Anatomic distribution:

Four histiotypes:

  1. Embryonal type: 60%, intermediate prognosis
  2. Alveolar type: 15%, trunk and extremities, poor prognosis
  3. Botryoid type: 6%, “bunch of grapes”, most often found in the vagina, uterus, NP, and middle ear
  4. 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