PMID- 33396988
OWN - NLM
STAT- MEDLINE
DCOM- 20210106
LR  - 20210106
IS  - 0529-5807 (Print)
IS  - 0529-5807 (Linking)
VI  - 50
IP  - 1
DP  - 2021 Jan 8
TI  - [ETV6-rearranged low-grade sinonasal non-intestinal-type adenocarcinoma: a 
      clinicopathological analysis].
PG  - 55-59
LID - 10.3760/cma.j.cn112151-20200417-00322 [doi]
AB  - Objective: To investigate the clinicopathologic features, immunophenotype, 
      molecular genetic changes of ETV6-rearranged low-grade sinonasal 
      non-intestinal-type adenocarcinoma (ETV6-RLGSNAC). Methods: Primary sinonasal 
      epithelial malignant tumors were collected from January 2015 to January 2020 in 
      the Department of Pathology, Eye, Ear, Nose and Throat Hospital affiliated to 
      Fudan University. Through morphological observation, immunohistochemical 
      detection and fluorescence in situ hybridization (FISH), ETV6-RLGSNAC was 
      screened out for clinicopathological feature analysis, and relevant literatures 
      were reviewed. Results: There were 550 cases of primary sinonasal epithelial 
      malignant tumors, among which 82 cases were adenocarcinoma. There were 29 cases 
      of low-grade non-intestinal adenocarcinoma, only 3 cases of ETV6-RLGSNAC were 
      screened out. Of the 3 patients, 2 cases were male and 1 case was female, with a 
      mean age of 54 years (range 37-64 years). The main clinical manifestations were 
      nasal stenosis, nasal obstruction and epistaxis. A neoplasm with smooth surfaces 
      was observed under nasal endoscopy. Imaging showed an expansive mass in the 
      sinonasal area. Gross examination showed gray-yellow cut surface with firm 
      texture and a maximum diameter of 2-3 cm. Microscopically, tumors were 
      non-encapsulated and well-circumscribed with expansive growth pattern. The tumor 
      cells were small and mild, cylindrical and cuboidal, and arranged in regular 
      glandular and trabecular patterns. The cytoplasm was eosinophilic and the nuclei 
      were basally located with inconspicuous nucleoli. By immunohistochemistry (IHC), 
      CK7, SOX-10, DOG1 and vimentin were positive and S-100 expressed in small 
      clusters of cells in all cases. GCDFP-15, CD56, CK20, mammaglobin, TTF-1, NR4A3 
      were all negative. The Ki-67 value-added index of all cases was low (<5%). ETV6 
      gene rearrangement was confirmed in all the cases by FISH, and two cases had 
      NTRK3 gene rearrangement. All three patients underwent radical resection after 
      diagnosis, and one also had adjuvant radiotherapy. All three patients were 
      available with a follow-up time of 12-25 months, and all were recurrence free. 
      Conclusions: ETV6-RLGSNAC is a rare low-grade and newly named non-intestinal 
      adenocarcinoma. The histomorphology is similar to other low-grade nasal sinonasal 
      adenocarcinomas and some salivary gland tumors. IHC and FISH are useful for the 
      diagnosis and differential diagnosis.
FAU - Zhai, C W
AU  - Zhai CW
AD  - Department of Pathology, Eye, Ear, Nose and Throat Hospital, Fudan University, 
      Shanghai 200031, China.
FAU - Yuan, C C
AU  - Yuan CC
AD  - Department of Pathology, Eye, Ear, Nose and Throat Hospital, Fudan University, 
      Shanghai 200031, China.
FAU - Wang, S Y
AU  - Wang SY
AD  - Department of Pathology, Eye, Ear, Nose and Throat Hospital, Fudan University, 
      Shanghai 200031, China.
LA  - chi
PT  - Journal Article
PL  - China
TA  - Zhonghua Bing Li Xue Za Zhi
JT  - Zhonghua bing li xue za zhi = Chinese journal of pathology
JID - 0005331
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Vimentin)
SB  - IM
MH  - *Adenocarcinoma/genetics
MH  - Adult
MH  - *Biomarkers, Tumor/genetics
MH  - Female
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - Vimentin
OTO - NOTNLM
OT  - Adenocarcinoma
OT  - Diagnosis, differential
OT  - Paranasal sinus neoplasms
OT  - Prognosis
EDAT- 2021/01/06 06:00
MHDA- 2021/01/07 06:00
CRDT- 2021/01/05 02:30
PHST- 2021/01/05 02:30 [entrez]
PHST- 2021/01/06 06:00 [pubmed]
PHST- 2021/01/07 06:00 [medline]
AID - 10.3760/cma.j.cn112151-20200417-00322 [doi]
PST - ppublish
SO  - Zhonghua Bing Li Xue Za Zhi. 2021 Jan 8;50(1):55-59. doi: 
      10.3760/cma.j.cn112151-20200417-00322.