Prostatic embryonal rhabdomyosarcoma in adults. A clinicopathologic review
β Scribed by Paul M. Waring; Ron C. Newland
- Publisher
- John Wiley and Sons
- Year
- 1992
- Tongue
- English
- Weight
- 1021 KB
- Volume
- 69
- Category
- Article
- ISSN
- 0008-543X
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β¦ Synopsis
Embryonal rhabdomyosarcoma of the prostate is a rare, highly malignant tumor that occurs predominantly in male infants and children, in whom it is the most common prostatic sarcoma. Six cases occurring in adults have been published, and the authors report three additional cases. The natural history is characterized by rapid growth, with the typical formation of large pelvic or abdominal masses, often leading renal failure due to bilateral ureteric obstruction. The tumor eventually disseminates widely, mainly to the lungs, bone, liver, and serosal surfaces, and unlike most other sarcomas, regional lymph node metastases are common. Combined modality therapy has resulted in marked improvement in survival rates and reduced surgical morbidity for children with these tumors. However, in adults the prognosis remains poor, with all patients dying of disseminated disease within 16 months of histologic diagnosis (mean survival, 8 months). Cancer 1992; 69:755-762.
Embryonal rhabdomyosarcoma at all sites occurs predominantly in children, but sporadic cases have been reported in adults as old as 80 years of age."2 It is the most common prostatic s a r ~o m a , ~ with a median age of occurrence of 5 years.4 We recently have seen three cases occurring in adults, and a thorough search of the literature revealed only six previously reported case^.^"-^ These nine cases form the first review of these
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We report on a newborn girl with Dubowitz syndrome (DS) and embryonal rhabdomyosarcoma (ERMS), with multiple chromosomal breakage (MCB). The tumor was resected but recurred in a few months, resulting in the infant's death. Malignancy and chromosomal breakage have been reported previously in DS. Howe
Using a subtractive hybridization method, we have cloned 48 cDNAs which are expressed in human primary myoblasts but down-regulated in the embryonal-rhabdomyosarcoma (RMS) cell line RD. Twenty-nine sequences could be identified as coding for previously known gene products, while 19 encode unknown pr