Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis of 18 patients

Abstract

Abstract: PURPOSE : To summarize the clinical features, diagnosis, treatment strategies and prognosis of alveolar soft part sarcoma (ASPS) of oral and maxillofacial region. METHODS : We performed a retrospective study in a consecutive series of 18 patients with ASPS in the oral and maxillofacial region between 1995 and 2013. Demographics, tumor sizes, sites, metastasis and diagnosis of disease, treatments provided, and follow-up outcomes were documented and analyzed. RESULTS : Eighteen patients (10 females, 8 males mean age of 20.2 years, range, 3-61 years) who were diagnosed pathologically had their primary tumor in the tongue (10), cheek (5), pharynx (1), gingival (2), with an average size of 4 cm (range, 1.5-6.5 cm). Two patients exhibited pulmonary metastases. Extensive resections were performed in all patients. Postoperative radiotherapy was delivered in 3 cases, and adjuvant systemic chemotherapy was given to 1 case. Mean follow-up periods were 33 months (range, 6-75 months). At the latest follow-up, one patient with lung metastases survived for 19 months, and one died 3 months after confirmation of local recurrence and multiple pulmonary metastases, the other patients remained in good health and were disease-free. CONCLUSIONS : ASPS of the oral and maxillofacial region has unique clinical characteristics. The immunohistochemical findings and PAS-positive results help to make the final diagnosis. The most commonly advocated and effective treatment for ASPS of oral and maxillofacial region remains to be radical surgical excision of the lesions.

Key words: Alveolar soft part sarcoma, Oral and maxillofacial region, Clinical features

CLC Number:

R739.8

WANG Hong-wei, QIN Xing-jun, YANG Wen-jun, XU Li-qun, JI Tong, ZHANG Chen-ping. Alveolar soft part sarcoma of the oral and maxillofacial region: clinical analysis of 18 patients[J]. China Journal of Oral and Maxillofacial Surgery, 2014, 12(6): 543-548.

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