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Ann Thorac Surg 1995;59:1390
© 1995 The Society of Thoracic Surgeons
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Introduction |
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 Top Introduction |
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DR BRADLEY M. RODGERS (Charlottesville, VA): I congratulate Dr Temeck and her co-authors on a very nice paper and a very nice presentation. I have had an opportunity to review the manuscript of this paper; obviously there is a tremendous amount of data in the manuscript that Dr Temeck could not report today, and I recommend the manuscript to all of you interested in this particular topic.
This has been a topic that has been the source of literally dozens of articles over the past 20 years, really since the early 1970s, and has generated a lot of interest and a lot of confusing data. I think there are several aspects of the current study that are somewhat unique and perhaps add to its significance.
First of all, it is a relatively large number of patients, 152, who have been followed up very carefully, and the data have been analyzed in a very careful statistical manner. All of these patients have had close and long-term follow-up, and, as you saw, the median potential follow-up was 10.6 years, which is quite long for most of the series that have been reported. The addition of multiple histologies in this report adds to its usefulness. Parenthetically, I object somewhat to the use of ``pediatric'' in association with these histologies, because most of these tumors span a very broad age range. The most common tumor seen in this series is osteogenic sarcoma, and the mean age of presentation of osteogenic sarcoma is 19 years, which my adult colleagues would say is well out of the pediatric surgical range.
I think most of us would find no argument with Dr Temeck and associates' conclusions and would agree with most of the data reported; certainly these data reflect the data that my colleagues and I reported in pediatric patients, those less than 19 years of age, several years ago in a smaller group of patients.
I would like to concentrate my comments on the rhabdomyosarcoma group, which came out as predicting a shorter survival and predicting a shorter pulmonary progression-free interval. As you recall, this is a very small group of patients (6 patients), and that is true of most series; the rhabdomyosarcomas comprise a relatively small group of the patients. Nonetheless, the median survival of the rhabdomyosarcoma patients was 0.4 years as compared with a median survival of 3.1 years for osteogenic sarcoma.
If you look at the series that have been reported, the results for rhabdomyosarcoma have varied and most series do not show this negative predictive value; certainly our own series, with a very small number of patients, did not show a negative predictive value. At first blush this would seem paradoxic, because of all of the tumors that we are dealing with in this series and most, rhabdomyosarcoma probably has the best adjunctive therapy available. Chemotherapy and radiation, at least for the primary lesion, is as successful or more successful in rhabdomyosarcoma patients as it is in any of these other tumors. All of these patients received preoperative adjunctive chemotherapy, radiation therapy, or both for their primary tumors, and maybe we are seeing a selection process here where we are selecting out tumors that are unresponsive to chemotherapy by looking only at those that metastasize after chemotherapy for the primary malignancy. If this is the case, then we may begin to see more failures of our surgical resection of metastases in the future as we develop better chemotherapy and better adjunctive measures for all of these types of tumors.
I have several questions I would like to address to Dr Temeck. First of all, could we be seeing a selection process in the patients that are referred to the National Cancer Institute for therapy? Is it possible that these patients are those with disease that the primary physicians have deemed more aggressive or less responsive to initial attempts at chemotherapy? As you review their charts and review the outside course of these patients, is there any evidence that this is a group of patients with more virulent disease than most of us deal with in a more primary care setting?
Also, with a large number of patients like this and a large number of histologies, did you notice any change in the histologic grade of these tumors as more and more metastasectomies were performed as compared with the primary tumor? Likewise, because you are at the National Cancer Institute, have you done any molecular genetic studies on these tumors that might help differentiate responders from nonresponders or survivors from nonsurvivors?
Along the lines of my comments on rhabdomyosarcoma and its therapy, we have seen significant changes in our chemotherapy for some of these tumors; if you break down the data between the first and second decades of this study, do you see any change in the results of your data?
I think that everybody would agree that the ability to resect the metastatic disease completely is critical for survival in these patients. Is there anything from the computed tomographic scans in these patients that would help us to predict resectability?
I appreciate the opportunity to discuss this very nice paper.
DR PETER C. PAIROLERO (Rochester, MN): I too appreciated the opportunity of hearing this paper and agree with what has been said. I would like to ask about the implications of the left side; why would the left side have a worse prognosis? Also, I did not hear you say that we should not be doing resections in those patients who have rhabdomyosarcoma. Finally, I too disagree with the title of your presentation, this being pediatric malignancies. Certainly, these tumors span a greater age than just pediatrics. Half of your patients were between 19 and 33 years of age.
DR WILLARD A. FRY (Evanston, IL): In your presentation you stress the adverse prognostic predictor of incomplete resection. Would you contemplate a different type of incision, particularly when you have left-sided lesions? Specifically, have you considered using a clamshell incision? Our transplantation colleagues suggest that sometimes the exposure is better with that than with a median sternotomy.
DR TEMECK: I thank the discussants for their questions. There were several protocols that spanned the period of the study, and patients were eligible for referral to our institution as soon as the primary diagnosis was made. Fifty-eight percent of the patients presented for initial treatment at the National Institutes of Health. The remaining patients had metastatic disease upon referral, previous treatment, or both of these factors, putting them at ``high risk.''
Selection criteria for the study focused on the histologies as being seen more often in the pediatric age range group. All of these histologies were high grade. Molecular genetic studies were not included in this investigation. The computed tomographic scans were evaluated as to the number of nodules detected and, as stated in the report, no correlation between nodule size/number and survival was detected. We do not have an explanation why left-sided disease appeared to indicate a poorer prognosis. We have found that the median sternotomy approach gives excellent exposure of both sides even up to and including a fourth reoperation. In addition, the thoracotomy approach can be used in certain patients, usually after a number of median sternotomies. We have not used the clamshell approach, at least in this series, although it certainly can be used to treat these patients.
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