UI - 21411341
AU - Liang SX; Lakshmanan Y; Woda BA; Jiang Z
A high-grade primary leiomyosarcoma of the bladder in a survivor of
SO - Arch Pathol Lab Med 2001 Sep;125(9):1231-4
AD - Department of Pathology, University of Massachusetts Medical School,
Worcester, MA 01655, USA.
Second nonocular malignancies develop with increased incidence in
patients with hereditary retinoblastoma. Osteosarcoma is by far the most
common type with an incidence of up to 50%, followed by soft tissue
sarcomas. Visceral leiomyosarcoma is extremely rare and only 2 cases
have been reported in the past 2 decades, one in the liver and another
one in the urinary bladder, both of which developed after
cyclophosphamide therapy. Here we report a case of vesical
leiomyosarcoma that was diagnosed in a 49-year-old woman 47 years after
the diagnosis of a hereditary retinoblastoma. The patient's
retinoblastoma was treated with unilateral enucleation without adjuvant
radiation or chemotherapy. We believe that this is the first report of
vesical leiomyosarcoma occurring in a patient with retinoblastoma
without a prior history of radiation or chemotherapy. This report is
significant not only because of the rarity of vesical leiomyosarcoma as
a second nonocular tumor in retinoblastoma patients, but also because of
the infrequency of vesical leiomyosarcoma in general. We also
investigated the potential molecular pathogenesis of the leiomyosarcoma.
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