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Tumor Discovery Expert consensus of NUT carcinoma

NUT carcinoma patients who have undergone non-R0 led to further shrinkage of the lesions, but this effect
resections, exhibit lymph node positivity, and receive post- lasted only 2 months, resulting in an OS of 17 months.
65
operative adjuvant treatments. Many NUT carcinoma In another case, a 22-year-old patient with supraglottic
patients are not candidates for surgery at the time of NUT carcinoma (stage cT3N2bM0) achieved a complete
diagnosis; for such patients, concurrent chemoradiotherapy response after concurrent chemoradiotherapy (total
becomes a viable treatment option for inoperable tumors. radiation dose: 70 Gy, 2 Gy × 35F; cisplatin 80 mg/m every
2
Standardization of radiation doses for NUT carcinoma 3 weeks), although ovarian metastasis occurred 3 weeks
66
remains elusive. Radiation plans should incorporate at after completing radiotherapy. In the case of pulmonary
least three-dimensional conformal techniques, with a NUT carcinoma, combined radiotherapy and anlotinib
preference for intensity-modulated radiotherapy. The resulted in a 78% reduction in tumor size within
radiation dose and target area should be tailored to the 2 months. A patient with locally advanced head-and-
67
patient’s overall health, tumor site involvement, and tumor neck NUT carcinoma achieved complete remission after
staging. These parameters should be informed by radiation concurrent chemoradiotherapy and alternating regimens
doses and target areas used for advanced malignancies in of vincristine, doxorubicin, and cyclophosphamide, with
similar tumor types. IFO and etoposide. Adolescent patients tend to have a
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poor prognosis, with a median survival of 8 months (range:
Radiation therapy generally employs curative doses,
although palliative doses may be used if the tumor is located 4.5 – 28.8 months) for five NUT carcinoma patients aged 7
58
near critical organs or if the patient has limited tolerance – 16 years who received radiotherapy and chemotherapy.
for treatment. Radiation doses typically range from 50 Gy Before radiotherapy, it is necessary to assess the
to 70 Gy, with studies showing that NUT carcinoma patient’s general condition, diet, speech, and any organs
patients who received doses exceeding 50 Gy have a higher affected by irradiation. For NUT carcinoma patients with
survival rate. 56,64 Before radiation therapy, a comprehensive high-risk factors such as positive lymph nodes or non-R0
evaluation of the patient’s overall health, dietary habits, resection and those who are unsuitable for surgery, an
speech, and any organs that may be irradiated within the MDT evaluation should be conducted before treatment to
target area is required. It is recommended that the primary determine the most appropriate comprehensive treatment,
tumor area is irradiated with 65 – 70 Gy and the affected which should primarily involve concurrent or sequential
regional lymph nodes with 50 – 54 Gy” to: “In cases where chemoradiotherapy. The radiation target area and dose
radiotherapy is used exclusively, it is recommended to can be based on those used for other malignancies at
deliver a dose of 65 – 70 Gy to the primary tumor and corresponding sites.
lymph node lesions, and 50 – 54 Gy to elective lymph node (a) Recommendation 5
regions. The radiation dose should be adjusted based on Patients with high-risk factors, such as lymph node
1
the lesions and the extent of local disease involvement, positivity or non-R0 resection, especially those with
including tumor margins and neurovascular invasion. The inoperable NUT carcinoma, particularly in the head-and-
target area should encompass as many microlesions as neck region, should undergo evaluation by specialized
possible, with recommendations to irradiate the expanded physicians or an MDT before treatment, aiming to select
primary tumor site and any involved lymph node regions. comprehensive therapy, primarily based on concurrent
Chemotherapy drugs used during concurrent chemoradiotherapy. The radiotherapy protocol and dosage
chemoradiotherapy for NUT carcinoma patients are can be guided by delineating target areas and radiation
typically based on those used for other malignancies at doses for other corresponding malignant tumors (level
the same anatomical sites, with platinum-based agents of evidence: grade 3; recommendation level: strongly
being the most common. In one case of head and neck recommended).
NUT carcinoma with primary lesions in the ethmoid and
sphenoid sinuses, the lesions involved both optic nerves and 4.8.3. Chemotherapy
the skull base 1.5 months postoperatively. After 11 sessions The response rate of NUT carcinoma to chemotherapy
of concurrent chemoradiotherapy and hyperthermia is approximately 40%, but it tends to develop resistance
(total radiation dose: 69.96 Gy, 2.12 Gy × 33F; cisplatin, regardless of the type of chemotherapy regimen used. 20,64
temozolomide 75 mg/m ), the lesions shrank significantly. Anthracyclines, cisplatin, alkylating agents, etoposide,
2
However, due to side effects from radiotherapy, the patient gemcitabine, irinotecan, and taxanes have been reported as
was unable to complete the treatment, which led to an part of chemotherapy combinations for NUT carcinoma.
increase in the dose of temozolomide (200 mg/m ), but the However, almost all chemotherapy regimens demonstrate
2
tumor continued to progress. Eventually, adding lapatinib only temporary efficacy or no therapeutic response. 1,20,56,64,69

Volume 3 Issue 4 (2024) 19 doi: 10.36922/td.4904