Biphenotypic sinonasal sarcoma in a toddler: a case report and literature review.

IF 2.2 3区 医学 Q2 OTORHINOLARYNGOLOGY European Archives of Oto-Rhino-Laryngology Pub Date : 2025-04-01 Epub Date: 2024-12-07 DOI:10.1007/s00405-024-09121-x
Tzu-Kung Wang, Ta-Jen Lee, Po-Hung Chang, Chia-Chen Wu, Chia-Hsiang Fu
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Abstract

Introduction: Biphenotypic sinonasal sarcoma (BSNS) is an extremely rare malignant tumor characterized by neural and myogenic differentiation. No documented cases of BSNS have been reported in toddlers.

Case report: A 1-year-11-month-old girl presented with significant unilateral nasal bleeding. Identification of the bleeding source during the initial assessment was particularly challenging given the patient's agitated state. Subsequent exploration under anesthesia revealed an exophytic tumor arising from the middle turbinate. Afterwards, the patient underwent tumor excision via transnasal endoscopy, with no immediate complications. Immunohistochemistry confirmed a diagnosis of BSNS. No signs of recurrence or distant metastasis were observed over a two-year follow-up period.

Conclusion: Massive, persistent nasal bleeding due to sinonasal malignancies in very young children is exceedingly uncommon. For young patients with recurrent or atypical epistaxis, clinicians should consider a neoplastic origin, given the difficulty in pinpointing the bleeding source. This case aims to remind physicians to include such rare malignancies in differential diagnoses, stressing the need for heightened awareness during pediatric assessments.

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儿童双表型鼻窦肉瘤1例报告及文献复习。
双表型鼻窦肉瘤(BSNS)是一种极其罕见的恶性肿瘤,以神经和肌源性分化为特征。在幼儿中没有BSNS的病例报告。病例报告:1岁11个月大的女孩表现为明显的单侧鼻出血。考虑到患者的激动状态,在初步评估中确定出血源尤其具有挑战性。随后在麻醉下探查发现中鼻甲有外生性肿瘤。术后患者经鼻内镜行肿瘤切除术,无直接并发症。免疫组化证实BSNS的诊断。在两年的随访期间没有观察到复发或远处转移的迹象。结论:幼童因鼻腔恶性肿瘤而导致大量、持续性鼻出血极为罕见。对于复发性或非典型鼻出血的年轻患者,临床医生应考虑肿瘤起源,因为很难确定出血来源。本病例旨在提醒医生将这种罕见的恶性肿瘤纳入鉴别诊断,强调在儿科评估中提高认识的必要性。
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来源期刊
CiteScore
5.30
自引率
7.70%
发文量
537
审稿时长
2-4 weeks
期刊介绍: Official Journal of European Union of Medical Specialists – ORL Section and Board Official Journal of Confederation of European Oto-Rhino-Laryngology Head and Neck Surgery "European Archives of Oto-Rhino-Laryngology" publishes original clinical reports and clinically relevant experimental studies, as well as short communications presenting new results of special interest. With peer review by a respected international editorial board and prompt English-language publication, the journal provides rapid dissemination of information by authors from around the world. This particular feature makes it the journal of choice for readers who want to be informed about the continuing state of the art concerning basic sciences and the diagnosis and management of diseases of the head and neck on an international level. European Archives of Oto-Rhino-Laryngology was founded in 1864 as "Archiv für Ohrenheilkunde" by A. von Tröltsch, A. Politzer and H. Schwartze.
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