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.

Keywords: NUT carcinoma; VDC-IE regimen; concurrent chemoradiotherapy.

Figure 1.

Magnetic resonance imaging (MRI) revealed a left posterior ethmoid sinus mass (A, arrows), and positron emission tomography (PET)-computed tomography (CT) revealed a mass that showed an increased 18F-fluorodeoxyglucose (FDG) uptake (B).

Figure 2.

Endoscopic findings of the middle nasal meatus in an operation. The tumor (asterisk) extended from the posterior ethmoid sinus into the skull base and the orbit (dotted line). lp: lamina papyracea, ms: maxillary sinus, mt: middle turbinate, sb: skull base

Figure 3.

Microscopic findings of the resected tumor. Hematoxylin and Eosin staining (A, ×200). NUT staining (B, ×200).

Figure 4.

Results of a fluorescence in situ hybridization (FISH) analysis of NUTM1 showing a NUTM1 split signal (arrows) and a fused signal (arrowhead) in the tumor.

Figure 5.

Clinical course. CPA: cyclophosphamide, CR: complete response, DXR: doxorubicin, ETP: etoposide, IE: ifosphamide, and etoposide, IFO: ifosfamide, m: months, PBR: proton beam radiotherapy, RT: radiotherapy, VAC: vincristine, actinomycin D, and cyclophosphamide, VCR: vincristine, VDC: vincristine, doxorubicin, and cyclophosphamide, X: initiation of therapy

Figure 6.

Magnetic resonance imaging (MRI) (A) and positron emission tomography (PET)-computed tomography (CT) (B) after treatment. MRI and PET-CT did not reveal any abnormalities with signs of relapse.