Very late recurrence of Ewing sarcoma (ES): Report on three cases and review of literature.

Abstract

e20005 Background: ES is the second malignant bone tumour in children and young adults. Most relapses occur the first years following the diagnosis. We report 3 very late ES recurrence (11, 20, 24 y after primary tumour). 2 occurred a long time after a first relapse. Methods: P 1: In 1991 a 21 y old girl was treated for tibia non metastatic ES by neo and adjuvant chemotherapy, extra-tumoral surgery. Tumour necrosis (TN) was 100%. In 2002 after local trauma, she had a first local relapse. She remained 3 y in remission, after second line of treatment included complete surgery. She died in 2007, after a second local recurrence, in spite of contaminated surgery, chemotherapy and radiotherapy. P 2: In 1990 an11 y old boy was treated for superior femoral ES by neoadjuvant chemotherapy, surgery. TN was 93%. Parents refused adjuvant chemotherapy. In 1993, he presented a first inguinal local relapse treated by neo and adjuvant chemotherapy, partial surgery, local radiotherapy. TN was 5%. In June 2010, 17 years after the first relapse, he presented a sacral metastasis and one isolated pulmonary metastasis; he was treated by chemotherapy and sacral radiotherapy. He is still in treatment, with stable pulmonary and no evolutive sacral disease. P 3: In 1986, a ten y old girl was treated for L3 non metastatic ES by neoadjuvant and adjuvant chemotherapy, contaminated surgery. TN was 100%. She didn’t have radiotherapy. In January 2011, 7 and 9 years after 2 pregnancies, she had local relapse Diagnosis was delayed for 3 months after erroneous interpretation of symptoms. Results: In Rizzoli experience of ES outcome, patients who relapse after combined treatment is very poor. However, when the relapse is late, patients seem to be more receptive to traditional treatment and outcome better. Dubois reported in 2008, 2 ES late relapses during pregnancy. Conclusions: These cases pointed out the need to remain vigilant for ES, a long time after diagnosis. Late relapses must be envisaged as a cause of symptoms, to avoid diagnosis delay.