Epithelioid Inflammatory Myofibroblastic Sarcoma: Case Series With a First Report of CLTC::ALK Fusion in an Aggressive Disease

IF 3.1 2区 医学 Q2 GENETICS & HEREDITY
Daisy Maharjan, Carina Dehner, Ali Alani, Robert Bell, Sheila Segura
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引用次数: 0

Abstract

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare and clinically aggressive variant of inflammatory myofibroblastic tumor (IMT). It typically presents in children and young adults, often affecting the abdominal cavity. It is characterized by the presence of plump, polyhedral, and epithelioid cells, and a distinctive nuclear or perinuclear ALK staining on immunohistochemistry. Various ALK fusion partners have been identified in EIMS, including RANBP2, RRBP1, EML4, and VCL. In this report, we present four cases of EIMS involving the abdominal cavity, including the first case with a CLTC::ALK fusion, which has previously been associated only with nonaggressive IMT.

上皮样炎性肌成纤维细胞肉瘤:一例侵袭性疾病CLTC: ALK融合的病例系列
上皮样炎性肌纤维母细胞肉瘤(EIMS)是一种罕见的临床侵袭性炎性肌纤维母细胞瘤(IMT)。它通常出现在儿童和年轻人,经常影响腹腔。其特征是存在饱满、多面体和上皮样细胞,免疫组织化学上有独特的核或核周ALK染色。在EIMS中发现了多种ALK融合伙伴,包括RANBP2、RRBP1、EML4和VCL。在本报告中,我们报告了4例累及腹腔的EIMS,包括第一例CLTC: ALK融合,该病例以前仅与非侵袭性IMT相关。
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来源期刊
Genes, Chromosomes & Cancer
Genes, Chromosomes & Cancer 医学-遗传学
CiteScore
7.00
自引率
8.10%
发文量
94
审稿时长
4-8 weeks
期刊介绍: Genes, Chromosomes & Cancer will offer rapid publication of original full-length research articles, perspectives, reviews and letters to the editors on genetic analysis as related to the study of neoplasia. The main scope of the journal is to communicate new insights into the etiology and/or pathogenesis of neoplasia, as well as molecular and cellular findings of relevance for the management of cancer patients. While preference will be given to research utilizing analytical and functional approaches, descriptive studies and case reports will also be welcomed when they offer insights regarding basic biological mechanisms or the clinical management of neoplastic disorders.
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