Clinical investigation: Sarcoma
Long-term results with radiation therapy for pediatric desmoid tumors

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Abstract

Purpose: To retrospectively review the treatment and outcome of pediatric patients with desmoid tumor who received radiation therapy at a single institution.

Materials and Methods: Thirteen pediatric patients received radiation therapy for desmoid tumor at St. Jude Children’s Research Hospital between 1962 and 1998. Only 2 of the patients reviewed received treatment prior to 1976. The median dose of external beam irradiation was 50 Gy.

Results: At the time of this report, 10 of 13 patients have had tumors that recurred after radiation therapy and 3 have died from their disease. One additional patient was harboring a recurrence, and 1 had not been followed long enough to suggest that the patient had achieved disease control. One patient remained locally controlled after radiation therapy with long-term follow-up (196 months). The median time to recurrence following radiation therapy was 19 months (range, 3–135 months). Eight of the 13 patients suffered substantial tumor and treatment-related morbidity.

Conclusions: Desmoid tumors in pediatric patients are locally aggressive tumors that are likely to recur after radiation therapy. Alternatives to radiation therapy should be sought for the treatment of these tumors, and efforts should focus on low-morbidity therapies aimed at inhibiting the growth of these unique tumors.

Introduction

Radiation therapy as an adjunct to surgery provides long-term control and preservation of function for desmoid tumors in adults 1, 2, 3, 4, 5. For children, there is limited data to suggest that radiation therapy is useful, and some consider that children have control rates with radiation therapy that are inferior to those observed for adults 2, 6, 7, 8, 9. Most children are treated with radiation therapy using guidelines outlined for adults. Under these circumstances, radiation therapy is used as an adjuvant modality when recurrence is inevitable or when recurrence and/or second surgery are likely to result in substantial morbidity. Radiation therapy is used more routinely at the time of recurrence, when the recurrent tumor is not completely resectable, and when the patient has achieved the age of skeletal maturity.

Desmoid tumors require doses in excess of 50 Gy to achieve local control 1, 2, 3, 5, 10, 11, 12. Doses in this range are likely to cause substantial morbidity and serve as the basis for investigators to search for alternatives to radiotherapy in the adjuvant treatment of these patients 13, 14, 15. Because the natural history of these tumors appears to be under the influence of endogenous hormones 8, 16 there is a potential for host factors, unique to pediatric patients, to promote the growth of these locally aggressive tumors. In this paper, we review our institutional experience with these tumors and have confined our observations to those patients who received radiation therapy in the course of their management. The objective was to further define the role of radiation therapy in the treatment of the pediatric patient.

Section snippets

Materials and methods

Thirteen patients were identified who received radiation therapy for desmoid tumor at St. Jude Children’s Research Hospital between November 1962 and March 1998. The median age at diagnosis was 10 years (range, 2–17 years). There were 9 female and 4 male patients. Six of 13 (46%) were white and 7 of 13 (54%) were black including all of the male patients. We grouped the tumors according to their site of presentation: 6 involved paraspinal or chest-wall sites, 4 involved the extremities, 2

Results

Pertinent patient and treatment-related details are presented in Table 1. Only 5 of the patients included in this report received radiation therapy immediately following diagnosis; the remaining 8 were treated following local recurrence. The median dose to the primary site using external beam irradiation was 50 Gy (range, 32–60 Gy). One patient received a split course of external beam irradiation, and 1 patient was treated with brachytherapy (24 Gy) in addition to external beam irradiation (50

Discussion

The role of radiation therapy in the treatment of pediatric patients with desmoid tumors should be considered different from that of adults. In this series, only 1 patient appears to be locally controlled with long-term follow-up. Notwithstanding the large time period covered by this review, the failure rate with radiation therapy is high and leads us to speculate that these tumors are biologically different from their adult counterpart. It may be plausible that certain host factors influence

Acknowledgements

Supported in part by Cancer Center Support CORE Grant, P30 CA 21765 and the American Lebanese Syrian Associated Charities (ALSAC).

References (29)

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