Akademik Veri Yönetim Sistemi
FRONTIERS IN ONCOLOGY, cilt.16, ss.1-7, 2026 (SCI-Expanded, Scopus)
Abstract
Primary pulmonary NUT carcinoma (PPNC)
is an exceptionally rare and aggressive malignancy driven by NUTM1
rearrangements, with a median overall survival (OS) of 5–9 months. While
BRD4–NUTM1 accounts for approximately 70% of cases, the rarer NSD3–NUTM1 fusion,
reported in approximately 6% of NUT carcinomas, is poorly characterized, and
data on optimal radiotherapy (RT) techniques and adaptive strategies remain
extremely limited. We describe a 27-year-old man with PPNC harboring an
NSD3–NUTM1 fusion (cT4N2M1c), confirmed by next-generation sequencing (NGS)
following equivocal NUT immunohistochemistry. Given rapidly progressive
post-obstructive atelectasis and airway compromise, a staged, adaptive thoracic
RT strategy was employed: urgent hypofractionated IMRT was initiated with 5 × 3
Gy, followed by 15 × 2 Gy using the initial plan, as early cone-beam CT showed
insufficient anatomical change for immediate replanning. Subsequent tumor
regression and right lung re-expansion enabled repeat CT simulation and adaptive
volumetric modulated arc therapy (VMAT) for the final 9 × 2 Gy, achieving a
cumulative tumor-effect EQD2α/β10Gy of 64.25 Gy. Interim PET/CT revealed
in-field thoracic regression but widespread systemic progression;
cisplatin–etoposide was added during the remaining RT course, followed by multisite
palliative RT and consolidation cisplatin–etoposide–ifosfamide chemotherapy.
Despite an initial metabolic response, hepatic progression precluded planned
immunotherapy. OS was 8.8 months. This case suggests that staged,
dose-escalated adaptive thoracic RT is feasible in selected patients with
disseminated PPNC and compromised pulmonary function, providing durable
in-field control and meaningful symptom palliation as part of a multimodal
treatment approach. Molecular confirmation by NGS is essential for non-BRD4
fusions, and systemic therapy should be incorporated as early as safely
feasible to address the high risk of distant dissemination.