Mesenchymal / mixed epithelial & mesenchymal tumors

Alveolar soft parts sarcoma

Topic Completed: 1 May 2007

Minor changes: 17 May 2021

Copyright: 2007-2021, PathologyOutlines.com, Inc.

PubMed Search: Alveolar soft parts sarcoma cervix

Branko Perunovic, D.M.
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Cite this page: Perunovic B. Alveolar soft parts sarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cervixalveolarsoftpartssarcoma.html. Accessed December 3rd, 2021.
Definition / general
  • Very rare
  • Usually ages 30 to 40 years
  • Associated with abnormal uterine bleeding
  • Patients often do well, but may die of metastatic disease
Case reports
Microscopic (histologic) description
  • Well circumscribed tumor with loss of central cohesion causing a pseudoalveolar pattern
  • Nests are separated by thin - walled, sinusoidal vascular spaces
  • Cells are large with distinct cell borders, resembling gemistocytic astrocytes
  • Contain PAS+ diastase resistant intracytoplasmic crystals
  • Small nuclei with prominent nucleoli
Microscopic (histologic) images

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Nests of tumor cells with PAS+ crystals

Positive stains
Negative stains
Molecular / cytogenetics description
  • t(X;17)(p11;q25) - TFE3 - ASPL fusion transcript
Electron microscopy description
  • Rhomboid, rod - shaped or spicular crystals with a regular lattice pattern and electron dense secretory granules
  • Crystals consist of filaments 6 nm in diameter, arranged in parallel arrays with periodicity of 10 nm
  • Basal lamina surrounds groups of tumor cells with prominent mitochondria, glycogen and lipid
Differential diagnosis
  • Clear cell carcinoma: often papillary or cystic with hobnail cells, cytoplasm is more clear, may have focal PAS+ areas in cytoplasm, but diastase sensitive
  • Metastatic renal cell carcinoma
  • Paraganglia: solid nests of neuroendocrine cells surrounded by S100+ sustentacular cells; negative for muscle markers, no PAS+ diastase resistant crystals
Additional references
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