[Therapeutic difficulties in soft tissue sarcoma occurring in children with neurofibromatosis type 1 - own...

Neurofibromatosis type I (NF1) is one of the most common genetic disorders in man, predisposing to benign and malignant tumours. The most common malignancies comprise nervous system tumours, less frequently soft tissue sarcomas (STS) and leukaemia - myelodysplasia syndrome. Herein we report five cases of STS diagnosed in children affected with NF1 (3 girls and 2 boys, age: 8 months -17 years). Neurogenic tumours were diagnosed in three children (malignant peripheral nerve-sheath tumour in two and malignant triton tumour in one), while soft tissue sarcomas of rhabdomyosarcoma origin were found in two patients. In four cases the primary tumours were highly locally advanced, unresectable and located in pelvis minor. All patients received protocols for soft STS: CWS-91, 96 and 2002. The paper presents the clinical symptomatology, the course and outcome in children with NF1 affected with STS.

basing on our own observations STS in NF1 seems to have poor prognosis in spite of combined therapy. Since early diagnosis is essential, children with NF1 should remain under the care of the oncologist.