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

Eur Arch Otorhinolaryngol. 2024 Dec 7. doi: 10.1007/s00405-024-09121-x. Online ahead of print.

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.

Keywords: Biphenotypic sinonasal sarcoma; Epistaxis; Malignancy; Sinonasal tract; Toddler.