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Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other
Anaplastic variant of embryonal rhabdomyosarcoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 16 July 2009, last major update July 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Clinical
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● 4% of embryonal rhabdomyosarcoma
● Mean age 6 years, 2/3 male, various sites
● Diffuse cases may have poorer prognosis (Am J Surg Pathol 1993;17:443)
Micro description
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● Enlarged atypical cells with hyperchromatic nuclei (3x larger than nuclei in neighboring tumor cells), often with atypical mitotic figures
● May be focal or diffuse
● Classic embryonal rhabdomyosarcoma also present
Micro images
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Extremely hyperchromatic nuclei are 3x larger than nuclei of adjacent tumor cells, mixed with smaller, undifferentiated, round and spindle cells
Bizarre mitotic figure (left side) Rare cells suggest skeletal muscle differentiation
Desmin staining
Positive stains
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● Muscle specific actin (80%), desmin (60%)
Molecular
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● Associated with 12q13-15 amplification (Histol Histopathol 2004;19:401)
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Anaplastic variant of embryonal rhabdomyosarcoma
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