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

Abstract

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.

Primary pulmonary nuclear protein of the testis midline carcinoma: case report and systematic review with pooled analysis

“…In terms of epidemiology, primary pulmonary NMC mainly affected children and young adults, with a median age of 29.5 years (range: 6-74 years), younger than in other reports ( 38 , 40 ). Both genders are equally affected.…”

“…cases of pulmonary NMC, with the objective of enhancing our understanding of the epidemiological, pathological, and immunohistochemical features of this disease. In terms of epidemiology, primary pulmonary NMC mainly affected children and young adults, with a median age of 29.5 years (range: 6-74 years), younger than in other reports (38,40). Both genders are equally affected.…”

“…Given the non-specific histological characteristics of NMC ( 2 , 8 , 28 , 40 , 41 ), most cases expressed p63, p40, CK6/7, EMA, CD99, Vimentin, and AE1/AE3 cytokeratins. In addition to these markers, NMC might also express TTF-1, CD56, Syn, CD30, and CAM5.2, which often leaded to misdiagnosis.…”

Nuclear protein in testis carcinoma of the lung

“…Initially, NUT carcinoma was thought to occur more commonly in children and young adults, but recent studies revealed that the tumor could occur at almost any age [28] , with nearly the same incidence in both men and women [25] . And pulmonary NUT carcinoma mainly occurred in young and middle-aged men with no history of smoking (2:1), and the male-to-female ratio was about 1.89 to 1 [29] . He et al.…”