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Review

Diagnosis, Treatment and Prognosis of Primary Pulmonary NUT Carcinoma: A Literature Review

by 1, 2 and 2,*
1
The First Clinical Medical College, Zhejiang Chinese Medical University, Hangzhou 310053, China
2
Department of Medical Oncology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310001, China
*
Author to whom correspondence should be addressed.
Curr. Oncol. 2022, 29(10), 6807-6815; https://doi.org/10.3390/curroncol29100536
Received: 25 July 2022 / Revised: 13 September 2022 / Accepted: 19 September 2022 / Published: 22 September 2022
NUT carcinoma is a rare, highly lethal cancer characterized with the rearrangement of the nuclear protein in testis (NUT) gene on chromosome 15q14, which primarily occurs in the midline organs. Primary pulmonary NUT carcinoma (NC) lacks characteristic clinical manifestations, which leads to the high rate of misdiagnose and nonstandard treatment. To date, fewer than one hundred cases have been reported worldwide. Here, a comprehensive literature search involving a total of 35 articles with 55 patients was conducted in this paper. We reviewed and analyzed the associated clinical and pathological characteristics, the efficacy of various treatment options and the prognosis. Pulmonary NC mainly occurred in middle young-aged men (median age, 36) with no smoking history (2:1) and would present with symptoms of cough (63.6%), dyspnea (29.5%), chest pain (18.2%) and hemoptysis (18.2%). The initial imaging frequently revealed large and irregular lesions in the lower lobe (46.5%) of the left or right lungs; lymph node metastasis was also prevalent (91.9%). A focal squamous differentiation with abrupt keratinization often occurred in the undifferentiated or poorly differentiated (93.75%) tumor cells, with abundant necrosis and numerous neutrophils infiltrated. The mean overall survival (OS) in patients of this malignant disease was 6.21 months, and the median OS was 4.4 months. According to our results, this disease is sensitive to radiotherapy, and chemoradiotherapy (either concurrent chemoradiotherapy or sequential chemoradiotherapy) was the most efficient therapeutic regimen to prolong the OS of patients with pulmonary NC. View Full-Text
Keywords: NUT carcinoma (NC); primary pulmonary NC; chemoradiotherapy NUT carcinoma (NC); primary pulmonary NC; chemoradiotherapy
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MDPI and ACS Style

Yuan, J.; Xu, Z.; Guo, Y. Diagnosis, Treatment and Prognosis of Primary Pulmonary NUT Carcinoma: A Literature Review. Curr. Oncol. 2022, 29, 6807-6815. https://doi.org/10.3390/curroncol29100536

AMA Style

Yuan J, Xu Z, Guo Y. Diagnosis, Treatment and Prognosis of Primary Pulmonary NUT Carcinoma: A Literature Review. Current Oncology. 2022; 29(10):6807-6815. https://doi.org/10.3390/curroncol29100536

Chicago/Turabian Style

Yuan, Jiaqian, Zhili Xu, and Yong Guo. 2022. "Diagnosis, Treatment and Prognosis of Primary Pulmonary NUT Carcinoma: A Literature Review" Current Oncology 29, no. 10: 6807-6815. https://doi.org/10.3390/curroncol29100536

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