Lara Schulz, Matthias Zähringer, Klaus-Henning Kahl, Nikolaos Balagiannis, Bertram Jehs, Johannes Doescher, Johannes Zenk, Georg Stüben, Maria Neu
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引用次数: 0
Abstract
Background: NUT carcinoma (NUTc) is a rare, aggressive squamous carcinoma defined by NUTM1 rearrangements. Outcomes in head and neck (H&N) disease are poor, and no uniform treatment standard exists; available evidence largely derives from case reports and small series.
Methods: We performed a structured narrative review of H&N NUTc with an emphasis on radiotherapy (RT) dose concepts, chemotherapy regimens, and outcome signals.
Results: Published evidence indicates poor overall survival, particularly in metastatic disease. Long-term remissions have been described in selected patients with non-metastatic disease treated with multimodal approaches combining surgery, multi-agent chemotherapy, and early integrated RT. Across reports, use of RT in first-line management and delivery of definitive-dose RT are repeatedly associated with better outcomes. Evidence for immune checkpoint inhibitors is limited to anecdotal single-patient reports.
Case vignette: A 19-year-old man with locally advanced supraglottic NUTc and bilateral cervical nodal disease (M0) presented with dysphagia and otalgia. Following laser debulking and temporary tracheostomy, induction cisplatin/doxorubicin/ifosfamide led to a complete metabolic response in positron-emission tomography/computed tomography (PET/CT) after two cycles of chemotherapy. Definitive PET/CT-guided intensity-modulated (IM)RT/image-guided (IG)RT was delivered to 73.2 Gy using an accelerated twice-daily schedule (2.0 Gy to the planning target volume [PTV] in the morning and 1.6 Gy boost to the initial gross target volume [GTV] in the afternoon; ≥ 8-hour interval). Acute toxicity was grade 3 dermatitis and grade 2 mucositis (CTCAE v5.0). After RT, chemotherapy was continued as consolidation and completed with vincristine/doxorubicin/ifosfamide (six cycles) due to acute kidney injury. Complete remission is ongoing at > 7 years, with persistent xerostomia and dysgeusia.
Conclusion: In selected non-metastatic H&N NUTc, durable control has been reported with timely systemic therapy plus curative-intent RT, often exceeding 50 Gy. This review summarizes dose concepts and case-based systemic regimens and provides a comparative table to support individualized treatment planning.
期刊介绍:
Strahlentherapie und Onkologie, published monthly, is a scientific journal that covers all aspects of oncology with focus on radiooncology, radiation biology and radiation physics. The articles are not only of interest to radiooncologists but to all physicians interested in oncology, to radiation biologists and radiation physicists. The journal publishes original articles, review articles and case studies that are peer-reviewed. It includes scientific short communications as well as a literature review with annotated articles that inform the reader on new developments in the various disciplines concerned and hence allow for a sound overview on the latest results in radiooncology research.
Founded in 1912, Strahlentherapie und Onkologie is the oldest oncological journal in the world. Today, contributions are published in English and German. All articles have English summaries and legends. The journal is the official publication of several scientific radiooncological societies and publishes the relevant communications of these societies.