Alveolar Soft Part Sarcoma: A Single-Center 26-Patient Case Series and Review of the Literature

Abstract

Background. Alveolar soft part sarcoma (ASPS) is a rare tumor, and little information is available regarding its clinical features and appropriate treatments. Methods. A retrospective review of 26 consecutive ASPS patients (12 male, 14 female; mean age of 27 years) treated at our institution over 30 years (mean followup; 71 months) was performed. Results. The primary tumor developed in the lower extremity (12), trunk (8), and upper extremity (6), with an average size of 7.2 cm (range, 2–14 cm). The AJCC stage at presentation was IIA (7), III (3), and IV (16). Surgical excision was performed in 20 patients (R0 18, R1 plus radiotherapy 2) without local recurrence. Six patients (stage IIA 3/7, stage III 3/3) later developed metastases after an average period of 28.7 months. The median survival of the 26 patients was 90 months, with overall 5/10-year survival rates of 64%/48%. AJCC stage and tumor size were significant prognostic factors. Significant palliation and slowing of metastasis progression were achieved with gamma knife radiotherapy. Nine patients receiving chemotherapy showed no objective response. Conclusions. ASPS is indolent but has a high propensity for metastasis. Early diagnosis and complete excision of the small primary tumor are essential in the treatment of ASPS.

Magnetic resonance imaging and computed tomography features of alveolar soft-part sarcoma in the right deltoid muscle: A case report

“…The tumor was well enhanced on contrast-enhanced CT and MRI, and exhibited vascular signal voids. These reliable radiologic findings are well correlated with the high vascularity of ASPS in pathological specimens (10).…”

Huge alveolar soft part sarcoma of the retroperitoneum –

“…ASPS has a slight female predilection in younger patients, which is hypothesized to be caused by an extra X chromosome that may be susceptible to mutation [10]. However, there is a male preponderance in older patients [5,11]. Despite all the latest findings, the origin of ASPS is still unknown.…”

“…The frequency of brain metastases is almost three times higher than in other sarcomas. In most cases they are associated with other extracranial metastases, such as lungs and bones [5,11]. The frequency of local recurrence varies among large studies between 10% and 31% [11][12][13].…”

“…It is characterized by indolent growth with a high frequency of late metastases [4]. Brain metastases are present almost 3 times more often than in other sarcomas [5]. It usually appears as a painless mass, which may be highly vascularized in radiological findings.…”

Alveolar Soft Part Sarcoma

“…Differences in biologic characteristics of pediatric and adult ASPS have been proposed as a possible explanation. 15 In a case-series study reported by Ogura et al, 29 pediatric tumors tended to be smaller than those in adult patients, with the larger tumor size resulting in increased risk of distant metastasis. Radical resection is the treatment of choice for localized disease.…”