ABSTRACT
Biphenotypic sinonasal sarcoma (BSNS) is a recently described, low-grade, slow-growing sarcoma with neural and myogenic features with exclusive location in sinonasal track and characteristic PAX3- MAML3 gene fusion. Differentiating this tumor from its commoner mimics needs knowledge of this entity to avoid over treatment. This tumor has unique morphology, clinical course, and genetics. We report this in a 47-year-old female who was diagnosed with such a rare, solitary fibrous tumor—hemangiopericytoma (HPC-SFT) on limited initial biopsy. On subsequent excision, typical morphology and immunohistochemistry helped to clinch the diagnosis.
ABSTRACT
BACKGROUND AND OBJECTIVES: Sinonasal sarcomas are relatively rare tumors, accounting for only about 1 percent of all sinonasal malignancies. The purpose of this study is to analyze the clinical features, treatment modalities and outcomes, survival and prognostic factors of sinonasal sarcomas. SUBJECTS AND METHOD: We retrospectively reviewed the medical records of 20 patients who were diagnosed and treated for sinonasal sarcomas between July 1993 and June 2006. Potential prognostic factors including age, tumor size, histology and adjuvant treatment were evaluated. RESULTS: According to histologic subtype, twenty cases consisted of 7 rhabdomyosarcomas, each 2 cases of spindle cell sarcoma, Ewing's sarcoma, angiosarcoma, and each 1 case of fibrosarcoma, leiomyosarcoma, hemangiopericytoma, peripheral neuroectodermal tumor, myxoid sarcoma, osteosarcoma, chondrosarcoma. The mean follow-up period was 61 months. Fourteen patients (65%) had undergone surgical approach with adjuvant chemotherapy or radiotherapy. Four patients (20%) had undergone surgical treatment only. Two patients (15%) had undergone chemotherapy or radiotherapy. At 5 years, the overall survival rates of sinonasal sarcomas were 65%. The survival rates according to the treatment modality were 100% (surgery only), 66% (surgery with adjuvant chemotherapy or radiotherapy), and 33% (chemotherapy or radiotherapy), respectively. The overall recurrence rates of the sinonasal sarcomas were 25%. The univariate analysis showed that the treatment modality and surgical margin are important prognostic factors for survival. CONCLUSION: The 5-year survival rate of sinonasal sarcomas was 65%. Early diagnosis and wide excision with safe resection margin are important for the treatment of sinonasal sarcomas.