The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Strong similarity to renal cell carcinoma, clear cell type Variably cellular smears composed of large cells with an abundant finely … Nakashima Y, Kotoura Y, Kasakura K, Yamamuro T, Amitani R, Ohdera K. Alveolar soft-part sarcoma. Founded in 2006, Cure Alveolar Soft Part Sarcoma International aims to connect ASPS patients and their families and friends with the best information available about this rare sarcoma. 5% of all soft-tissue sarcomas. Cancer 1952; 5: 100-11.8. A clinico-pathologic study of half a century. Adv Anat Pathol 1999; 6: 125-39.Exons 3-8 or 4-8: The breakpoint in ASPSCR1 (17q25; blue), previously termed ASPL, is invariant. The overall prognosis is poor given the high frequency of metastatic disease 1 . It may also be called alveolar soft tissue sarcoma, alveolar sarcoma of soft parts, or ASP sarcoma. A comprehensive retrospective review was performed of clinical presentation, treatment, outcome, and patterns of failure in a consecutive series of patients with localized or metastatic ASPS between 1975 and 2008. Alveolar soft-part sarcomas are clinically and morphologically distinct soft-tissue sarcomas, with an unknown histogenesis. Ultrastruct Pathol 1988; 12: 137-44.ASPS is one of more than 50 kinds of sarcoma. Soft tissue sarcoma. Alveolar soft part sarcoma (ASPS) is a rare neoplasm. the usual nature of the Alveolar Soft Part Sarcoma is relatively indolent, but almost 79 percent of affected individuals develop metastatic disease. Ackerman L, Phelps C. Malignant granular cell myoblastoma of the gluteal region. It accounts for 0.5 to 1% of all soft tissue sarcomas. Smetana H, Scott W. Malignant tumors of nonchromaffin paraganglia. ASPS usually starts in the leg, although it can also begin in other parts of the body, including the arms, head, or neck. A report of ten cases. Analysis charges will be incurred based on the number of cells analyzed per probe set. In children, ASPS can be found in the head and neck, especially around the tongue or eye socket. 22. PAS+. Alveolar soft part sarcoma; Other names: Alveolar soft-tissue sarcoma: Micrograph of an alveolar soft part sarcoma, showing the characteristic alveolar-like architecture and cells with eccentric nuclei and abundant eosinophilic cytoplasm. Only 0.2 to 1 percent represents Alveolar Soft Part Sarcoma of all types of soft tissue sarcoma. PAS-D. TFE3. It is most frequently found in young adults and teenagers and often begins in the lower extremities. Alveolar soft‐part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young patients. TFE3 fusions activate MET signaling by transcriptional up-regulation, defining another class of tumors as candidates for therapeutic MET inhibition. Therapeutic vulnerability of an in vivo model of alveolar soft part sarcoma (ASPS) to antiangiogenic therapy. The fusion of the genetic material synthesis an abnormal protein.The confirmatory result can be obtained by performing a biopsy. Salvati M, Cervoni L, Caruso R, Gagliardi FM, Delfini R. Sarcoma metastatic to the brain: a series of 15 cases. Alveolar soft part sarcoma. Demetri GD, Antonia S, Benjamin RS, et al. Prolonged survival is possible even with metastasis, although the long-term disease-related mortality rate is high. Ordonez NG, Mackay B. Alveolar soft-part sarcoma: a review of the pathology and histogenesis. Alveolar soft part sarcoma. Every person is different and prognosis will depend on many factors, such as:You can help speed up the development of new treatments by giving researchers the tools they need.Alveolar soft part sarcoma, or ASPS, is a cancer that can come from different types of soft tissue, such as muscle, fat, or nerves.