Alveolar Soft Part Sarcoma
Alveolar soft part sarcoma (ASPS) is a rare sarcoma that typically arises in the lower extremity in adolescents and young adults between 15 and 40 years of age. Distribution by age and site in adults is shown in Figs. 17.1 and 17.2 for all adult ASPS. In children, a number of cases arise from the tongue and orbit, where it can be confused to some degree with embryonal rhabdomyosarcoma. ASPS is extremely rare, even at referral centers, which have been hard pressed to identify more than one to two patients a year per center in published series [1–3]. It frequently presents with innumerable small, round metastatic lesions in the lungs and shows a very slow rate of progression, one reason for the late presentation of what is often a primary >10 cm in greatest dimension. Progression is typically slow, but ultimately taking the patient’s life after 10–15 years of metastatic disease. Brain metastases are a particular feature of ASPS, with an incidence at least thrice of that of other sarcomas in one series and documentation in other series [4–7].
KeywordsMetastatic Disease Brain Metastasis Tyrosine Kinase Inhibitor Late Presentation Great Dimension
- 2.Pennacchioli E, Fiore M, Collini P, et al. Alveolar soft part sarcoma: clinical presentation, treatment, and outcome in a series of 33 patients at a single institution. Ann Surg Oncol. 2010:17(12):3229–33.Google Scholar
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