[Ewing's sarcoma in children--current surgical treatment options, evaluation of our patients].

PURPOSE OF THE STUDY: Neoplastic diseases continue to arouse much concern in the general population. This also applies to bone tumors. Until recently, these neoplasms, most frequently occurring in children and adolescents, were considered to have the worst possible prognosis with a minimal opportunity for a successful outcome of treatment. This is a likely reason for the still deeply rooted belief that amputation of the affected limb is necessary and disease prognosis is uncertain, which also applies to Ewing's sarcoma. Only lately could these patients be offered a hope of successful treatment including limb salvage.

MATERIAL: In the period from 1984 to mid-2003, 78 patients with Ewing's sarcoma were treated in our department. Their age ranged from 3 to 25 years, with the maximum occurrence between 13 and 17 years. We performed 27 reconstructions, 13 amputations or exarticulations and 24 resections without replacements. In 13 patients, the procedure was restricted to diagnostic biopsy only, because the tumor was inoperable.

METHODS: The diagnosis was made on the basis of diagnostic biopsy and, subsequently, neoadjuvant chemotherapy was administered. When this resulted in tumor regression, definitive surgery was performed, involving tumor resection and bone replacement either with autograft or allograft, or with an individual prosthesis. When an implant was not necessary for maintaining limb function, a simple resection was carried out. In the patients in whom the tumor failed to regress, amputation or exarticulation was inevitable. Inoperable tumors were managed by megadose chemotherapy and by radiotherapy. The patients after orthopedic surgery receive adjuvant therapy, including bone marrow transplantation in indicated cases.

RESULTS: The evaluation of our 78 patients showed that patient survival is not related to the surgical procedure used. In patients with an early diagnosis and a positive response of the tumor to chemotherapy, the reconstruction procedure appeared to be sufficiently radical in terms of cancer control while preserving limb function; these patients showed neither a significant increase in disease recurrence nor metastatic dissemination. Similar outcomes were also recorded in patients with a simple resection. However, amputation and exarticulation cannot completely be avoided and they are necessary in the patients whose tumor failed to respond to chemotherapy or in whom radical removal of the tumor is not possible. The poorest outcome was recorded in the patients with inoperable tumors, in whom it was not possible to carry out a radical orthopedic operation. The time between the onset of pain and initiation of therapy is also an important factor affecting the treatment outcome. Complications of reconstructive surgery for Ewing's sarcoma recorded in our patient group included osteomyelitis and graft fracture in addition to relapse and metastatic dissemination followed by death.

DISCUSSION: Reconstructive surgery for Ewing's sarcoma is carried out in our department in patients with a confirmed diagnosis, in whom neoadjuvant therapy has resulted in tumor regression and in whom the extent and site of a tumor permit this sort of procedure. If this is not possible, limb amputation is indicated. If amputation is not possible due to tumor localization, megadose chemotherapy is administered. The results of long-term survival evaluation of our patients undergoing resection and replacement show that the procedure has been sufficiently radical, because no local recurrence or metastatic dissemination followed by death were recorded. Disease recurrence, death, graft osteomyelitis and fracture of a graft inclusive of osteosynthetic material are the complications reported.

CONCLUSIONS: This study evaluated the current techniques of treating Ewing's sarcoma, with emphasis on reconstructive surgery leading to limb salvation and maintenance of its full function. The importance of this approach is obvious when we realize that the patients are mostly children and adolescents.