Pediatric NUT-midline carcinoma: Therapeutic success employing a sarcoma based multimodal approach

A subset of poorly differentiated squamous cell carcinomas, NUT midline carcinomas (NMC) are characterized by a translocation t(15;19)(q13;p13) [1 French CA, Miyoshi I, Kubonishi I, et al. BRD4-NUT fusion oncogene: A novel mechanism in aggressive carcinoma. Cancer Res. 2003;63(2):304–307.[PubMed], [Web of Science ®] , [Google Scholar]]. The prognosis is generally dismal [2 Bauer DE, Mitchell CM, Strait KM, et al. Clinicopathologic features and long-term outcomes of NUT midline carcinoma. Clin Cancer Res. 2012;18(20):5773–5779.[Crossref], [PubMed], [Web of Science ®] , [Google Scholar]] and therapeutic success has been limited to exceptional cases [3 Mertens F, Wiebe T, Adlercreutz C, et al. Successful treatment of a child with t(15;19)-positive tumor. Pediatr Blood Cancer. 2007;49(7):1015–1017.[Crossref], [PubMed], [Web of Science ®] , [Google Scholar]]. We present two cases of pediatric NMC from two different institutions treated according to a multimodal sarcoma approach involving surgery, chemotherapy, and focal radiotherapy. One patient has remained in complete continuous remission for over 6 years, while the other is in CR in early follow-up off therapy. Our proposed multimodal strategy apparently meets the aggressive biologic nature of NMC and should be considered for further evaluation in this context potentially in the setting of a clinical trial.