PMID- 36591513 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20230103 IS - 2234-943X (Print) IS - 2234-943X (Electronic) IS - 2234-943X (Linking) VI - 12 DP - 2022 TI - EWSR1::SMAD3-rearranged fibroblastic tumor: A case with twice recurrence and literature review. PG - 1017310 LID - 10.3389/fonc.2022.1017310 [doi] LID - 1017310 AB - EWSR1::SMAD3-rearranged fibroblastic tumor is a recently described entity that mostly occurs in acral locations. Only 15 cases have been reported in the English literature, with a wide age range and marked female predominance. The most common sites are the foot, followed by the hand and the distal lower leg. There are four cases that recurred locally during 5-120 months of follow-up, with no metastases to date. Herein, we presented a case of EWSR1::SMAD3-rearranged fibroblastic tumor that recurred twice in a 20-year-old man. The patient presented with a second recurrent painful nodule in the left plantar of the second toe. Grossly, the lesion was pale solid and well-defined, measuring 9 x 8 x 9 mm in size. Histological examination revealed a monomorphic spindle cell tumor composed of cellular fascicles of bland fibroblasts in a collagenous to myxoid stroma with low mitotic activity, which evoked a wide spectrum of differential diagnoses. Immunohistochemically, the tumor cells were diffusely and strongly positive for ERG while negative for S100, alpha-SMA, CD34, and other vascular markers. An unbalanced rearrangement of EWSR1 was demonstrated by fluorescence in situ hybridization (FISH), and a gene fusion between EWSR1 exon 7 and SMAD3 exon 6 was confirmed by RT-PCR and Sanger sequencing. This case recurred twice within 6 years with no sign of further relapse and metastasis at another 9-month follow-up since the last surgery, indicating that this tumor was benign but prone to local recurrence. Nevertheless, more cases and further studies are needed to better interpret the biological behavior of this new entity. CI - Copyright (c) 2022 Yang, Fan, Yin, Liu, Zhao, Wang and Cheng. FAU - Yang, Li AU - Yang L AD - State Key Laboratory of Cancer Biology, Department of Pathology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University (Air Force Medical University), Xi'an, China. FAU - Fan, Linni AU - Fan L AD - State Key Laboratory of Cancer Biology, Department of Pathology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University (Air Force Medical University), Xi'an, China. FAU - Yin, Zhiyong AU - Yin Z AD - Department of Cardiology, Xijing Hospital, Fourth Military Medical University (Air Force Medical University), Xi'an, China. FAU - Liu, Yixiong AU - Liu Y AD - State Key Laboratory of Cancer Biology, Department of Pathology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University (Air Force Medical University), Xi'an, China. FAU - Zhao, Danhui AU - Zhao D AD - State Key Laboratory of Cancer Biology, Department of Pathology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University (Air Force Medical University), Xi'an, China. FAU - Wang, Zhe AU - Wang Z AD - State Key Laboratory of Cancer Biology, Department of Pathology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University (Air Force Medical University), Xi'an, China. FAU - Cheng, Hong AU - Cheng H AD - State Key Laboratory of Cancer Biology, Department of Pathology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University (Air Force Medical University), Xi'an, China. LA - eng PT - Case Reports DEP - 20221215 PL - Switzerland TA - Front Oncol JT - Frontiers in oncology JID - 101568867 PMC - PMC9798226 OTO - NOTNLM OT - ERG OT - EWSR1 OT - SMAD3 OT - case report OT - fibroblastic tumor COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/01/03 06:00 MHDA- 2023/01/03 06:01 PMCR- 2022/01/01 CRDT- 2023/01/02 04:57 PHST- 2022/08/11 00:00 [received] PHST- 2022/11/25 00:00 [accepted] PHST- 2023/01/02 04:57 [entrez] PHST- 2023/01/03 06:00 [pubmed] PHST- 2023/01/03 06:01 [medline] PHST- 2022/01/01 00:00 [pmc-release] AID - 10.3389/fonc.2022.1017310 [doi] PST - epublish SO - Front Oncol. 2022 Dec 15;12:1017310. doi: 10.3389/fonc.2022.1017310. eCollection 2022.