Journal
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
Volume 12, Issue 5, Pages 383-389Publisher
SAGE PUBLICATIONS INC
DOI: 10.2350/08-08-0518.1
Keywords
alveolar rhabdomyosarcoma; PAX3-FKHR fusion; prostate
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Alveolar rhabdomyosarcoma (RMS) is I of 2 plain subtypes of RMS in the pediatric age group and tends to occur in the extremities. The urogenital tract is another common site for RMS, but this typically involves the embryonal subtype including sarcoma botryoides We report a 28-year-old male with a prostatic tumor that was excised en bloc and showed a RMS with separate areas of embryonal and solid alveolar morphologies at the light microscopic level. Both areas showed diffuse nuclear expression for myogenin, and both areas expressed the PAX3-FKHR fusion gene, a genetic change associated with alveolar but not embryonal RMS. A review of the literature documented only 5 cases of RMS primary to the prostate showing alveolar or mixed histology Ours is the 6th case and the 1st with molecular findings. Although the diagnostic category of mixed embryonal/alveolar RMS remains in use, the nature of this type of RMS is incompletely understood. In our case, although the morphology was mixed embryonal/alveolar, at the genetic level this tumor was alveolar in nature.
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