The role of surgery in the treatment of localized Ewing's sarcoma

This study includes 193 patients affected with localized Ewing's sarcoma and treated at the Rizzoli Orthopaedic Institute between April 1972 and May 1988. It is the purpose of this study to evaluate the efficacy of surgical treatment with regard to the prognosis of patients affected with E.S. and to define its indications as well as its limits as compared with radiotherapy. Ninety patients were treated surgically: 13 underwent amputation (localization of the disease in the leg and foot), 4 underwent debulking, 48 had resection of the tumor without any reconstruction (expendable bones), while for 25 other patients resection of the tumor required alloplastic or allograft replacement. In cases of E.S. localized in the pelvis and spine, surgery did not obtain improvement in prognosis. In the other sites, the percentage of disease-free patients increased from 57% to 72% in cases where surgery +/- radiotherapy was used instead of radiotherapy alone, while the percentage of local recurrences decreased from 18% to 5.5%, respectively. As for functional results, the highest incidence of excellent to good scores was obtained with surgery associated with radiotherapy (69%). The incidence of complications was only slightly different for the two groups.     

The role of resection in the treatment of pulmonary metastases from Ewing's sarcoma

We have investigated the role of resection in the treatment of patients with isolated pulmonary metastases from Ewing's sarcoma. In a retrospective review, 19 patients with the diagnosis of Ewing's sarcoma treated at the National Cancer Institute from 1965 to 1985 who underwent chest exploration for resection of pulmonary nodules were evaluated. Ten patients (53%) were made disease free by resection of pulmonary metastases, six patients (32%) were found to have unresectable disease, and three patients (16%) were found to have benign pulmonary disease. The actuarial 5 year survival rate of the 10 patients successfully made disease free by resection was 15%, and the median survival of this group was 28 months. In contrast, the median survival of the six patients not made disease free was 12 months, and no patient with residual disease was alive 22 months after thoracotomy (p2 = 0.0047). There were no postoperative deaths and only three minor postoperative complications for 25 operative procedures. Various prognostic variables were analyzed to determine their influence on postmetastasectomy survival. There was no difference in postmetastasectomy survival between patients who underwent resection of synchronous versus metachronous pulmonary metastases (p2 = 0.90). Patients who underwent resection of fewer than four malignant nodules had a significantly longer postmetastasectomy disease-free survival (p2 = 0.0019) and overall survival (p2 = 0.06) than those undergoing resection of four or more nodules. Patients who underwent resection of metastases that developed during chemotherapy had a significantly shorter postmetastasectomy survival that those who underwent resection of metastases that developed after chemotherapy (p2 = 0.0295). Our data show that selected patients with Ewing's sarcoma metastatic to the lungs may benefit from an aggressive surgical approach. Also, a significant proportion of these patients will have benign pulmonary disease and can thus avoid additional intensive systemic therapy.     

Radiation treatment of Ewing's sarcoma and osteogenic sarcoma.

At the Princess Margaret Hospital, Toronto, during the period 1960 to 1975, radiation therapy was given to 59 patients with Ewing's sarcoma without overt metastases. Over this period treatment was modified to include, as well as irradiation to the primary tumour, total body irradiation (TBI) and chemotherapy according to various regiments. The 5-year survival rate of 38% in 26% patients who received TBI suggests that this may be of value. In patients with osteogenic sarcoma preoperative irradiation was not found to be of value. Recently the resection of pulmonary metastases combined with chemotherapy has increased the survival rate. When the primary site makes resection impracticable, the response to irradiation and chemotherapy is encouraging. Three cases so treated are described.     

Acetabular osteoarticular allograft after Ewing's sarcoma resection

Ewing's sarcoma of the pelvis traditionally has been a difficult problem in orthopaedic oncology. Multiple studies have had inferior outcomes when compared with outcomes of patients with similar stage disease involving only the extremities. A major reason for this discrepancy is the difficulty associated with resection and reconstruction of pelvic tumors. Often the tumors are deemed nonresectable and are treated with chemotherapy and radiation or radiation alone. Patients treated in this manner have lower 5-year survival rates, higher local recurrence rates, and multiple long-term complications. The case of a 4-year-old girl with Ewing's sarcoma of the left hemi-pelvis is reported. Combined with adjuvant chemotherapy, this patient was treated with resection followed by osteoarticular allograft reconstruction. At 2 years followup, the patient was disease-free with an excellent functional outcome.     

Ewing's sarcoma. Prognostic factors, disease control, and the reemerging role of surgical treatment

Advances in the treatment of Ewing's sarcoma have been dramatic. Present treatment protocols control local disease by radiotherapy, surgery, or both; systemic spread is limited by aggressive multiagent chemotherapy. In patients with localized osseous Ewing's sarcoma, five-year survival rates now range from 54% to an estimated 74%. With late relapse not uncommon, control of the primary lesion is critical to long-term survival. Several studies now show improved local control and possibly improved survival of patients with surgical treatment of primary osseous Ewing's sarcoma.     

Function after partial pelvic resection for Ewing's sarcoma

Seven patients with Ewing's sarcoma of the pelvis were treated by chemotherapy followed by wide resection of the primary tumour. Although good function after operation is possible, survival in this series reflects the poor prognosis associated with the disease; two patients died, two are alive with local recurrence and metastases and three patients are alive with no evidence of disease.     

Treatment of Ewing's sarcoma in the cooperative Ewing's sarcoma study group

Multidisciplinary treatment approaches have greatly improved the prognosis of patients with Ewing's sarcoma. Four- or five-drug combination chemotherapy regimens are combined with radiation and/or surgery for local control. Currently, in the Cooperative Ewing's Sancoma Study (CESS) trials, approximately 60% of the protocol patients with Ewing's sarcoma show a relapse-free 10-year survival. For improvement of local control, preoperative radiotherapy and/or brachytherapy may be helpful. The early detection of blood and/or bone marrow contamination by molecular diagnosis can lead to a new clinical staging system. A myeloablative regimen consisting of simultaneous total-body irradiation and chemotherapy may improve the prognosis of patients with disseminated disease.     

Conservative surgery in breast cancer. Significance of resection margins

We approached the issue of surgical margins in the conservative treatment of breast cancer by examining the literature germane to four precise questions: At what distance from the macroscopic margin of the tumour should the resection margin be? To what extent do histologically clear resection margins indicate complete local control of the disease? To what extent do histologically involved margins indicate persistence of disease? and Does the local recurrence rate correlate with the status of the resection margin? We propose categorizing margin involvement into five groups (absent, focal, minimal, moderate and extensive involvement) according to strict histological criteria, and assigning increasingly aggressive subsequent treatments according to the extent of any margin involvement.     

Ewing's sarcoma of the hand: localization and treatment

Ewing's sarcoma of the hand is rare. Only 20 cases of Ewing's sarcoma of the hand have been reported in the literature. Although Ewing's sarcoma of the hand is rare and highly malignant, modern combinations of treatment are possibly curative due to the tumors surgical accessibility and apparent restricted involvement of only tubular bone. This case study illustrates currently accepted combination therapy consisting of chemotherapy, radiation therapy, and surgical tumor reduction. In spite of the advances of adjuvant therapy, surgical removal of the tumor still remains the most effective treatment.     

Combined modality treatment of Ewing's sarcoma of the maxilla

BACKGROUND: Primary bone sarcomas arising in osseous structures of the head and neck are rare. These tumors are often incompletely resected and treated with radiotherapy for local control. METHODS: We report a case of a 9-year-old girl with a maxillary Ewing's sarcoma. This patient was successfully treated with neoadjuvant chemotherapy followed by surgical resection and prosthetic reconstruction of the primary site. The surgical approach that was used consisted of a subtotal maxillectomy by means of a facial degloving approach, sparing the orbital contents and the inferior orbital rim and orbital floor. RESULTS: This approach produced excellent cosmetic, functional, and oncologic outcome. The patient remains without evidence of disease recurrence more than 4 years after surgery. CONCLUSIONS: This case illustrates a novel surgical approach to the resection of a maxillary Ewing's sarcoma and highlights the need for a multidisciplinary team approach to the management of head and neck sarcomas in children.     

Emergency treatment for primary Ewing's sarcoma of the ribs

Two cases of primary Ewing's sarcoma of the ribs necessitating emergency treatment are reported. Treatment consisted of a combination of radiotherapy, chemotherapy, and surgical therapy. The operation was urgent and early in one patient and delayed in the other. Both remain disease free 12 and 14 months after operation, respectively.     

Ewing's sarcoma in the retroperitoneum

The patient was a female aged 18. Hematuria was discovered at an examination two years previously at which time the patient was complaining of right lumbar pain. The patient visited our hospital with a chief complaint of right abdominal tumor in June, 1988. A retroperitoneal tumor, 77 X 45 mm in size, was observed by CT and ultrasonic examination, and the right kidney was in hydronephrosis. An operation was performed in September, 1988, based on a diagnosis of retroperitoneal tumor and hydronephrosis. The tumor was seen to have involved the right ureter. Subsequently the tumor was surgically removed; right total nephroureterectomy and partial cystectomy were performed at that time. The removed tumor was 90 X 70 mm in size and 120 g in weight; it was histopathologically diagnosed as extraskeletal Ewing sarcoma. A systemic examination was carried out postoperatively, but no abnormalities were observed. As of September, 1990, the patient had had no recurrence and was being kept observation on an outpatient basis. There have been a total of 210 case reports regarding extraskeletal Ewing sarcoma published worldwide, including our case and 26 cases in Japan, so far as we know. However those of a lesion of retroperitoneal origin are extremely rare, numbering only 11 cases worldwide and 2 Japan. Herein we report our case of extraskeletal Ewing sarcoma with reference to the other 209 cases reported in the literature to date.     

Video-assisted thoracic surgery for Ewing's sarcoma of the mediastinum in a 3-year-old girl.

We report of the experience of video-assisted thoracic surgery (VATS) for a Ewing's sarcoma at the posterior mediastinum in a 3 year old girl. The patient developed common cold like symptoms and developed rapid lower limb paraplegia. A chest computed tomography (CT) showed a solid mass adjacent to the vertebrae. This was diagnosed as a Ewing's sarcoma histopathologically by CT-guided needle biopsy (CT-NB). The mass was pressing on a nerve root in the vicinity of a vertebral foramen. As the tumor reduced after induction chemotherapy, with improvement of paraplegia, the patient underwent thoracoscopic examination followed by complete removal of the tumor by VATS. After surgery, the patient underwent postoperative chemo-radiotherapy. Five years has passed after operation without tumor recurrence. VATS could be an option as combined therapy for Ewing's sarcoma when well controlled and localized under induction therapy.     

Chest wall resection for Ewing's sarcoma of the rib: an unnecessary procedure

Approximately 10% of all cases of Ewing's sarcoma arise from a rib. Conventional management has included chest wall resection (3 or more ribs) and radiation therapy. These forms of therapy have led to complications such as scoliosis and local deformity. The addition of radiation therapy can result in damage to the lung and adjacent viscera and also potentiate pulmonary restrictive disease. Between 1971 and 1978, 9 patients were treated with surgery, radiation therapy, and combination chemotherapy (three- or four-drug regimen). Only 2 patients (22%) survive. Since 1979, 14 patients were entered into a new protocol consisting of sequential induction chemotherapy, followed by delayed surgical resection whenever feasible. Three patients had complete resection of their primary lesion at onset. Initially, 7 patients had either biopsy (N = 4) or incomplete chest wall resection N = 3). All 4 patients with biopsy only at diagnosis had excellent responses to induction chemotherapy, allowing delayed resection of the involved rib without chest wall resection. Overall, 12 of 14 patients (86%) treated since 1979 survive, with only 2 receiving radiation therapy for residual disease in the primary rib site.     

Primary Ewing's sarcoma of the temporal bone.

Primary cranial Ewing's sarcoma is exceptionally rare. Only ten cases of such a tumor had been reported heretofore in the literature. We describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent radiation and chemotherapy. Neither recurrence nor distant metastasis was noted at 12 months after surgery. Although the prognosis of Ewing's sarcoma in general is often poor because of early metastasis to the lungs and/or to other bones, a review of the literature suggested that the same tumor occurring in the cranium can often be successfully managed by intensive therapy with radical excision and radiochemotherapy. This inference was supported by the case reported here.     

Primitive neuroectodermal tumor. Ewing's sarcoma

Primitive neuroectodermal tumor is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors. It is important to recognize each of these entities, because each carries unique therapeutic and prognostic implications. However, accurate diagnosis of these tumors is hindered by their significant morphologic overlap and complicated by their rarity. These neplasm are highly aggressive that tend to recurence and to metastatize. Standard therapy combining surgery, chemotherapy, radiation and genetic therapy. We report a case of primitive neuroectodermal tumor of the kidney in a 50 year old female patient.     

Ewing's sarcoma simulating vertebra plana.

Five cases of vertebra plana have been reported, three with spontaneous regeneration and two caused by Ewing's sarcoma. It is emphasized that the diagnosis eosinophilic granuloma must rely on surgical biopsy.