Desmoplastic Small Cell Tumor
Clinical
- Age 3-54 years, mean 22-25
- 5:1 male:female
- Vast majority arise in peritoneal cavity
- Occasional cases in tunica vaginalis
- Rare cases in pleura and lung
- Very rare cases ethmoid sinus, scalp, kidney and pancreas
- Usually presents with studding of peritoneal surface
- Very poor prognosis
