PMID- 36752907
OWN - NLM
STAT- MEDLINE
DCOM- 20230731
LR  - 20230731
IS  - 1432-1335 (Electronic)
IS  - 0171-5216 (Linking)
VI  - 149
IP  - 9
DP  - 2023 Aug
TI  - Clinical and molecular features of pulmonary NUT carcinoma characterizes diverse 
      responses to immunotherapy, with a pathologic complete response case.
PG  - 6361-6370
LID - 10.1007/s00432-023-04621-5 [doi]
AB  - PURPOSE: Nuclear protein in testis (NUT) carcinoma is an uncommon malignant 
      cancer characterized by NUTM1 rearrangement. We aimed to investigate the 
      clinicopathological and molecular features and immunotherapy of pulmonary NUT 
      carcinoma. METHODS: Immunohistochemistry (IHC) for NUT (C52B1) and programmed 
      cell death ligand 1 (PD-L1: 22C3) and fluorescence in situ hybridization (FISH) 
      for NUTM1 break and BRD4-NUTM1 fusion were performed on six pulmonary NUT 
      carcinoma samples. RESULTS: The 6 pulmonary NUT carcinoma samples were obtained 
      from 5 males and 1 female, with ages ranging from 31 to 73 years (average, 
      46 years). Five tumors occurred in the lobes, with one in the trachea. 
      Pathologically, all cases showed primitive-appearing round to epithelioid cells 
      growing in nests and sheets. Squamous differentiation and abrupt keratinization 
      were observed. All tumors expressed the NUT protein and p63, and 4 tumors showed 
      focal synaptophysin, but PD-L1 expression was not observed. All cases displayed 
      NUTM1 rearrangement, 5 had BRD4-NUTM1 fusion, and one had an unknown partner. 
      Three patients presented regional lymph node involvement at diagnosis. Five 
      patients underwent intensive radiation and/or chemotherapy. Furthermore, 2 
      patients (1 and 2) received a combination of PD-L1 inhibitor and chemotherapy. 
      Patient 1 exhibited a poor response and soon showed tumor progression and 
      metastasis; however, patient 2 responded remarkably and achieved pathologic 
      complete response (pCR) without uncontrollable adverse events. The overall 
      survival time was 2.9 months. CONCLUSIONS: Pulmonary NUT carcinoma exhibits 
      poorly differentiated morphological features with diffuse NUT staining, low PD-L1 
      expression, and NUTM1 rearrangement. Despite its poor prognosis, it presents a 
      diverse response to immunotherapy. Immune checkpoint inhibitors (ICIs) need to be 
      further explored in NUT carcinoma.
CI  - (c) 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, 
      part of Springer Nature.
FAU - Chen, Min
AU  - Chen M
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      610041, China.
FAU - Chen, Xiaohua
AU  - Chen X
AD  - Department of Pathology, The Second People's Hospital of Yibin, Yibin, 644000, 
      China.
FAU - Zhang, Ying
AU  - Zhang Y
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      610041, China.
FAU - Wang, Weiya
AU  - Wang W
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      610041, China.
FAU - Jiang, Lili
AU  - Jiang L
AD  - Department of Pathology, West China Hospital, Sichuan University, Chengdu, 
      610041, China. 879876047@qq.com.
LA  - eng
PT  - Journal Article
DEP - 20230208
PL  - Germany
TA  - J Cancer Res Clin Oncol
JT  - Journal of cancer research and clinical oncology
JID - 7902060
RN  - 0 (B7-H1 Antigen)
RN  - 0 (BRD4 protein, human)
RN  - 0 (Cell Cycle Proteins)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (Transcription Factors)
RN  - 0 (NUTM1 protein, human)
SB  - IM
MH  - Female
MH  - Humans
MH  - Male
MH  - B7-H1 Antigen/genetics
MH  - Cell Cycle Proteins
MH  - Immunotherapy
MH  - In Situ Hybridization, Fluorescence
MH  - *Lung Neoplasms/genetics/therapy
MH  - *Neoplasm Proteins/genetics
MH  - Nuclear Proteins/genetics/metabolism
MH  - Oncogene Proteins, Fusion/genetics
MH  - Transcription Factors/genetics
OTO - NOTNLM
OT  - Immunotherapy
OT  - NUTM1 rearrangement
OT  - Nuclear protein in testis (NUT) carcinoma
OT  - Pathologic complete response
EDAT- 2023/02/09 06:00
MHDA- 2023/07/24 06:42
CRDT- 2023/02/08 11:17
PHST- 2022/11/03 00:00 [received]
PHST- 2023/01/27 00:00 [accepted]
PHST- 2023/07/24 06:42 [medline]
PHST- 2023/02/09 06:00 [pubmed]
PHST- 2023/02/08 11:17 [entrez]
AID - 10.1007/s00432-023-04621-5 [pii]
AID - 10.1007/s00432-023-04621-5 [doi]
PST - ppublish
SO  - J Cancer Res Clin Oncol. 2023 Aug;149(9):6361-6370. doi: 
      10.1007/s00432-023-04621-5. Epub 2023 Feb 8.