Very late local recurrence of Ewing's sarcoma – can you ever say 'cured'? A report of two cases and literature review

Abstract

Current studies show that, following achieving remission in patients with non-metastatic Ewing's sarcoma, 30-40% of these patients will still develop recurrent disease (local and/or metastases) at some after treatment of the primary tumour. In both cases, disease remission had been achieved. Both patients originally underwent non-surgical combined modality therapy following initial diagnosis and both were rendered disease-free. After a long latent phase, both started experiencing unexplained local symptoms. These were investigated at their local hospital but tumour recurrence was not initially considered as a cause of these symptoms. The two patients were eventually referred back to our institution after the diagnosis of recurrent Ewing's sarcoma was established. Both have successfully undergone complete excision of the recurrences and are receiving postoperative adjuvant therapy. These cases highlight the need to remain vigilant for local recurrence, however long after the initial diagnosis.

Imaging Pediatric Bone Sarcomas

“…However, rare cases of delayed recurrence at 16 and 19 years after diagnosis have been reported 48 . More than half of these patients will have pulmonary or skeletal disease 24 .…”

Ewing sarcoma with very late metastasis in the skull: a case report

“…We encountered a case of ES, that recurred in the cranium, 21 years after the onset of the initial disease. Four cases with the development of local recurrence or metastasis more than 10 years after treatment had previously been reported [5][6][7]. However, there have been no reports of cranial metastasis, occurring 20 years after the initial treatment (Additional file 1: Table S1).…”

“…However, late complications and second malignant neoplasms (SMNs) remain a problem, and these require a long follow-up period after treatment [1,2]. Although recurrence typically occurs within 5 years of treatment [3,4], there have been several reports on local recurrence or metastasis after 15 years or more [5][6][7]. However, the very late metastasis of ES in the skull has not been reported.…”

Late recurrence of a tumor of Ewing’s sarcoma family of tumors: report of a case

“…The morphologic, immunohistochemical, and molecular biological characteristics from both specimens supported that the second tumor was a local recurrence of an ESFT. Several prior studies established that the longest time until recurrence of ESFT was 19 years [ 9 ]. Our case demonstrates the possibility of very late local recurrence of ESFT.…”