PMID- 33314685 OWN - NLM STAT- MEDLINE DCOM- 20211119 LR - 20231110 IS - 1759-7714 (Electronic) IS - 1759-7706 (Print) IS - 1759-7706 (Linking) VI - 12 IP - 3 DP - 2021 Feb TI - Distinct clinicopathological features of pulmonary primary angiomatoid fibrous histiocytoma: A report of four new cases and review of the literature. PG - 314-323 LID - 10.1111/1759-7714.13727 [doi] AB - BACKGROUND: The aim of this study was to highlight the clinicopathological features of pulmonary primary angiomatoid fibrous histiocytoma (PPAFH) to assist with a differential diagnosis. METHODS: There were 10 previous reports in the literature and four new PPAFH cases reviewed in this study. Immunohistochemistry (IHC), fluorescence in situ hybridization (FISH) and DNA and RNA-based next-generation sequencing (NGS) was performed in the four new cases reported here. RESULTS: In the four new PPAFH cases, the ages of occurrence were in patients age from 33 to 55 years and tumor sizes were from 1.5 to 8 cm. Three of four (75.0%) tumors were located in the endobronchus. The most common morphological changes included delineated fibrous capsule (100%, 4/4), lymphoplasmacytic cuff (100%, 4/4), and dense or richly lymphoplasmatic infiltration (100%, 4/4). IHC analysis revealed that the tumor cells of four cases expressed vimentin and TLE1, ALK and CD163 or CD68 was positive in three cases, epithelial membrane antigen (EMA), desmin was positive in two cases, and SMA focal positive expression was observed in two cases. EWSR1 gene rearrangement was positive in all PPAFH cases (100%, 4/4) by FISH detections and four cases were confirmed as EWSR1-CREB1 fusion variant by DNA and RNA based NGS. No regional lymph nodes and distal metastasis, recurrences and death of disease after surgical excision were recorded in all four cases. CONCLUSIONS: PPAFH is a very unusual pulmonary primary mesenchymal tumor and the clinicopathological features are like other unusual sites counterparts, but with a smaller tumor size, related with large airway, with a tendency to exhibit benign biological behavior, with EWSR1 gene rearrangement and higher frequency of EWSR1-CREB1 gene fusion. KEY POINTS: Significant findings in the study: In comparison with "classic somatic" and nonpulmonary visceral angiomatoid fibrous histiocytoma, pulmonary primary angiomatoid fibrous histiocytoma display distinct clinicopathological features and prognosis. What this study adds The study provided the pathological differential diagnostic criteria and clinico-pathological features for pulmonary primary angiomatoid fibrous histiocytoma. CI - (c) 2020 The Authors. Thoracic Cancer published by China Lung Oncology Group and John Wiley & Sons Australia, Ltd. FAU - Wang, Zheng AU - Wang Z AD - Department of Pathology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China. FAU - Zhang, Liping AU - Zhang L AD - Department of Pathology, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Ren, Li AU - Ren L AD - Department of Pathology, Air Force Medical Center of PLA, Beijing, China. FAU - Liu, Dongge AU - Liu D AUID- ORCID: 0000-0001-8203-0913 AD - Department of Pathology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China. FAU - Du, Jun AU - Du J AD - Department of Pathology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China. FAU - Zhang, Min AU - Zhang M AD - Department of Radiology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China. FAU - Lou, Ge AU - Lou G AD - Department of Pathology, the Second Affiliated Hospital of Harbin Medical University, Harbin, China. FAU - Song, Ying AU - Song Y AD - Berry Oncology Corporation, Fuzhou, China. FAU - Wang, Yin AU - Wang Y AD - Berry Oncology Corporation, Fuzhou, China. FAU - Wu, Chunyan AU - Wu C AD - Department of Pathology, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, China. FAU - Han, Guiping AU - Han G AD - Department of Pathology, the Second Affiliated Hospital of Harbin Medical University, Harbin, China. LA - eng GR - Z.W./Beijing Hospital Clinical Research 121 Project/ GR - 8164114 (Z.W.)/National Nature Science Foundation of China grant/ PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20201212 PL - Singapore TA - Thorac Cancer JT - Thoracic cancer JID - 101531441 SB - IM MH - Adult MH - Female MH - Histiocytoma, Benign Fibrous/*diagnosis/pathology MH - Humans MH - Male MH - Middle Aged PMC - PMC7862796 OTO - NOTNLM OT - Differential diagnosis OT - EWSR1 gene fusion OT - histological change OT - metastasis OT - pulmonary primary angiomatoid fibrous histiocytoma EDAT- 2020/12/15 06:00 MHDA- 2021/11/20 06:00 PMCR- 2021/02/01 CRDT- 2020/12/14 11:17 PHST- 2020/09/14 00:00 [received] PHST- 2020/10/15 00:00 [revised] PHST- 2020/10/16 00:00 [accepted] PHST- 2020/12/15 06:00 [pubmed] PHST- 2021/11/20 06:00 [medline] PHST- 2020/12/14 11:17 [entrez] PHST- 2021/02/01 00:00 [pmc-release] AID - TCA13727 [pii] AID - 10.1111/1759-7714.13727 [doi] PST - ppublish SO - Thorac Cancer. 2021 Feb;12(3):314-323. doi: 10.1111/1759-7714.13727. Epub 2020 Dec 12.