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J Thorac Cardiovasc Surg 2000;119:1154-1161
© 2000 The American Association for Thoracic Surgery


GENERAL THORACIC SURGERY

EWING SARCOMA OF THE RIB: RESULTS OF AN INTERGROUP STUDY WITH ANALYSIS OF OUTCOME BY TIMING OF RESECTION

Robert C. Shamberger, MD, FACS, Michael P. LaQuaglia, MD, FACS, Mark D. Krailo, PhD, James S. Miser, MD, Douglas J. Pritchard, MD, Mark C. Gebhardt, MD, FACS, John H. Healey, MD, Nancy J. Tarbell, MD, Christopher J.H. Fryer, MD, Paul A. Meyers, MD, Holcombe E. Grier, MD, Pediatric Oncology Group Children’s Cancer GroupAddress for correspondence: Robert C. Shamberger, MD, FACS, Department of Surgery, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115 (E-mail: Shamberger@a1.tch.harvard.edu).

From the Departments of Surgery (R.C.S.) and Orthopedic Surgery (M.C.G.) and the Division of Pediatric Hematology/Oncology (H.E.G.) at Children’s Hospital, Boston, Mass; the Dana Farber Cancer Institute (H.E.G.) and Harvard Medical School; the Department of Surgery (M.P.L.), Orthopedic Surgery (J.H.H.), and Pediatrics (P.A.M.) at Memorial Sloan Kettering Cancer Institute, New York, NY; University of Southern California School of Medicine, Los Angeles, Calif (M.D.K.); the Division of Pediatrics, City of Hope, Duarte, Calif (J.S.M.); the Division of Orthopedic Oncology, Mayo Clinic, Rochester, Minn (D.J.P.); the Department of Radiation Oncology, Massachusetts General Hospital and Harvard Medical School (N.J.T.), Boston, Mass; and the Department of Pediatrics, King Khalid National Guard Hospital, Jeddah, Kingdom of Saudi Arabia (C.J.F.).

This work was supported in part by the following grants from the National Institutes of Health: CA-02649, CA-02971, CA-03161, CA-03526, CA-03750, CA-03888, CA-05436, CA-05587, CA-07306, CA-07431, CA-10198, CA-10382, CA-11233, CA-11796, CA-13539, CA-13809, CA-14560, CA-15525, CA-17829, CA-20320, CA-20549, CA-25408, CA-26044, CA-26126, CA-26270, CA-27678, CA-28383, CA-28439, CA-28476, CA-28851, CA-28882, CA-29013, CA-29139, CA-29293, CA-29314, CA-29691, CA-30969, CA-32053, CA-33587, CA-33603, CA-33625, CA-36015, CA-41573, CA-42764, CA-53128, CA-69177, CA-69428.

Address for reprints: Robert C. Shamberger, MD, FACS (#8850), c/o: Pediatric Oncology Group, 645 North Michigan Ave, Ste 910, Chicago, IL 60611.

Objective: We sought to establish the outcome and optimal therapeutic sequence for patients with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the chest wall.
Methods: Patients 30 years of age or younger with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the bone were randomly assigned to receive vincristine, doxorubicin, cyclophosphamide, and dactinomycin or those drugs alternating with ifosfamide and etoposide. Local control was obtained with an operation, radiotherapy, or both.
Results: Fifty-three (13.4%) of 393 patients had primary tumors of the chest wall (all rib). Event-free survival at 5 years was 57% for the chest wall compared with 61% for other sites (P > .2). Ifosfamide and etoposide improved outcome in the overall group (5-year event-free survival, 68% vs 54%; P = .002), and a similar trend occurred in chest wall lesions (5-year event-free survival, 64% vs 51%). Patients with chest wall lesions had more attempts at initial surgical resection (30%) than those with other primary tumor sites (8%, P < .01). The attempt at initial resection for chest wall lesions did not correlate with size. Initial resections at other sites were restricted to smaller tumors. Initial resection resulted in negative pathologic margins in 6 of 16 patients, whereas the delayed resection resulted in negative margins in 17 of 24 patients (P = .05). Although there was no difference in survival by timing of the operation in rib lesions, a higher percentage of patients with initial surgical resection received radiation than those with resection after initial chemotherapy (P = .13).
Conclusions: Although rib primary tumors are significantly larger than tumors found in other sites, their outcome is similar. We favor delayed resection whenever possible to minimize the number of patients requiring radiation therapy.




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