Alveolar soft-part sarcoma of the head and neck: clinical and imaging features in five cases.

Background And Purpose: Alveolar soft-part sarcoma (ASPS) of the head and neck is an extremely rare malignancy. Although the clinical and imaging features of this tumor have been reported, a periodic review of unusual tumors is useful. The purpose of this study was to describe the clinical and imaging features of ASPS of the head and neck.

Methods: Between January 1990 and May 2004 at our institution, five head and neck ASPS were diagnosed in five patients (two male and three female patients; age range, 4-22 years). Clinical and imaging findings were reviewed retrospectively. Imaging studies consisted of contrast material-enhanced CT (in four patients), MR imaging (in four patients), and digital subtraction angiography (in two patients).

Results: The locations of the tumor were tongue in two cases, larynx in one case, buccal space in one case, and paravertebral space in one case. This tumor appeared as a large lobulating-contoured mass with high signal intensity and flow voids on T2-weighted images and showed strong enhancement on contrast-enhanced CT and MR images. Preoperative angiography showed high vascularity. Wide surgical excisions were performed in four cases. Mean follow-up periods were 16 months (range, 6-30 months), and no recurrence was noted except for the laryngeal case.

Conclusion: ASPS should be included in the differential diagnosis of head and neck tumor when a slow-growing, large mass with high signal intensity and flow voids on T2-weighted images and strong enhancement on contrast-enhanced CT or MR image is seen, particularly in young female patients.