Brain metastasis of crystal-deficient, CD68-positive alveolar soft part sarcoma: ultrastructural features and differential diagnosis

Ultrastruct Pathol. 2015 Feb;39(1):69-77. doi: 10.3109/01913123.2014.950778. Epub 2014 Sep 30.

Abstract

We report a case of alveolar soft part sarcoma (ASPS) presenting as an isolated frontal lobe metastasis. The tumor demonstrated little or no immunoreactivity for a broad panel of antibodies yet strong, diffuse immunoreactivity with CD68. On electron microscopy, the characteristic rectangular to rhomboid crystalline inclusions of ASPS were not present. Electron-dense granules resembling peroxisomes were present, sometimes in association with elongated granular structures having a periodic, lattice-like arrangement. Metastatic ASPS was confirmed by demonstration of an ASPSCR1-TFE3 fusion and imaging studies that excluded metastatic Xp11.2 translocation renal cell carcinoma. The primary site was subsequently identified in the lower extremity.

Keywords: Alveolar soft part sarcoma; CD68; brain metastasis.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / genetics
  • Brain Neoplasms / diagnosis
  • Brain Neoplasms / secondary*
  • Brain Neoplasms / ultrastructure*
  • Carcinoma, Renal Cell / diagnosis
  • Diagnosis, Differential*
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Intracellular Signaling Peptides and Proteins
  • Leg / pathology
  • Male
  • Microscopy, Electron, Transmission
  • Oncogene Proteins, Fusion / genetics
  • Recombinant Fusion Proteins / genetics
  • Sarcoma, Alveolar Soft Part / diagnosis*
  • Sarcoma, Alveolar Soft Part / secondary*
  • Sarcoma, Alveolar Soft Part / ultrastructure*

Substances

  • ASPSCR1 protein, human
  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
  • CD68 antigen, human
  • Intracellular Signaling Peptides and Proteins
  • Oncogene Proteins, Fusion
  • Recombinant Fusion Proteins
  • TFE3 protein, human