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Ann Thorac Surg 1995;60:1454
© 1995 The Society of Thoracic Surgeons
As Originally Published in 1988:
Updated in 1995 by Bhaskar N. Rao, MD, Neyssa Marina, MD, William H. Meyer, MD, Elizabeth I. Thompson, MD, F. Ann Hayes, MD, James W. Pate, MD, and Carol A. Greenwald, MD
Departments of Surgery, Hematology-Oncology, and Radiation Oncology, St. Jude Children's Research Hospital, and The University of Tennessee, Memphis, Tennessee
We previously reported the excellent outcome for children with Ewing's sarcoma arising in the rib, indicating that chest wall resection was unnecessary [1]. With 7 years of additional follow-up, the survival of this cohort of patients is maintained with acceptable morbidity.
Results
Group 1
Of the 9 patients treated from 1971 through 1978 (group 1), the same 2 patients survive (16+ and 22+ years). Both exhibit loss of lung volume, chronic atelectasis, and mild scoliosis.
Group 2
Fourteen patients were originally described; subsequently, 1 additional patient was treated on this protocol. The demographic and outcome data are shown in Table 1
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The remaining 5 patients underwent initial biopsy and induction chemotherapy. At delayed operation, only the involved rib or a segment of another was resected. In 2 patients with microscopic tumor present in the resected specimen, postoperative local radiotherapy was given. These 5 patients survive disease free with minimal long-term complications.
Comment
Ewing's sarcoma is the second most common bone tumor in children and adolescents [2]. Approximately 10% of these tumors arise in the rib [3]. Significant progress in understanding the biology of these tumors has contributed to our present therapeutic approach. About 50% to 60% of patients presenting without metastases will be cured [2, 4, 5].
A large proportion of patients, particularly with pelvic or rib tumors, have bulky soft tissue masses at diagnosis [3, 4]. Chemotherapy has been shown predictably to cause tumor regression and, therefore, contributes to improving local control [2, 4]. Radiotherapy alone can effectively obtain local control in 60% to 90% of selected cases [2, 4, 5]. This approach to rib primary tumors has major disadvantages. Long-term morbidity includes restrictive pulmonary disease, hypoplasia of the chest, scoliosis, and other visceral injury. In 1 of our patients death was attributed to multimodal therapy. All 6 surviving patients treated with supplemental radiotherapy have demonstrated these sequelae. In contrast, only 3 of 6 patients not receiving local radiotherapy have documented minimal morbidity.
Since 1987 we have used more aggressive chemotherapeutic regimens to treat Ewing's sarcoma and its biologically related tumors (eg, peripheral neuroepithelioma, extraosseous Ewing's tumor, Askin tumor) based on the experience from the above group of patients [5]. Patients with chest wall or rib tumors first undergo a generous open biopsy. Sufficient tissue is obtained for routine histologic diagnosis, immunohistochemistry, and molecular pathology. All patients then receive multiagent aggressive chemotherapy. Tumors that can be completely resected with low morbidity undergo complete surgical resection. If tumor margins are negative, no radiotherapy is delivered. For incomplete tumor resection with good prior response to chemotherapy, local radiotherapy at moderate doses is delivered. In those rare instances where responses are poor, chest wall resection may be indicated. Of 10 patients with rib or chest wall primary tumors treated from 1987 through 1992 with delayed operation, 7 survive with a follow-up ranging from 4 to 6 years.
Acknowledgments
Supported in part by Cancer Center support CORE grant P30CA21765 from the National Cancer Institute, Bethesda, MD, and by the American Lebanese Syrian Associated Charities, Memphis, Tennessee.
Footnotes
Address reprint requests to Dr Rao, St. Jude Children's Research Hospital, 332 N Lauderdale, Memphis, TN 38105-2794.
References
This article has been cited by other articles:
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  S. K. Chowdhury, K. S. V. K. Subbarao, M. Nachiappan, K. Agrawal, S. K. Chowdhury, K. S. V. K. Subbarao, M. Nachiappan, and K. Agrawal Primary Neoplasm of the Chest Wall: Surgical Management Asian Cardiovasc Thorac Ann, September 1, 2000; 8(3): 249 - 252. [Abstract] [Full Text] [PDF]
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 R. C. Shamberger, M. P. LaQuaglia, M. D. Krailo, J. S. Miser, D. J. Pritchard, M. C. Gebhardt, J. H. Healey, N. J. Tarbell, C. J.H. Fryer, P. A. Meyers, et al. EWING SARCOMA OF THE RIB: RESULTS OF AN INTERGROUP STUDY WITH ANALYSIS OF OUTCOME BY TIMING OF RESECTION J. Thorac. Cardiovasc. Surg., June 1, 2000; 119(6): 1154 - 1161. [Abstract] [Full Text] [PDF]
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 R. J. Coombs, E. A. Bayar, Y. H. Matloub, and M. E. Velasco Pediatric Case of the Day RadioGraphics, January 1, 1999; 19(1): 241 - 244. [Full Text] [PDF]
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