Alveolar Soft Part Sarcoma of the Oro-Maxillofacial Region in the Pediatric Age Group: Immunohistochemical and Ultrastructural Diagnosis of Two Cases

Abstract

Alveolar soft part sarcoma (ASPS) is infrequent in children. While head and neck locations, including the orbit and tongue, are described, only six cases of sinonasal ASPS are reported in the literature. We report two cases of pediatric oro-maxillofacial ASPS. The first case presented as a sinonasal mass in a 13-year-old girl, while the second was a tongue lesion in a 4-year-old female. Histologic examination, TFE3 immunopositivity, and ultrastructural findings of rhomboid crystalline inclusions helped confirm the diagnosis. The diagnosis of ASPS is challenging in children and in uncommon sites like the head and neck. Patients should be routinely followed up for detection of residual or recurrent disease, particularly in cases with positive resection margins.