Peripheral primitive neuroectodermal tumour of the hand in an adult

We present a case of a 28 year-old woman who presented with a peripheral primitive neuroectodermal tumour of the right hand involving the fifth metacarpal and little finger. She underwent excision biopsy, then ray amputation of her right little finger. Initial radiological staging did not reveal metastasis but, after recurrence locally at two years and in spite of adjuvant chemotherapy and radiotherapy, the patient died of progressive metastatic disease 4 years later. The current concepts and difficulties in diagnosing these tumours are discussed.