Background:

Spindle/round cell sarcoma of the sinonasal tract is a high-grade mesenchymal tumor. It is found in the soft tissues of the lower extremities, rarely in the head and neck. In rare cases it can be found in the nasal cavity.

Case summary:

15-year-old male patient presented with recurrent epistaxis for 3 months. A 30-degree rigid endoscopy revealed a mass in the left nasal cavity. A contrast-enhanced CT scan of the paranasal sinuses showed a heterogeneous and hypodense lesion involving the left nasal cavity with extension into the maxillary, frontal, and ethmoid sinuses, abutting the orbit and base of the skull.

An initial biopsy of the mass revealed only inflammation and no malignancy.

A follow-up MRI showed significant progression. Due to the rapid growth, a tumor resection with R2 margins was performed to obtain multiple biopsies of the nasal mass, which showed high grade spindle cell sarcoma.

Further workup and management was done by PET-MRI, interdisciplinary tumor board case presentation and decision of chemotherapy according to iEURO-Ewing protocol. The patient has regular follow up with involved specialties and is doing well so far.

Conclusion:

Spindle/round cell sarcoma of the sinonasal tract is a rare entity with high mortality. Therefore, clinical, radiographic, and histopathologic manifestations should be given critical attention for more accurate diagnosis and further appropriate management. Further studies and evidence-based treatment protocols are needed. This article outlines the role of relevant imaging and a multidisciplinary team approach.

Nein