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alveolar soft part sarcoma

Monday 8 March 2004, by JA

alveolar soft-part sarcoma


Definition: Alveolar soft-part sarcoma is a rare soft tissue malignant tumor that commonly arises in skeletal muscles or musculofascial planes in children and young adults.

Digital cases

- JRC:3169 : Alveolar soft-part sarcoma.
- JRC:11247 : Alveolar soft-part sarcoma.


- alveolar soft part sarcoma (ASPS). IHC: +TFE3. NB: pseudoalveolar pattern w/ central discoherence; vascular invasion a common finding

- compact alveolar soft part sarcoma


- well defined nests of cells separated by fibrous stroma
- alveolar pattern if cells discohesive
- composed of large polygonal cells with granular eosinophilic cytoplasm, vesicular nuclei, prominent nucleoli
- vascular invasion common; also characteristic rod-shaped crystalloids
- no / rare mitotic figures, minimal pleomorphism


- soft parts
- lung (26369552)


- t(X;17)(p11;q25) (12769020, 12045953, 11244503)(TFE3/ASPL gene fusion)

Molecular biology

- ASPSCR1-TFE3 / ASPL-TFE3 gene fusion


- frequent metastases to lung, brain, and bone


- Zhong M, De Angelo P, Osborne L, Keane-Tarchichi M, Goldfischer M, Edelmann L, Yang Y, Linehan WM, Merino MJ, Aisner S, Hameed M. Dual-color, break-apart FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of Xp11 translocation renal cell carcinoma and alveolar soft part sarcoma. Am J Surg Pathol 2010;34(6):757-66. PMID: 20421778

- Lazar AJ, Lahat G, Myers SE, Smith KD, Zou C, Wang WL, Lopez-Terrada D, Lev D. Validation of potential therapeutic targets in alveolar soft part sarcoma: an immunohistochemical study utilizing tissue microarray. Histopathology. 2009 Dec;55(6):750-5. PMID: 20002771

- Aulmann S, Longerich T, Schirmacher P, Mechtersheimer G, Penzel R. Detection of the ASPSCR1-TFE3 gene fusion in paraffin-embedded alveolar soft part sarcomas. Histopathology. 2007 Jun;50(7):881-6. PMID: 17543078

- Pennacchioli E, Fiore M, Collini P, Radaelli S, Dileo P, Stacchiotti S, Casali PG, Gronchi A. Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment, and Outcome in a Series of 33 Patients at a Single Institution. Ann Surg Oncol. 2010 Jul 1. [Epub ahead of print]. PMID: 20593242