A high-grade primary leiomyosarcoma of the bladder in a survivor of retinoblastoma.

Abstract:

: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.

journal_name

Arch Pathol Lab Med

authors

Liang SX,Lakshmanan Y,Woda BA,Jiang Z

doi

10.1043/0003-9985(2001)125<1231:AHGPLO>2.0.CO;2

subject

Has Abstract

pub_date

2001-09-01 00:00:00

pages

1231-4

issue

9

eissn

0003-9985

issn

1543-2165

journal_volume

125

pub_type

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