Clinical Features, Therapy and Long-Term Outcomes of NUT Carcioma in China

Abstract

Abstract Background NUT carcinoma is a rare, poorly differentiated tumor typically driven by a t(15;19) rearrangement leading to a NUT gene rearrangement event. This uniformly fatal tumor has promoted targeted therapy, yet the clinical characteristics of Chinese patients with NUT carcinoma and the efficacy of all treatment have not been systematically summarized. In order to better understanding the disease characteristic and treatments, correlate them with outcome, we have here compiled findings pertaining to a large population of such patients. Methods A clinical database from all know cases of NUT carcinoma was established. Pathologic, demographic, and survival data of 33 patients were analyzed by questionnaires, the largest cohort studied of NMC patients to data in China. Results Primary tumors sites included nasal cavity (n = 6), maxillary sinus (n = 5),tonsil (n = 1), thyroid (n = 1), lung (n = 16), mediastinum (n = 1), submandibular(n = 1), vulva(n = 1), external auditory canal༈n = 1༉. The patient age ranged from 2 to 69 years with the male/female ratio of 1.3/1,11 of them died, the media overall survival for those patients was 5.5 months. Multivariate analysis proved that the primary site NUT carcinoma was not related to the prognosis, alternative application of different chemotherapy regimen or combination of immunotherapy on the basis of chemotherapy can effectively control the tumor, and combination of radiotherapy on the basis of chemotherapy can benefit the survival of patients, which is the icing on the cake. Radical surgery can help keep the tumor in a stable state and even delay the recurrence of the tumor. Radical surgery combined with chemotherapy, radiotherapy and immunotherapy can benefit the survival of patients. Notably, NUT progresses quickly when therapy is terminated. Unfortunately, radiotherapy alone does not significantly improve outcome in China. Conclusion Breaking the traditional understanding that NUT carcinoma tends to occur in the midline, and not all originate from squamous cell carcinoma. The finding that conventional chemotherapy and radiotherapy have been not sensitively. Chemotherapy combined with immunotherapy or early surgical might improve overall survival.