Syndrome-associated soft tissue tumours

Abstract

Soft tissue neoplasms may be associated with a variety of genetic disorders and malformation syndromes, especially when they arise in children, adolescents and early adulthood. This review summarizes the principal histopathological types of soft tissue tumours which occur in various syndromes, with an emphasis on pathological features, genetic aspects and considerations for the diagnostic pathologist.

Keywords: Gardner fibroma; Li-Fraumeni syndrome; desmoid-type fibromatosis; malignant peripheral nerve sheath tumour; malignant rhabdoid tumour; neurofibroma; neurofibromatosis type I; rhabdomyosarcoma; schwannoma.