样和玻璃样变/巢状模式。肿瘤细胞总是表达NUT（携带NUTM2的肿瘤除外），并且可能有其他标志物的异常表达。在分子层面，NUT重排肉瘤通常涉及MAD转录家族中的基因。那些NUT重排肉瘤似乎具有独特的临床和病理特征。虽然BET抑制剂对NUT癌有效，但它们可能对NUT重排肉瘤无效。
