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Soft tissue tumour

Aetiology

 Unknown in most cases

 Genetic associations:

 Neurofibromatosis type 1, type 2

 Retinoblastoma

Related to RB-1 gene mutation as well as the

RT given

 Li-Fraumeni syndrome

Mutation of p53 tumour suppressor gene

 Familial adenomatous polyposis (FAP)

Gardner’s syndrome: mutation of APC gene

on chromosome 5q21, associated with

epidermoid cysts, desmoid tumours,
osteomas and colonic polyps.

Gorlin’s syndrome: increase risk of

mediastinal sarcomas

 Genetic alterations due to unknown causes

are also common