Alveolar Soft Part Sarcoma

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  • Unknown cell of origin (although possibly striated muscle)


  • Should be considered esp in anterior thigh lesions as bx can result in a large amount of bleeding
  • ?0.5-1% of all soft-tissue sarcomas


  • Usually occurs in adolescents and young adults
  • F>M
  • ? pulsatilve mass/bruit
  • Thigh and buttock most common location in adults
  • Head and neck most common in children and infants (orbit, tongue)
  • Slow growing and painless
  • Cerebral metastases cause sxs
  • Unusual locations


  • X-rays may reveal the rare underlying bony erosion
  • Nuclear medicine studies
    • Bone scan shows soft-tissue uptake
    • FDG PET
      • Heterogeneous intense uptake to variable uptake reported
  • Angiogram can show the rich vascularity
    • Large vessels sometimes seen with marginal fat suggesting AV malformation
  • CT: soft tissue mass with attenuation ? to skeletal muscle
    • Lesion enhances markedly, especially peripherally
  • MRI: intermediate signal intensity on T1-weighted images (90% greater than muscle--due to slowly flowing blood through the extensive vascularity) and very bright intensity on T2; intense uptake of gadolinium
    • Poorly defined margins, serpiginous vessels evident (serpentine flow voids in 67%)
    • Central necrosis in 75%


  • Extremely vascular lesion, well to poorly circumscribed and friable, occasionally extending to the subcutaneous tissue
  • Yellow-white-gray-red with foci of hemorrhage, extracapsular invasion


  • Large rounded to polygonal tumor cells usually in nests in a pseudoalveolar pattern (from poor central cellular cohesion) separated by vascular spaces
  • Cells have abundant granular cytoplasm (intracytoplasmic crystals) and distinct cell borders
  • Vascular invasion is common
  • ± ? pleomorphism
  • ± ? mitoses
  • Peripherally dilated veins
  • Special stains:
    • + PAS (diastase resistant) rod-shaped crystals (80%)
    • + reticulin outlining nests of cells
  • Immunohistochemistry:
    • S-100 and neuron-specific enolase occasionally +
    • Vimentin, muscle-specific actin, and desmin have been reported by some to be +
    • Cathepsin K +
      • ASPSCR1-TFE3 t(X;17)(p11;q25) carcinomas - for cathepsin K
    • + VEGFR 1, 2, 3 reported
    • + PDGRR- alpha and beta reported
  • Cytology
    • Fragility of cytoplasm
    • Prominence of bare nuclei
    • Granular-appearing background or the presence of cytoplasmic fragments
    • 3-dimensional alveolar structures
  • EM:
    • Rod-shaped crystals (structurally similar to actin filaments) in a lattice pattern
    • Prominent SER and Golgi apparatus
    • Nests of tumor cells surrounded by a basal lamina
  • Cytometry:
    • Usually a diploid peak
  • Cytogenetics
    • t(X;17)(p11;q25) results in ASPSCR1-TFE3 fusion gene*** fusion product functions as aberrant transcription factor
      • nuclear immunoreactivity for TFE3
      • immunoreactivity for monocarboxylate transporter 1 and CD147
    • trisomy 7, monosomy 8, and monosomy 18 also reported
    • Dual-color break-apart FISH assay reported


  • Arteriovenous malformation
  • Hemangioma
  • MFH
  • PVNS
  • Metastatic renal cell carcinoma
  • Paraganglioma
  • Granular cell tumor
  • Melanoma
  • Rhabdomyosarcoma


  • Massive hemorrhage can occur with bx or resection
  • Up to 1/3 develop metastases before detection of a primary
  • ?60% 5 yr survival falls to 15% at 20 yrs (metastases reported 33 yrs after inital presentation)
  • poor response to chemotherapy and/or radiation
  • Novel txs
    • Sunitinib
      • Multitargeted receptor tyrosine kinase inhibitor
        • Care must be taken postop with STSG as necrosis has been reported
        • Mediated by PDGFRB, VEGFR2, and RET (all expressed in tumor cells)


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