Treatment
The treatment of NUT carcinoma often includes surgery, radiation therapy, and chemotherapy. No single approach may be advocated for all patients. When feasible, complete surgical resection has been associated with improved outcomes. However, since the disease is often locally advanced and/or distantly metastatic at initial presentation, complete resection often cannot be safely performed. A variety of radiation therapy and chemotherapy modalities have been used. These have included combination standard cytotoxic regimens applied in other carcinomas, sarcomas, germ cell tumors, and other solid tumors. Not uncommonly an initial response to therapy is observed. However the disease often recurs rapidly in a treatment-refractory manner. Due to the rarity of the disease, its varied clinical manifestations, and the non-uniform treatments administered, it has been challenging to evaluate the efficacy of various therapeutic approaches. Nevertheless, recent anecdotal reports suggest that ifosfamide-based regimens can lead to long term complete responses in both adults and children. These regimens include the Ewing Sarcoma Group (SSG) IX regimen for children, and a combination of ifosfamide, etoposide, and vorinostat in adults. It has also recently become known that a subset of NUT carcinomas express high levels of PDL1, and these tumors can be responsive to immune checkpoint inhibitors. Indeed, an increasing number of PDL1+ patients are having a checkpoint inhibitor added to their treatment regimen.
One goal of this Registry is to collect clinical data regarding response to various treatments and to share this information with the medical community. Registry staff are available to provide consultation on an individual case basis.