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Detailed information of RHABDOMYOSARCOMA
RHABDOMYOSARCOMA
DEFINITION:
A striated muscle sarcoma characterized usually by a mass and
varied clinical manifestations.
EPIDEMIOLOGY:
- incidence: ?
- 3rd most common extracranial solid tumor in children
- makes up 4-8% of all childhood cancers (under age 15 years)
- age of onset:
- two incidence peaks: 2 - 4 years
- 12 - 16 years
- risk factors:
- familial - chrom.#: 11p15.5
- family history of breast or brain cancer
- neurofibromatosis
- associated features:
- soft tissue sarcomas, CNS neoplasms and adrenocortical
carcinomas in children and siblings of those with
rhabdomyosarcoma
- central nervous system (CNS) and genitourinary (GU)
malformations
PATHOGENESIS:
- tumor site and patient age related:
- bladder, prostate, vagina
- head and neck
2. Older Children and Young Adults
- paratesticular
- trunk and abdomen
3. Any Age
2. Histologic Types
- predominant variant in infants and small children
- found in 80% of distal urinary tract tumors, 60% of head
and neck tumors, 50% of tumors in all other sites except trunk
and perineum
- botryroid form is a subtype
2. Alveolar
- predominant variant in striated muscle masses of trunk and
limb
- associated with increased mortality
3. Pleomorphic
4. Undiffereniated
- arise from a primitive cell type that occurs in
mesenchymal tissue at any site
- primary tumor occurs at all sites except in brain
CLINICAL FEATURES:
- ptosis, exophthalmos, eyelid swelling
2. Nasopharyngeal and Middle Ear Tumors
- airway obstruction, chronic otitis media, discharge,
polypoid mass, spread to adjacent meninges
3. Neck Tumors
- mass, pain, cervical and brachial plexus palsy
2. Genitourinary Tract
- urinary obstruction, hematuria
2. Vagina and Uterine Tumors
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Pediatric Database - RHABDOMYOSARCOMA
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