Background <p>NUT carcinoma (NUTc) is a&#xa0;rare, aggressive squamous carcinoma defined by <i>NUTM1</i> rearrangements. Outcomes in head and neck (H&amp;N) disease are poor, and no uniform treatment standard exists; available evidence largely derives from case reports and small series.</p> Methods <p>We performed a&#xa0;structured narrative review of H&amp;N NUTc with an emphasis on radiotherapy (RT) dose concepts, chemotherapy regimens, and outcome signals.</p> Results <p>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.</p> Case vignette <p>A&#xa0;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&#xa0;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&#xa0;3 dermatitis and grade&#xa0;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 &gt; 7&#xa0;years, with persistent xerostomia and dysgeusia.</p> Conclusion <p>In selected non-metastatic H&amp;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&#xa0;comparative table to support individualized treatment planning.</p>

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Head and neck NUT carcinoma—radiotherapy dose, chemotherapy choice, and outcomes: lessons from a 7-year complete remission

  • Lara Schulz,
  • Matthias Zähringer,
  • Klaus-Henning Kahl,
  • Nikolaos Balagiannis,
  • Bertram Jehs,
  • Johannes Doescher,
  • Johannes Zenk,
  • Georg Stüben,
  • Maria Neu

摘要

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.