Recurrent malignant Triton tumour: first report on a long time survivor.

Abstract

Malignant Triton tumour represents an extremely rare mesenchymal malignancy exhibiting histopathologic patterns of peripheral nerve sheath tumours and rhabdomyoblastic components, the latter usually determining the mostly fatal outcome. We report on a 26-year old patient diagnosed with malignant Triton tumour who developed multiple recurrences despite repeated aggressive surgery, chemo- and radiotherapy during an 8-year period. After Northern blotting analysis of an excised in-transit metastasis had revealed expression of retinoic receptors alpha and gamma, the patient received experimental treatment with isotretinoin and interferon-alpha for one year and remains without any evidence of disease for more than three years. This is the first report on a long-term survivor of multiple recurrences of malignant Triton tumour.