PMID- 35094698
OWN - NLM
STAT- MEDLINE
DCOM- 20220407
LR  - 20220407
IS  - 1746-1596 (Electronic)
IS  - 1746-1596 (Linking)
VI  - 17
IP  - 1
DP  - 2022 Jan 30
TI  - Clinicopathological and molecular study of 10 salivary gland clear cell 
      carcinomas, with emphasis on rare cases with high grade transformation and 
      occurring in uncommon sites.
PG  - 18
LID - 10.1186/s13000-022-01200-z [doi]
LID - 18
AB  - BACKGROUND: As a rare salivary gland malignancy, clear cell carcinoma (CCC) is 
      easily misdiagnosed. This study identified the features that allow better 
      recognition of the clinicopathological and molecular characteristics and the 
      prognosis of CCC, focusing on high-grade transformation (HGT) in this tumor and 
      cases arising in uncommon sites. METHODS: Clinicopathological and follow-up data 
      for 10 CCC samples were retrieved. Immunohistochemical (IHC) staining was 
      performed, and fluorescence in situ hybridization (FISH) was used to detect EWSR1 
      gene rearrangements, EWSR1-ATF1 gene fusions, and MAML2 gene rearrangements. 
      RESULTS: Histologically, typical CCCs comprised bland polygonal or round cells 
      with clear cytoplasm. In contrast with typical CCCs, HGT tumor cells exhibited 
      nuclear pleomorphism, high nuclear-to-cytoplasmic ratios, high mitotic activity, 
      and necrosis. Rare morphologic features such as pseudopapillae, gland-like 
      spaces, and entrapped ducts were also observed. Occasionally, tumors involving 
      the oral cavity might arise from the overlying epithelium of the mucosal surface. 
      Immunohistochemically, all the cases expressed p63, p40, and CK5/6, while 
      myoepithelial-related markers were uniformly negative in all cases. HGT exhibited 
      a wild type p53 expression pattern. FISH demonstrated EWSR1 rearrangement (10/10) 
      and EWSR1-ATF1 fusion (4/5); however, MAML2 remained intact (0/3). CONCLUSIONS: 
      CCCs with HGT or occurring in uncommon sites are extremely rare. Combining 
      morphology based IHC and molecular detection provided reliable evidence that the 
      HGT component represented a transformation of CCC rather than the coexistence of 
      another tumor and helped differentiating CCCs in uncommon sites from their 
      mimics, avoiding potential misdiagnosis and inappropriate therapy. The overall 
      prognosis for CCCs is good, except for the HGT cases, which needed continued 
      treatment.
CI  - (c) 2022. The Author(s).
FAU - Xuan, Lanlan
AU  - Xuan L
AD  - Department of Pathology, Anqing Hospital, Anhui Medical University, Anqing 
      Municipal Hospital, Anqing, 246003, China.
AD  - Department of Pathology, Beijing Tongren Hospital, Capital Medical University, 
      Beijing Key Laboratory of Head and Neck Molecular Diagnostic Pathology, Beijing, 
      100730, China.
FAU - Wang, Suxia
AU  - Wang S
AD  - Department of Pathology, Yantai Yuhuangding Hospital of Qingdao University, 
      Yantai, 264000, Shandong, China.
FAU - Wei, Jianguo
AU  - Wei J
AD  - Department of Pathology, Shaoxing People's Hospital, Shaoxing, 312000, China.
FAU - Yuan, Jianwei
AU  - Yuan J
AD  - Department of Oncology Surgery, Anqing Hospital, Anhui Medical University, Anqing 
      Municipal Hospital, Anqing, 246003, China.
FAU - Liu, Honggang
AU  - Liu H
AUID- ORCID: 0000-0002-2496-7676
AD  - Department of Pathology, Beijing Tongren Hospital, Capital Medical University, 
      Beijing Key Laboratory of Head and Neck Molecular Diagnostic Pathology, Beijing, 
      100730, China. liuhg1125bjtr@126.com.
LA  - eng
PT  - Journal Article
DEP - 20220130
PL  - England
TA  - Diagn Pathol
JT  - Diagnostic pathology
JID - 101251558
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (RNA-Binding Protein EWS)
SB  - IM
MH  - *Adenocarcinoma, Clear Cell/genetics
MH  - Biomarkers, Tumor/genetics
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - RNA-Binding Protein EWS/genetics/metabolism
MH  - *Salivary Gland Neoplasms/diagnosis/genetics/metabolism
MH  - Salivary Glands/pathology
PMC - PMC8802448
OTO - NOTNLM
OT  - ATF1
OT  - Clear cell carcinoma
OT  - EWSR1
OT  - High-grade transformation
OT  - Hyalinizing
OT  - MAML2
COIS- The authors declare that they have no competing interests.
EDAT- 2022/02/01 06:00
MHDA- 2022/04/08 06:00
PMCR- 2022/01/30
CRDT- 2022/01/31 05:33
PHST- 2021/10/18 00:00 [received]
PHST- 2022/01/15 00:00 [accepted]
PHST- 2022/01/31 05:33 [entrez]
PHST- 2022/02/01 06:00 [pubmed]
PHST- 2022/04/08 06:00 [medline]
PHST- 2022/01/30 00:00 [pmc-release]
AID - 10.1186/s13000-022-01200-z [pii]
AID - 1200 [pii]
AID - 10.1186/s13000-022-01200-z [doi]
PST - epublish
SO  - Diagn Pathol. 2022 Jan 30;17(1):18. doi: 10.1186/s13000-022-01200-z.