Soft Tissue Sarcoma of the Nasal Cavity and Paranasal Sinuses: A Case Series Identifying Common Features in Diverse Pathology

Introduction: Sinonasal tract sarcomas rarely arise in the nasal cavity and paranasal sinuses. Malignant transformation of various soft tissue components have led to categorization of sarcomas into several different subtypes. Despite this variability, the majority of soft tissue sarcomas are treated in a similar fashion typically consisting of chemotherapy and surgical excision. Overall and disease-free survival in patients with sarcomas of the nasal cavity is poor. We describe a series of patients with soft tissue sarcoma of the nasal cavity and paranasal sinuses presenting to a single institution with the objective of identifying differences and similarities in clinical characteristics and survival among different types of sarcomas.

Methods: A retrospective review of all patients presenting to a single institution from 2007 to 2014 with a diagnosis code of sinonasal cavity malignancy were reviewed. Chart review performed to evaluate treatment, length of follow-up, patient characteristics, and survival. Overall survival and disease-free survival were calculated.

Results: Of 77 patients with sinonasal malignancy, only 6 (7.8%) had diagnosis of sarcoma on final pathology. Osteosarcoma was the most common type (three of six patients) with additional subtypes of fibrosarcoma, leiomyosarcoma, and intermediate grade pleomorphic sarcoma. Overall, two patients had been treated previously with radiation to the head and neck. Average time to disease onset from radiation treatment was 9.5 years. The average age at diagnosis was 53 years (range, 19–76 years). The most common location was the maxillary sinus (four of seven patients). Other locations included ethmoid sinus, sphenoid sinus, inferior turbinate, and nasal septum. Most lesions involved multiple subsites. All patients were treated with surgical resection. Overall, three patients had neoadjuvant chemotherapy followed by surgical resection. Overall, two patients had surgical resection alone. One patient had postoperative radiation for positive margins. Two patients developed recurrent disease. All patients survived with a length of follow-up ranging from 2 to 98 months (median follow-up of 49 months). Disease-free survival at 5 years was 66.7%. Overall survival at 5 years was 100%.

Conclusion: Sarcomas of the sinonasal tract represent a diverse group of tumors that together represent a rare malignancy of this location. Our series of patients had improved survival compared with other studies. Also, in patients who had received prior radiation for other sinonasal disease, sarcoma appeared relatively soon posttreatment which is an unusual feature for radiation-induced sarcoma.