Alveolar soft part sarcoma of the head and neck. A disease of children and young adults.

Abstract

Alveolar soft part sarcoma (ASPS) is a rare malignancy of uncertain histologic origin that predominantly afflicts female children and young adults. Forty-three cases occurring in the head and neck have been identified, to which we add a case. The most frequent sites of occurrence in the head and neck are orbit and tongue. Treatment is universally surgical, with limited roles for adjuvant radiotherapy and chemotherapy. Representative populations of patients with orbital, non-orbital and extremity ASPS are compared statistically. Improved disease-free intervals in orbital ASPS vs non-orbital ASPS and head and neck ASPS vs extremity ASPS are documented. Debate exists regarding the histologic origin of ASPS. The additional case presented does not corroborate recent immunohistochemical studies suggesting myogenic origin. Nevertheless, the clinical pattern of disease offers additional support to the myogenic hypothesis of histologic origin.