NUT Carcinoma Without Upfront Surgical Resection: A Case Report

Abstract

Nuclear protein in testis carcinoma is a rare and highly aggressive carcinoma associated with a 70% mortality rate 1 year from diagnosis and a median survival of only 6.5 months. No established treatment protocol exists, although some success has been achieved using a multimodal approach including early surgical resection and adjuvant chemotherapy and radiation. Prior studies have not demonstrated successful treatment in the absence of upfront surgical resection. We describe the first reported case of a patient with unresectable nuclear protein in testis carcinoma treated successfully with definitive chemotherapy using the Scandinavian Sarcoma Group IX Protocol and concurrent radiation therapy, but without surgical resection.

Figure 1A and 1B.

Axial and sagittal CT imaging showing a 5.4 × 3.2 × 4.3 cm large destructive sinonasal tumor encompassing the upper nasal cavity, nasal septum, bilateral sphenoid sinus, and bilateral maxillary sinus, asymmetrically on the right. The nasal septum is deviated and partially destroyed secondary to the large tumor. The mass extends intracranially through the cribiform plate as well as the medial temporal wall on the right. There is also extension through the lamina propecia into the right posterior orbit. Figure 1C. PET/CT demonstrating mass of size 5.0 × 5.0 × 4.3 cm with associated SUV of 8. There were no other sites of FDG avidity and no evidence of distant metastatic disease.

Figure 2A.

The initial phase of treatment included the bilateral cervical lymphatics and a 1cm clinical target volume (CTV) margin around the gross disease. Planning target volume (PTV) 1 (3mm expansion on CTV1) is shown in green (outer line) (CTV1 in yellow (middle line), gross tumor volume (GTV) in red (innermost line present on second panel)). Figure 2B. Boost target volume for the primary site, with gross disease (GTV) delineated in red (innermost line), with a 5 mm CTV in yellow (middle line) and 3mm PTV in green (outer line).