Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Mediastinal Alveolar Soft Part Sarcoma

  • Annikka WeissferdtEmail author
  • Cesar A. Moran
Reference work entry


ASPS; Malignant granular cell myoblastoma; Malignant myoblastoma; Malignant tumor of non-chromaffin paraganglia


Alveolar soft part sarcoma (ASPS) is a rare and very distinctive sarcoma of young patients. The tumor was originally described in 1952 as a unique soft tissue malignancy and named after its distinctive pseudoalveolar histologic appearance (Christopherson et al. 1952). Since its first description, numerous reports of such tumors have been published; however, uncertainty still exists today with regard to the line of differentiation and clinical behavior of these lesions. Speculation about the cell of origin had previously included the non-chromaffin paraganglia, a malignant form of angioreninoma or an unusual myogenic tumor; however, over the years ultrastructural and immunohistochemical studies have failed to support these assumptions. The more recent discovery that ASPS belongs to a group of translocation-associated sarcomas has raised the possibility...

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References and Further Reading

  1. Christopherson, W. M., Foote, F. W., Jr., & Stewart, F. W. (1952). Alveolar soft-part sarcomas; structurally characteristic tumors of uncertain histogenesis. Cancer, 5, 100–111.PubMedCrossRefGoogle Scholar
  2. Flieder, D. B., Moran, C. A., & Suster, S. (1997). Primary alveolar soft-part sarcoma of the mediastinum: A clinicopathological and immunohistochemical study of two cases. Histopathology, 31, 469–473.PubMedCrossRefGoogle Scholar
  3. Folpe, A. L., & Deyrup, A. T. (2006). Alveolar soft-part sarcoma: A review and update. Journal of Clinical Pathology, 2006(59), 1127–1132.CrossRefGoogle Scholar
  4. Karnauchow, P. N., & Magner, D. (1963). The histogenesis of alveolar soft part sarcoma. Journal of Pathology and Bacteriology, 86, 169–177.PubMedCrossRefGoogle Scholar
  5. Khanna, P., Paidas, C. N., & Gilbert-Barness, E. (2008). Alveolar soft part sarcoma: Clinical, histopathological, molecular, and ultrastructural aspects. Fetal and Pediatric Pathology, 27, 31–40.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.MD Anderson Cancer CenterHoustonUSA