Rhabdomyosarcoma
- The most common soft-tissue sarcoma (50%)
- The most common primary orbital malignancy in childhood. this tumor should
be a consideration in any child between the ages of 7 and 8 who has a
rapidly progressing unilateral proptosis.
- Other sarcomas:
- Liposarcoma
- Fibrosarcoma
- Angiosarcoma
- Neurofibrosarcoma
- Synovial sarcoma (most common NRSTS)
- Leiomyosarcoma
- Arises from striated skeletal muscle
- Accounts for 5-8% of all childhood cancers
- Most commonly present with mass (+/-pain)
- Increased risk in Neurofibromatosis
Age:
- Peak incidence: 1-5 yrs
- 10% occurs in 1st year of life
- 70% appear by first decade
Anatomic distribution:
- Head & Neck (40%)
- GU tract (20%)
- Trunk (10%)
- Retroperitoneal & other
Four histiotypes:
- Embryonal type: 60%, intermediate prognosis
- Alveolar type: 15%, trunk and extremities, poor prognosis
- Botryoid type: 6%, “bunch of grapes”, most often found in the vagina,
uterus, NP, and middle ear
- Pleomorphic type: 1%, adult form
About 20% of patients have undifferentiated sarcomas.
**Histiotype determines treatment plan and prognosis.**
Tumor |
Age of Presentation (years) |
Met sites |
Hepatoblastoma |
1 |
lymph nodes |
Neuroblastoma |
2 |
bone, BM, liver |
Wilm's Tumor |
3 |
lungs, liver |
Rhabdomyosarcoma |
1-5 |
LN, lungs, BM, Bones |
Abdominal masses
CHLA Board Review 2005