Authors: Rick R Van Rijn Jim C H Wilde Johannes Bras Foppe Oldenburger Kieran M C McHugh Johannes H M Merks
Publish Date: 2008/03/07
Volume: 38, Issue: 6, Pages: 617-634
Abstract
Rhabdomyosarcoma RMS is the most common paediatric softtissue sarcoma and constitutes 3–5 of all malignancies in childhood 1 In children the agestandardized annual incidence rate for RMS is between 4 and 7 per million 2 This review focuses on the imaging of all RMS occurring outside the head and neck region which comprise 40 of RMS around 15 are genitourinary GU nonbladder prostate tumours GUNBP ie paratesticular vaginal and uterine tumours 10 are bladder prostate tumours BP 15 occur in the limbs and 20 occur in other sites ie thoracic or abdominal tumoursClinical symptoms vary widely based on the location of the primary tumour but in general presenting symptoms often are indolent with nonspecific or minimal symptoms at the start mimicking innocent general paediatric diseases Often the duration or progression of symptoms alerts the physician to the presence of a malignant tumour Sitespecific symptoms in GUNBP tumours are a paratesticular mass in boys that may be painful or not while girls may present with a grapelike botryoid vaginal extrusion of mucosanguineous tissue or micturition problems BP tumours may present with urinary retention haematuria constipation or an abdominal mass Limb tumours often present with a painless swelling and/or with enlarged regional lymph nodes while presentation of tumours in other locations may vary from biliary obstruction in tumours of the biliary tract to painless masses all depending on their primary site
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