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Distinguishing these tumors from other cranial conditions is difficult, especially when they involve viral associations or specific gene fusions. The recently described DEK::AFF2 non-keratinizing SCC (NKSCC) subtype fits head and neck neoplasm classification and remains supported by fewer than 30 reported cases. Case summary: A 55-year-old woman developed long-standing ear discomfort, then hearing loss and obstruction. Biopsy confirmed moderately differentiated squamous carcinoma, and final diagnosis showed NKSCC linked to a positive DEK::AFF2 fusion. She underwent surgical excision and three cycles of local radiation, but lumbar metastasis emerged 19 months later, followed by neck lymph node swelling; she died nine months afterward despite metastatic removal. Conclusion: DEK::AFF2 fusion-associated NKSCC of the middle ear shows a poor prognosis and an emerging clinical challenge.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/dekaff2-fusion-associated-middle-ear-non-keratinizing-squamous-cell-carcinoma-a-case-report/340343/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/dekaff2-fusion-associated-middle-ear-non-keratinizing-squamous-cell-carcinoma-a-case-report/340343.png","ImageObject",300,407,{"name":92,"@type":93},"Skyler","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-29","2026-09-22",true,{"@type":102,"interactionType":103,"userInteractionCount":44},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"Why is DEK::AFF2 non-keratinizing squamous cell carcinoma hard to distinguish clinically?","Question",{"text":112,"@type":113},"Primary middle ear SCC is rare, and the non-keratinizing basaloid subtype is exceptionally uncommon. Differential diagnosis can be difficult because distinguishing features may overlap with viral factors or other cranial conditions, and DEK::AFF2 NKSCC is supported by few reported cases.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What was the patient’s presentation and how was the diagnosis confirmed?",{"text":117,"@type":113},"A 55-year-old woman had left external auditory canal discomfort for over 10 years, later developing hearing loss and a sensation of obstruction with intermittent ringing but no vertigo. An unusual mass was biopsied and confirmed as moderately differentiated epithelial squamous cancer cells, and the final diagnosis confirmed NKSCC linked to a positive DEK::AFF2 fusion.",{"name":119,"@type":110,"acceptedAnswer":120},"What treatments were provided and what was the disease outcome?",{"text":121,"@type":113},"The patient underwent surgical excision followed by three cycles of local radiation therapy. Metastasis to the lumbar vertebrae occurred 19 months post-treatment, neck lymph node swelling appeared three months after examination, and she died nine months later despite surgical removal of the metastatic lesion.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},340343,1790122654,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":44,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":34,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144},2336464648746,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","| W | J | C | O | World Journal of\u003Cbr>Clinical Oncology |\n| --- | --- | --- | --- | --- |\n\nSubmit a Manuscript: [https://www.f6publishing.com](https://www.f6publishing.com) World J Clin Oncol 2024 December 24; 15(12): 1501-1506  \nDOI: 10.5306/wjco.v15.i12.1501 ISSN 2218-4333 (online)  \nCASE REPORT  \nDEK::AFF2 fusion-associated middle ear non-keratinizing squamous  \ncell carcinoma: A case report  \nYi-Wen Sun, Ying Zhou, Xiao-Yang Liu, Dan-Hua Shen  \nSpecialty type: Oncology  \nProvenance and peer review:  \nUnsolicited article; Externally peer reviewed.  \nPeer-review model: Single blind  \nPeer-review report’s classification Scientific Quality: Grade B  \nNovelty: Grade B  \nCreativity or Innovation: Grade B  \nScientific Significance: Grade B  \nP-Reviewer: Liu K  \nReceived: June 30, 2024  \nRevised: September 19, 2024  \nAccepted: September 30, 2024  \nPublished online: December 24, 2024  \nProcessing time: 114 Days and 0.5 Hours  \nYi-Wen Sun, Ying Zhou, Xiao-Yang Liu, Dan-Hua Shen, Department of Pathology, Peking University People’s Hospital, Beijing 100044, China  \nCorresponding author: Dan-Hua Shen, MD, Chief Doctor, Department of Pathology, Peking University People’s Hospital, No. 11 Xizhimen South Street, Beijing 100044, China.  \n[shendanhuapkuph@163.com](shendanhuapkuph@163.com)  \nAbstract  \nBACKGROUND  \nPrimary squamous cell carcinoma (SCC) of the middle ear is rare, with nonkeratinizing basaloid types being exceptionally uncommon. Distinguishing these cancers, often caused by viral factors (e.g., human papillomavirus or Epstein-Barr virus), or specific genetic alterations (e.g., bromodomain-containing protein 4-nuclear protein in testis fusion gene or Ewing sarcoma breakpoint region 1 gene fused with FLI chromosomal rearrangement), from other cranial conditions, is difficult. The recently identified DEK::AFF2 non-keratinizing SCC (NKSCC) is a novel subtype, fitting the World Health Organization classification of head and neck neoplasms. Less than 30 cases have been reported, highlighting the need for further studies.  \nCASE SUMMARY  \nA 55-year-old female patient first exhibited signs of illness over 10 years ago with persistent discomfort in the left external auditory canal, accompanied by skin irritation and bleeding. One month prior to seeking professional help, she experienced hearing loss and a sensation of obstruction in the affected ear, intermittently accompanied by ringing sounds, but no dizziness. An unusual mass was detected in the left auditory canal, confirmed through biopsy as moderately differentiated epithelial squamous cancer cells. This led to her admission to our hospital, where the final diagnosis confirmed as “NKSCC linked to a positive DEK::AFF2 fusion”. The patient underwent surgical excision, followed by three cycles of local radiation therapy. Yet, metastasis to the lumbar vertebrae occurred 19 months post-treatment, followed by neck lymph node swelling detected three months after a physical examination. The patient died nine months later despite surgical removal of the metastatic lesion.  \nCONCLUSION  \nDEK::AFF2 gene fusion-associated NKSCC of the middle ear carries a grim prognosis and presents an emerging challenge.  \n WJCO  [https://www.wjgnet.com](https://www.wjgnet.com) 1501 December 24, 2024  Volume 15  Issue 12   \nSun YW et al. DEK::AFF2 fusion  \nKey Words: Carcinoma; DEK::AFF2; Fusion; Pathology; Case report  \n©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.  \nCore Tip: DEK::AFF2 non-keratinizing squamous cell carcinoma remains an understudied entity because of its rarity. This rare variant warrants increased attention and further exploration due to its potential severity and metastatic ability.  \nCitation: Sun YW, Zhou Y, Liu XY, Shen DH. DEK::AFF2 fusion-associated middle ear non-keratinizing squamous cell carcinoma: A case report. World J Clin Oncol 2024; 15(12): 1501-1506  \nURL: [https://www.wjgnet.com/2218-4333/full/v15/i12/1501.htm](https://www.wjgnet.","cbCainhOkEYdbIt3","https://ap.wps.com/l/cbCainhOkEYdbIt3","pdf",9379803,"English","# Introduction\n## Case summary\n## Conclusion","[{\"question\":\"Why is DEK::AFF2 non-keratinizing squamous cell carcinoma hard to distinguish clinically?\",\"answer\":\"Primary middle ear SCC is rare, and the non-keratinizing basaloid subtype is exceptionally uncommon. Differential diagnosis can be difficult because distinguishing features may overlap with viral factors or other cranial conditions, and DEK::AFF2 NKSCC is supported by few reported cases.\"},{\"question\":\"What was the patient’s presentation and how was the diagnosis confirmed?\",\"answer\":\"A 55-year-old woman had left external auditory canal discomfort for over 10 years, later developing hearing loss and a sensation of obstruction with intermittent ringing but no vertigo. An unusual mass was biopsied and confirmed as moderately differentiated epithelial squamous cancer cells, and the final diagnosis confirmed NKSCC linked to a positive DEK::AFF2 fusion.\"},{\"question\":\"What treatments were provided and what was the disease outcome?\",\"answer\":\"The patient underwent surgical excision followed by three cycles of local radiation therapy. Metastasis to the lumbar vertebrae occurred 19 months post-treatment, neck lymph node swelling appeared three months after examination, and she died nine months later despite surgical removal of the metastatic lesion.\"}]","DEK::AFF2 fusion-associated middle ear non-keratinizing squamous cell carcinoma - A case report | PDF",1790035924,18]