Characterization of alveolar soft part sarcoma of the tongue: A clinico-pathologic study and scoping review

Abstract

Background: Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumour. There is a dearth of literature analyzing its features on the tongue.
Objectives: This study aims to conduct a scoping review to describe the essential clinico-pathologic features, treatment modalities and outcome of previously reported tongue ASPS (TASPS) and new cases at our center.
Methods: A search of databases (PubMed, Medline, Cochrane and Google Scholar) and the internet for articles on TASPS written in English was conducted. Information extracted included clinico-pathological and demographic data. Descriptive statistics was used for analysis.
Results: A total of 49 articles were eligible for this study. In all, 81 cases were utilized. Asian studies accounted for most cases 35(43.2%) and a slight female preponderance of 1.1 was seen. Most cases - 38 (46.9%), occurred in the 1st decade and the base of tongue was the most common location in 19 (39.6%) cases. Also, tumour metastasis was present in 14 (25.9%) cases. Transcription Factor E3 (TFE3) – 8 (24.2%) and Neuron Specific Enolase (NSE) – 8 (24.2%) were the most common immunohistochemical stains used and were both expressed 7 out of 8 cases (87.5%). Most common treatment modality was surgery and 42 (82.4%) cases managed by surgery alone were free of disease at < 5 years of follow up.
Conclusions: TASPS slightly affected the female gender and tongue base more commonly. It occurred more in the first two decades of life. Use of standard investigative tools for management will allow for better appraisal of research findings.