Superficial CD34-Positive Fibroblastic Tumor on the Chest Wall of an 8-Year-Old Girl: A Case Report and Literature Review.

IF 1.2 4区 医学 Q4 HEMATOLOGY Pediatric Hematology and Oncology Pub Date : 2021-09-01 Epub Date: 2021-04-01 DOI:10.1080/08880018.2020.1859659
Si Ying Li, Hai Lan Zhang, Yu Zuo Bai
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引用次数: 3

Abstract

This study reports a case of superficial CD34-positive fibroblastic tumor (SCPFT) in a child and analyze the major known clinicopathological features of SCPFT and other skin mesenchymal tumors, contributing to an accurate diagnosis of this rare disease. We summarize the clinicopathologic features of an 8-year-old girl who was diagnosed with SCPFT and 46 previously reported SCPFT cases. Post-operative histopathologic examination of the current case showed the tumor lesion was well-circumscribed; tumor cells were spindled-to-polygonal with a fascicular pattern; most nuclei displayed hyperchromasia and low mitotic rate; intranuclear pseudoinclusions could be found; and abundant eosinophilic cytoplasm and partial myxoid stroma were observed. Immunohistochemistry revealed strong and diffuse CD34-positivity, vimentin staining positively but no S-100, SMA, NSE, CD31, desmin, cytokeratin, STAT6, β-catenin, MDM2, or ERG expression. The Ki-67 and CD68 labeling indexes were approximately 1%. There were no rearrangements of PDGFB or PRDM10 tested by FISH. After surgical resection, the patient had no signs of recurrence or metastasis at a 6-month follow-up. The present case is the first that describes SCPFT in children and has significant clinical implications. SCPFT should be differentiated from other skin mesenchymal tumors. The presented compilation of all so far published SCPFT cases will help in diagnosing successfully SCPFT and increasing awareness of this tumor to guide clinical practice.

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8岁女童胸壁表面cd34阳性纤维母细胞瘤1例报告并文献复习。
本研究报告1例儿童浅表cd34阳性纤维母细胞瘤(SCPFT),分析SCPFT和其他皮肤间充质肿瘤已知的主要临床病理特征,有助于准确诊断这种罕见疾病。我们总结了一名8岁女孩被诊断为SCPFT的临床病理特征和46例先前报道的SCPFT病例。本病例术后组织病理学检查显示肿瘤病灶界限清楚;肿瘤细胞呈梭形到多边形,呈束状;多数核呈高染色,有丝分裂率低;核内可见假包涵体;可见丰富的嗜酸性细胞质和部分粘液样基质。免疫组化显示cd34呈强烈弥散性阳性,vimentin染色呈阳性,但S-100、SMA、NSE、CD31、desmin、细胞角蛋白、STAT6、β-catenin、MDM2和ERG未表达。Ki-67和CD68标记指数约为1%。FISH检测未发现PDGFB或PRDM10的重排。手术切除后,患者在6个月的随访中没有复发或转移的迹象。本病例是第一个描述儿童SCPFT的病例,具有重要的临床意义。SCPFT应与其他皮肤间质肿瘤鉴别。目前所有已发表的SCPFT病例的汇编将有助于成功诊断SCPFT,并提高对这种肿瘤的认识,以指导临床实践。
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来源期刊
CiteScore
2.60
自引率
5.90%
发文量
71
审稿时长
6-12 weeks
期刊介绍: PHO: Pediatric Hematology and Oncology covers all aspects of research and patient management within the area of blood disorders and malignant diseases of childhood. Our goal is to make PHO: Pediatric Hematology and Oncology the premier journal for the international community of clinicians and scientists who together aim to define optimal therapeutic strategies for children and young adults with cancer and blood disorders. The journal supports articles that address research in diverse clinical settings, exceptional case studies/series that add novel insights into pathogenesis and/or clinical care, and reviews highlighting discoveries and challenges emerging from consortia and conferences. Clinical studies as well as basic and translational research reports regarding cancer pathogenesis, genetics, molecular diagnostics, pharmacology, stem cells, molecular targeting, cellular and immune therapies and transplantation are of interest. Papers with a focus on supportive care, late effects and on related ethical, legal, psychological, social, cultural, or historical aspects of these fields are also appreciated. Reviews on important developments in the field are welcome. Articles from scientists and clinicians across the international community of Pediatric Hematology and Oncology are considered for publication. The journal is not dependent on or connected with any organization or society. All submissions undergo rigorous peer review prior to publication. Our Editorial Board includes experts in Pediatric Hematology and Oncology representing a wide range of academic and geographic diversity.
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