ArticleThe American journal of surgical pathology2015
Adamantinoma-like Ewing family tumors of the head and neck: a pitfall in the differential diagnosis of basaloid and myoepithelial carcinomas.
Article in The American journal of surgical pathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 63 papers, 2 of them syntheses that pooled it.
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Who cites it
63 citing papers in PubMed, 2 syntheses or guidelines pooled it, 165 citations in OpenAlex.
- Clinical practice guidelines for the treatment of Ewing sarcoma (Spanish Sarcoma Research Group-GEIS).Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico · 2025Guideline
- Glomangiosarcoma-like Anaplastic Transformation in Papillary Thyroid Carcinoma: A Novel Form of Heterologous Differentiation and a Systematic Review of Heterologous Element Prevalence.Endocrine pathology · 2023Pooled it
- p63-Positive Diffuse Large B-cell Lymphoma of Oropharynx: A Treacherous Mimic of HPV-Related Squamous Cell Carcinoma-A Report of Three Cases.Head and neck pathology · 2026Article
- Proceedings of the 2026 North American Society of Head and Neck Pathology Companion Meeting, San Antonio, TX, March 22, 2026: It's a Trap!: Diagnostic Pitfalls in Sinonasal Pathology.Head and neck pathology · 2026Review
- Adamantinoma-like Ewing sarcoma with NRAS Q61R mutation-a hitherto unreported mutation in a case from the nasal cavity.Virchows Archiv : an international journal of pathology · 2025Article
- Rare FUS::FEV-Rearranged Adamantinoma-Like Ewing Sarcoma Arising in the Submandibular Gland.Head and neck pathology · 2025Article
- DEK::AFF2-Associated Papillary Squamous Cell Carcinoma of the Sinonasal Tract: Clinicopathologic Characterization of 9 Cases.Head and neck pathology · 2025Article
- Review
- Small cell osteosarcoma in gnathic bones in the maxilla: case report in a pediatric patient.Journal of cancer research and clinical oncology · 2025Article
- Adamantinoma: A SEER-based Epidemiological Analysis.Indian journal of surgical oncology · 2024Article
- Primary cardiac synovial sarcoma originating from the atrial septum and associated pulmonary infarction: a case report.Journal of cancer research and clinical oncology · 2024Article
- Clinicopathological analysis ofWorld journal of clinical cases · 2024Article
- DNA Methylation Profiling Distinguishes Adamantinoma-Like Ewing Sarcoma From Conventional Ewing Sarcoma.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2023Article
- Adamantinoma-like ewing sarcoma arising in the pancreatic tail: a case report of a rare entity and review of the literature.Diagnostic pathology · 2023Review
- A Low Grade Nasopharyngeal sarcoma With FUS::NACC1 Fusion and Immunohistochemical Evidence of Epithelial Differentiation: Expanding the Clinicopathologic Spectrum of an Emerging Entity.Head and neck pathology · 2023Article
- Top 10 Basaloid Neoplasms of the Sinonasal Tract.Head and neck pathology · 2023Review
- Adamantinoma-like Ewing sarcoma of the salivary glands: a case report and systematic literature review.Therapeutic advances in medical oncology · 2023Article
- Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall.Cureus · 2022Review
- Adamantinoma-Like Ewing Sarcoma of the Head and Neck: A Case-Series of a Rare and Challenging Diagnosis.Head and neck pathology · 2022Article
- Primary Mesenchymal Tumors of the Thyroid Gland: A Modern Retrospective Cohort Including the First Case of TFE3-Translocated Malignant Perivascular Epithelioid Cell Tumor (PEComa).Head and neck pathology · 2022Article
3 more citing papers are in PubMed but not listed here.
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Authors and funding
5 authors at 4 institutions in 2 countries.
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Abstract
Ewing sarcoma family tumors (EFTs) of the head and neck are rare and may be difficult to diagnose, as they display significant histologic overlap with other more common undifferentiated small blue round cell malignancies. Occasionally, EFTs may exhibit overt epithelial differentiation in the form of diffuse cytokeratin immunoexpression or squamous pearls, resembling the so-called adamantinoma-like EFTs and being challenging to distinguish from bona fide carcinomas. Furthermore, the presence of EWSR1 gene rearrangement correlated with strong keratin expression may suggest a myoepithelial carcinoma. Herein, we analyze a series of 7 adamantinoma-like EFTs of the head and neck, most of them being initially misdiagnosed as carcinomas because of their anatomic location and strong cytokeratin immunoexpression, and subsequently reclassified as EFT by molecular techniques. The tumors arose in the sinonasal tract (n=2), parotid gland (n=2), thyroid gland (n=2), and orbit (n=1), in patients ranging in age from 7 to 56 years (mean, 31 y). Microscopically, they departed from the typical EFT morphology by growing as nests with peripheral nuclear palisading and prominent interlobular fibrosis, imparting a distinctly basaloid appearance. Moreover, 2 cases exhibited overt keratinization in the form of squamous pearls, and 1 sinonasal tumor demonstrated areas of intraepithelial growth. All cases were positive for CD99, pancytokeratin, and p40. A subset of cases showed synaptophysin, S100 protein, and/or p16 reactivity, further confounding the diagnosis. Fluorescence in situ hybridization assays showed EWSR1 and FLI1 rearrangements in all cases. Our results reinforce that a subset of head and neck EFTs may show strong cytokeratin expression or focal keratinization, and are therefore histologically indistinguishable from more common true epithelial neoplasms. Thus, CD99 should be included in the immunopanel of a round cell malignancy regardless of strong cytokeratin expression or anatomic location, and a strong and diffuse CD99 positivity should prompt molecular testing for the presence of EWSR1 gene rearrangements.
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