Complete Response Induced by Concurrent Chemoradiotherapy in a Patient with NUT Carcinoma

Abstract

An 18-year-old man presented with sudden vision loss in his left eye. Magnetic resonance imaging revealed a tumor that had invaded the left optic nerve, originating from the left posterior ethmoid sinus. Immunohistochemical analyses identified positive staining for NUT protein in the nuclei of tumor cells. We diagnosed locally advanced NUT carcinoma (NC) and initiated concurrent chemoradiotherapy (CCRT), consisting of chemotherapy with vincristine, doxorubicin, and cyclophosphamide, alternating with ifosphamide and etoposide, plus radiation therapy. The patient achieved a complete response. CCRT can be a useful treatment option for adolescent and young-adult patients with locally advanced unresectable NC.

Case report: NUT carcinoma with MXI1::NUTM1 fusion characterized by abdominopelvic lesions and ovarian masses in a middle-aged female

“…NUT-mediated genome-wide histone modification is vital in the pathogenesis of NUT carcinoma via activating the histone acetyltransferase (HAT) p300, a factor required for enhancer function achievement and the transcription of oncogenes or tumor suppressor genes like MYC and SOX2 (15). Some cases have reported excellent tumor stabilization effects of Ewing sarcoma chemotherapy, concurrent Chemoradiotherapy (CCRT), and immunovirotherapy (34)(35)(36)(37). Nasal NUT carcinoma has remarkably responded to Ewing sarcoma-based chemotherapy regimen and concurrent radiation in several cases (34,35).…”

“…Nasal NUT carcinoma has remarkably responded to Ewing sarcoma-based chemotherapy regimen and concurrent radiation in several cases ( 34 , 35 ). And CCRT has been found helpful in achieving complete remission in several patients suffering from head, neck, and thoracic NUT carcinoma ( 36 , 38 ). Recently, a case demonstrated the feasibility of an add-on immunovirotherapy regimen in a patent with thoracic NUT carcinoma, which includes an oncolytic virus (talimogene laherparepvec (T-VEC), IMLYGIC ® ) together with the immune checkpoint inhibitor pembrolizumab as an add-on to a basic therapy (cytostatic chemotherapy, radiation therapy, and epigenetic therapy) and shows a significant improvement of tumor stabilization and the patient’s quality of life ( 37 ).…”

Therapeutic approaches to sinonasal NUT carcinoma: a systematic review

“…Concerning the treatment response, most patients ( n = 9) showed complete response to primary therapy but only three of them were AWOD at the end of their follow-up period [ 19 , 20 , 22 ]. On the other hand, the primary treatment of nine patients resulted in a disease progression and three patients showed only partial response to the therapy.…”

Case report: Immunovirotherapy as a novel add-on treatment in a patient with thoracic NUT carcinoma

“…After no initial response to carboplatin/paclitaxel, we initiated an induction chemotherapy based on the standard VIDE protocol for Ewing Sarcoma, as these agents so far have demonstrated the best pre-clinical and case-based evidence in NC so far ( 6 , 8 , 10 , 11 , 36 ). Because of intolerable side effects (severe confusion, hallucinations) ifosfamide was exchanged with cyclophosphamide, which was shown to be non-inferior in this treatment protocol in the therapy of Ewing sarcomas ( 37 ).…”

“…So far, there is no standard treatment regimen for palliation of metastatic disease stages. However, ifosfamide-based regimens and additional radiation therapy have shown some promising results, with published cases of some very rare partial or complete responses in non-thoracic NC (6,(8)(9)(10)(11). The combination of carboplatin and paclitaxel is frequently used in thoracic NC, but with only limited success and progression free survival rates below 3 months (12)(13)(14).…”