PMID- 31422593
OWN - NLM
STAT- MEDLINE
DCOM- 20190903
LR  - 20190903
IS  - 0529-5807 (Print)
IS  - 0529-5807 (Linking)
VI  - 48
IP  - 8
DP  - 2019 Aug 8
TI  - [Atypical epithelioid hemangioendothelioma: a clinicopathological analysis of 
      eight cases].
PG  - 620-625
LID - 10.3760/cma.j.issn.0529-5807.2019.08.007 [doi]
AB  - Objective: To study the clinicopathological features, diagnosis, and differential 
      diagnosis of atypical epithelioid hemangioendothelioma (EHE). Methods: Eight 
      cases of atypical EHEs were collected from Jiangsu Province Hospital (the First 
      Affiliated Hospital of Nanjing Medical University) between 2010 and 2018. 
      EnVision method and fluorescence in situ hybridization (FISH) were used to detect 
      immunophenotype, WWTR1-CAMTA1 and TFE3 gene rearrangement, respectively. Results: 
      There were 4 males and 4 females, ranging from 42 to 59 years (median 47.5 
      years). The tumors located in soft tissue (3 cases), lung (3 cases), liver (1 
      case) and chest wall (1 case). One soft tissue EHE involved also adjacent fibula 
      and pleural involvement was present in all three lung cases at the diagnosis. 
      Regional lymph node metastases were present in two cases (one involving soft 
      tissue tumor and one involving liver). Morphologically, the tumor cells were 
      epithelioid with abundant eosinophilic cytoplasm, moderate to marked nuclear 
      pleomorphism, irregular nuclear membrane, unevenly chromatin, and prominent 
      nucleoli. The cells arranged in cords, small nests or solid pattern. The mitotic 
      rate was 4.3 mitoses/2 mm(2) on average (ranging 2 to 9). Tumor necrosis was seen 
      in every case. Among all 8 cases, blister cells were found upon careful 
      observation. Myxohyaline stroma was present in 6 cases. Immunohistochemically, 
      tumor cells expressed CD31 (8/8), CD34 (7/8), ERG (8/8), CKpan (2/7), and CAMTA1 
      (4/6). None of the tested cases stained for TFE3 (0/6). WWTR1-CAMTA1 fusion gene 
      by FISH was found in all tested 6 cases and TFE3 gene rearrangement was not 
      detected in any. Available clinical follow-up was obtained in 7 cases and the 
      intervals range from 6 to 55 months (average 19.6 months). Six patients had 
      metastasis and 3 patients died of disease. One patient was alive with no evidence 
      of disease. Conclusions: Atypical EHE is a more aggressive tumor than classic 
      EHE, with histological features including high nuclear grade, increased mitotic 
      activity, the presence of solid growth pattern and tumor necrosis. The 
      differential diagnoses include epithelioid angiosarcoma, carcinoma and 
      epithelioid sarcoma.
FAU - Gong, Q X
AU  - Gong QX
AD  - Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital 
      of Nanjing Medical University), Nanjing 210029, China.
FAU - Fan, Q H
AU  - Fan QH
AD  - Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital 
      of Nanjing Medical University), Nanjing 210029, China.
FAU - Ding, Y
AU  - Ding Y
AD  - Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital 
      of Nanjing Medical University), Nanjing 210029, China.
FAU - Xiao, Q X
AU  - Xiao QX
AD  - Department of Pathology, Gannan Medical University, Jiangxi Province, Ganzhou 
      535000, China.
FAU - Wang, Q Y
AU  - Wang QY
AD  - Department of Pathology, the First People's Hospital of Suqian, Jiangsu Province, 
      Suqian 223800, China.
FAU - Tang, J L
AU  - Tang JL
AD  - Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital 
      of Nanjing Medical University), Nanjing 210029, China.
FAU - Song, Q Y
AU  - Song QY
AD  - Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital 
      of Nanjing Medical University), Nanjing 210029, China.
FAU - Wang, B
AU  - Wang B
AD  - Department of Pathology, Jiangsu Province Hospital (the First Affiliated Hospital 
      of Nanjing Medical University), Nanjing 210029, 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 (CAMTA1 protein, human)
RN  - 0 (Calcium-Binding Proteins)
RN  - 0 (Trans-Activators)
SB  - IM
MH  - Adult
MH  - Biomarkers, Tumor
MH  - Calcium-Binding Proteins
MH  - Female
MH  - *Hemangioendothelioma, Epithelioid
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Middle Aged
MH  - *Neoplasms, Vascular Tissue
MH  - Trans-Activators
OTO - NOTNLM
OT  - CAMTA1
OT  - Hemangioendothelioma, epithelioid
OT  - Hemangiosarcoma
EDAT- 2019/08/20 06:00
MHDA- 2019/09/04 06:00
CRDT- 2019/08/20 06:00
PHST- 2019/08/20 06:00 [entrez]
PHST- 2019/08/20 06:00 [pubmed]
PHST- 2019/09/04 06:00 [medline]
AID - 10.3760/cma.j.issn.0529-5807.2019.08.007 [doi]
PST - ppublish
SO  - Zhonghua Bing Li Xue Za Zhi. 2019 Aug 8;48(8):620-625. doi: 
      10.3760/cma.j.issn.0529-5807.2019.08.007.