A pediatric case of Alveolar Soft Part Sarcoma.Rev Stomatol Chir Maxillofac. 2007 Dec;108(6):547-50. Epub 2007 Oct 26.

INTRODUCTION: Alveolar Soft Part Sarcoma (ASPS) is a rare malignant tumor arising from skeletal muscles and occurring usually in young adults. It is a solid chemo resistant tumor. At time of diagnosis the disease is usually advanced and lung metastasis are present in 65% of the cases. The translocation (X; 17), producing an ASPL-TFE3 transcript fusion which is detected on tumoral cells wall, is specific of ASPS. The curative treatment is surgical. CLINICAL REPORT: We report the case of a child with an ASPS of the tongue and lung metastasis. DISCUSSION: This report presents two particularities. Firstly this type of sarcoma is very exceptional in a 2-year-old child. Secondly ASPS is not usually susceptible to chemotherapy.

 Alveolar soft part sarcoma of the endometrium with expression of CD10 and hormone receptors.APMIS. 2007 Jul;115(7):861-5.

Alveolar soft part sarcoma (ASPS) is a rare tumor of uncertain histogenesis, mainly localized in the extremities. ASPS originating in the uterine corpus is quite rare; only eight such cases have been reported in the literature. We here present another case of ASPS found in the endometrium in a 50-year-old woman. Metastatic malignant tumor, including ASPS from other organs, was excluded by physical examination and imaging modalities. Thallium 201 was only localized in the uterus. The tumor showed characteristic histological features of ASPS: alveolar architecture with fibrovascular septa and abundant eosinophilic granular cytoplasm with periodic acid-Schiff-positive crystalline material. Diffuse nuclear immunoreactivity for TFE3, a marker recently reported to be specific for ASPS, further supported the diagnosis of ASPS. Interestingly, this tumor was negative for myogenic markers, but positive for CD10, progesterone receptor, and estrogen receptor. These immunohistochemical results and the tumor location suggest a possible link between endometrial stromal cells and the development of this tumor.