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Alveolar Soft Part Sarcoma

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 38 Accesses

Definition

Alveolar soft part sarcoma (ASPS) is an extremely rare mesenchymal tumor of uncertain histogenesis. It is most common in young adults occurring predominantly in deep soft tissues of the extremities (Christopherson et al. 1952). Molecular hallmark is represented by a recurrent translocation, der(17)t(X;17)(p11.2;q25), resulting in a ASPSCR1-TFE3 gene fusion (Ladanyi et al. 2001).

Clinical Features

  • Incidence

    It accounts for less than 1% of all soft tissue sarcomas.

  • Age

    The peak incidence is in the second decade (median age: 25 years); however, the patient age range is rather broad (from 1 to 80 years) (Christopherson et al. 1952; Wang et al. 2016).

  • Sex

    Female predilection is mostly observed mostly in patients over 30 years and in children (Christopherson et al. 1952; Wang et al. 2016; Ogose et al. 2003).

  • Site

    Alveolar soft part sarcoma occurs most frequently in the deep soft tissue of lower extremities. In pediatric age, it involves mostly the head and neck region. More rarely...

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References and Further Reading

  • Argani, P., Antonescu, C. R., Illei, P. B., et al. (2001). Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: A distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. The American Journal of Pathology, 159, 179–192.

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  • Cho, Y. J., & Kim, J. Y. (2014). Alveolar soft part sarcoma: Clinical presentation, treatment and outcome in a series of 19 patients. Clinics in Orthopedic Surgery, 6(1), 80–86.

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  • Christopherson, W. M., Foote, F. W., & Stewart, F. W. (1952). Alveolar soft-part sarcomas; structurally characteristic tumors of uncertain histogenesis. Cancer, 5(1), 100–111.

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  • Flores, R. J., Harrison, D. J., Federman, N. C., et al. (2018). Alveolar soft part sarcoma in children and young adults: A report of 69 cases. Pediatric Blood & Cancer, 65(5), e26953.

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  • Green, W. M., Yonescu, R., Morsberger, L., et al. (2013). Utilization of a TFE3 break-apart FISH assay in a renal tumor consultation service. The American Journal of Surgical Pathology, 37, 1150–1163.

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  • Groisberg, R., Hong, D. S., Behrang, A., et al. (2017). Characteristics and outcomes of patients with advanced sarcoma enrolled in early phase immunotherapy trials. Journal for Immunotherapy of Cancer, 5, 100.

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  • Ladanyi, M., Lui, M. Y., Antonescu, C. R., et al. (2001). 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. Oncogene, 20(1), 48–57.

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  • Lam, S. W., Cleton-Jansen, A. M., Cleven, A. H. G., et al. (2018). Molecular analysis of gene fusions in bone and soft tissue tumors by anchored multiplex PCR-based targeted next-generation sequencing. The Journal of Molecular Diagnostics, 20, 653–663.

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  • Ogose, A., Yazawa, Y., Ueda, T., et al. (2003). Alveolar soft part sarcoma in Japan: Multi-institutional study of 57 patients from the Japanese Musculoskeletal Oncology Group. Oncology, 65(1), 7–13.

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  • Rekhi, B., Ingle, A., Agarwal, M., et al. (2012). Alveolar soft part sarcoma ‘revisited’: Clinicopathological review of 47 cases from a tertiary cancer referral centre, including immunohistochemical expression of TFE3 in 22 cases and 21 other tumours. Pathology, 44, 11–17.

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  • Wang, H., Jacobson, A., Harmon, D. C., et al. (2016). Prognostic factors in alveolar soft part sarcoma: A SEER analysis. Journal of Surgical Oncology, 113(5), 581–586.

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Author information

Authors and Affiliations

  1. Department of Pathology, Azienda Ospedale Università Padova, Padova, Italy

    Marta Sbaraglia

Authors
  1. Marta Sbaraglia
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Correspondence to Marta Sbaraglia .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Sbaraglia, M. (2020). Alveolar Soft Part Sarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5343-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5343-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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