Alveolar Soft Part Sarcoma

Alveolar Soft Part Sarcoma (ASPS) is a rare orphan malignant soft tissue tumor of uncertain cellular lineage with a somewhat indolent clinical course. It has been first described in 1952 by Christopherson, a fellow in surgical pathology at Memorial Sloan Kettering Cancer Center who reported 12 cases with similar clinical and pathology features (Christopherson et al., Cancer 5:100–111, 1952). Indeed, ASPS present a very distinctive clinical pattern compared to other sarcomas with a peculiar natural history marked by a high prevalence of brain metastasis, predominance in female patients, chemoresistance, and sensitivity to tyrosine kinase inhibitors. ASPS is driven by a specific translocation that involves chromosome X. Accurate diagnosis requires trained pathologists and molecular testing to lead the treatment accordingly, which should be based surgery if resectable and on tyrosine kinase inhibitors upfront in metastatic setting. This rare disease strikes the need to collaborate between expert centers worldwide and cross the boundaries between pediatric and adult oncology.