Nasal NUT carcinoma with repeated responses during multimodal treatment incorporating radiotherapy: A case report.

چکیده اصلی

RATIONALE: Nuclear protein in testis (NUT) carcinoma, formerly referred to as NUT midline carcinoma, is an exceptionally rare and aggressive malignancy. Evidence guiding radiotherapy-based management is limited, especially for nasal primary tumors with neuroaxis and systemic dissemination. PATIENT CONCERNS: A 40-year-old woman was referred after resection of a nasal cavity malignancy. During the subsequent course, she experienced orbital pain, headache, visual impairment, severe lumbar and radicular pain, bilateral lower-limb paralysis, urinary retention, malignant pleural effusion, and widespread metastatic disease. DIAGNOSES: Pathologic consultation supported nasal NUT carcinoma, with positive NUT immunostaining and a Ki-67 index of approximately 70%. External molecular testing was reported to confirm a NUTM1 rearrangement, although the original report could not be obtained for review, and the assay platform and fusion partner could not be independently verified. INTERVENTIONS: After surgery, the patient received postoperative VMAT/IMRT to 70 Gy in 35 fractions with concurrent cisplatin. Following leptomeningeal, cauda equina, and extensive osseous dissemination, she received palliative helical IMRT/Tomotherapy craniospinal irradiation to 15 Gy in 10 fractions. A later VMAT plan delivered 30 Gy in 10 fractions to one lumbar and 2 hepatic targets. Pembrolizumab, bevacizumab, temozolomide, pleural drainage, and intrapleural therapy overlapped with different treatment phases. OUTCOMES: During the first course, headache severity decreased from 8/10 to 2.5/10 on a visual analog scale, and clinically recorded Snellen visual acuity improved from 20/200 to 20/50. Pain relief was documented after craniospinal irradiation and after the third treatment course. Serial imaging was contemporaneously interpreted as indicating interval reduction in selected lesions after radiation-containing multimodal treatment phases, although uniform retrospective remeasurement was not feasible. According to telephone follow-up with the patient's family, the patient died approximately 12 months after surgery. LESSONS: Repeated clinical and imaging responses were observed following multiple phases of multimodal treatment incorporating radiotherapy. Because systemic therapies were administered during overlapping periods, the independent contribution of radiotherapy could not be isolated. Radiotherapy may provide clinically meaningful local or palliative benefit as part of individualized multimodal treatment in selected patients.

ترجمه فارسی چکیده

مزیت‌های علمی محدودی برای مدیریت مبتنی بر پرتودرمانی در کارسینومای پروتئین هسته‌ای در بیضه (NUT)، که پیش‌تر کارسینومای میانی نوت نامیده می‌شد، وجود دارد، به‌ویژه برای تومورهای اولیه بینی با گسترش به سیستم عصبی مرکزی و سیستمیک. یک زن ۴۰ ساله پس از برداشتن تومور بدخیم حفره بینی به مراجعه شد. پس از عمل جراحی، او به درمان‌های متعددی شامل پرتودرمانی و داروهای سیستمیک دسترسی داشت. علائم بیمار در طول درمان‌ها بهبود یافت، اما بیمار حدود ۱۲ ماه پس از عمل فوت کرد. در این گزارش، تأثیر بالینی پرتودرمانی در کنار سایر درمان‌ها بررسی شده است.

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