网状肌样牙源性肿瘤伴新型 STRN::ALK 融合:3岁男性中的2例报告。

IF 3.2 Q2 PATHOLOGY
Molly S Rosebush, Molly Housley Smith, Kitrina G Cordell, Nicholas Callahan, Waleed Zaid, Jeffrey Gagan, Justin A Bishop
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引用次数: 0

摘要

牙源性肿瘤是一个实体的集合体,从畸形瘤到破坏性的良性和恶性肿瘤,不一而足。有时,病理学家会遇到一些牙源性病变,这些病变明显具有牙源性,但又不符合既定的诊断标准。在这里,我们描述了两例这样的牙源性肿瘤,均为 3 岁男性。每个病例都表现为破坏性、放射状的下颌骨病变,由间充质细胞组成,其中一些细胞核具有独特的多叶结构,经常呈网状排列,并由具有灶性层状结构的类肌基质支撑。此外,还可见牙源性硬组织的产生。由于这两个病例具有独特的显微特征,我们对它们进行了新一代测序,发现它们携带相同的STRN::ALK癌基因融合。据我们所知,这两例病例是首次报道带有 STRN::ALK 基因重排的牙源性肿瘤。我们认为这种肿瘤可能与其他已知的牙源性肿瘤不同。两名患者均接受了手术切除和重建治疗。这种肿瘤患者的预后目前还不确定,但随着时间的推移,随着更多病例的发现和跟踪,预后会越来越明显。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Reticular Myxoid Odontogenic Neoplasm with Novel STRN::ALK Fusion: Report of 2 Cases in 3-Year-Old Males.

Odontogenic tumors represent a collection of entities ranging from hamartomas to destructive benign and malignant neoplasms. Occasionally, pathologists encounter gnathic lesions which clearly exhibit an odontogenic origin but do not fit within the confines of established diagnoses. Here, we describe two such odontogenic tumors, both affecting 3-year-old males. Each case presented as a destructive, radiolucent mandibular lesion composed of mesenchymal cells, some with unique multi-lobed nuclei, frequently arranged in a reticular pattern and supported by a myxoid stroma with focal laminations. Production of odontogenic hard tissues was also seen. Because of their unique microscopic features, both cases were investigated by next-generation sequencing and found to harbor the same STRN::ALK oncogene fusion. To our knowledge, these cases represent the first report of an odontogenic tumor with a STRN::ALK gene rearrangement. We propose the possibility that this neoplasm could be separate from other known odontogenic tumors. Both patients were treated with surgical resection and reconstruction. The prognosis of patients with this entity is currently uncertain but shall become more apparent over time as more cases are identified and followed.

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来源期刊
CiteScore
5.70
自引率
9.50%
发文量
99
期刊介绍: Head & Neck Pathology presents scholarly papers, reviews and symposia that cover the spectrum of human surgical pathology within the anatomic zones of the oral cavity, sinonasal tract, larynx, hypopharynx, salivary gland, ear and temporal bone, and neck. The journal publishes rapid developments in new diagnostic criteria, intraoperative consultation, immunohistochemical studies, molecular techniques, genetic analyses, diagnostic aids, experimental pathology, cytology, radiographic imaging, and application of uniform terminology to allow practitioners to continue to maintain and expand their knowledge in the subspecialty of head and neck pathology. Coverage of practical application to daily clinical practice is supported with proceedings and symposia from international societies and academies devoted to this field. Single-blind peer review The journal follows a single-blind review procedure, where the reviewers are aware of the names and affiliations of the authors, but the reviewer reports provided to authors are anonymous. Single-blind peer review is the traditional model of peer review that many reviewers are comfortable with, and it facilitates a dispassionate critique of a manuscript.
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