International Journal of Radiation Oncology*Biology*Physics
Clinical investigation: SarcomaLong-term results with radiation therapy for pediatric desmoid 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)
- et al.
Desmoid tumorsA 20-year radiotherapy experience
Int J Radiat Oncol Biol Phys
(1990) - et al.
Individualizing management of aggressive fibromatoses
Int J Radiat Oncol Biol Phys
(1998) - et al.
Radiotherapy and conservative surgery in the management of musculo-aponeurotic fibromatosis
Int J Radiat Oncol Biol Phys
(1988) - et al.
The desmoid syndromeNew aspects in the cause, pathogenesis and treatment of the desmoid tumor
Am J Surg
(1986) - et al.
Desmoid tumorsLocal control and patterns of relapse following radiation therapy
Int J Radiat Oncol Biol Phys
(1983) - et al.
Radiation therapy in the treatment of aggressive fibromatoses
Int J Radiat Oncol Biol Phys
(1981) - et al.
Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumors
Am J Surg
(1991) - et al.
The desmoid (Reitamo) syndromeEtiology, manifestations, pathogenesis, and treatment
Curr Probl Surg
(1988) - et al.
The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors
Int J Radiat Oncol Biol Phys
(1997) - et al.
The role of radiotherapy in the treatment of desmoid tumours
Radiother Oncol
(1986)
Radiation therapy in the management of desmoid tumors
Int J Radiat Oncol Biol Phys
MT1-MMP and MMP-2 mRNA expression in human ovarian tumorsPossible implications for the role of desmoplastic fibroblasts
Hum Pathol
Role of the matric metalloproteinase and tissue inhibitors of metalloproteinase familes in noninvasice and invasive tumors transplanted in mice with severe combined immunodeficiency
Urology
Aggressive infantile fibromatosis of the submandibular region
Intl J Oral Maxillofac Surg
Cited by (57)
Desmoid tumors of the head and neck in the pediatric population: Has anything changed?
2021, International Journal of Pediatric OtorhinolaryngologyRadiation Therapy for Aggressive Fibromatosis: The Association Between Local Control and Age
2018, International Journal of Radiation Oncology Biology PhysicsCitation Excerpt :Although suggested in a prior report from our institution focusing on children and young adults (12), this series is the first to present a direct comparison with adult patients. Both in our prior series and in a report from St Jude Children's Research Hospital (Memphis, TN), pediatric patients receiving RT for aggressive fibromatosis had poor outcomes (12, 13), which is not the case in the surgical literature (14, 15). An analysis of children treated with surgery for aggressive fibromatosis at St Jude Children's Research Hospital revealed significantly worse LC in children aged >12 years (HR, 5.08) (16).
Desmoid tumours of the head and neck in children: Review of management
2017, Annales Francaises d'Oto-Rhino-Laryngologie et de Pathologie Cervico-FacialeDesmoid tumours of the head and neck in children: Review of management
2017, European Annals of Otorhinolaryngology, Head and Neck DiseasesCitation Excerpt :However, it can be responsible for adverse effects (fibrosis, paraesthesia, oedema, fractures, skin ulcers) and radiation-induced second cancers that can be fatal [12,18]. External beam radiotherapy exposes children to a considerable risk of long-term cosmetic and functional sequelae (radiation-induced craniofacial bone growth inhibition, hypoplasia of the mandible and facial bones, panhypopituitarism, scoliosis, hypothyroidism, neuropathic ulcers, osteomyelitis, dermatitis with chronic ulcers that can sometimes be fatal) [5,6,8,11,17,19]. Radiotherapy is also associated with a risk of radiation-induced cancer in the irradiation fields (basal cell carcinoma, papillary thyroid carcinoma, osteosarcoma of the mandible) [5,11,18].
Sporadic desmoid tumors in the pediatric population: A single center experience and review of the literature
2017, Journal of Pediatric SurgeryAge-based treatment of aggressive fibromatosis in the head and neck region
2014, Journal of Oral and Maxillofacial SurgeryCitation Excerpt :Long-term cosmetic impairment, functional morbidity, growth disturbance, contracture, the risk of developing second malignancies, and poor recurrence control22 are of concern, making radiotherapy an unsuitable treatment measure for skeletally immature patients. Merchant et al,18 in a case series of 13 pediatric patients undergoing radiotherapy for desmoids tumor, showed that 10 of 13 patients had developed local recurrence, 3 patients had died of disease, 8 patients had severe radiation-associated complication, and only 1 patient remained with locally controlled disease. The recently published study by Rutenberg et al23 also showed that the locoregional control rate for patients younger than 18 years old who had undergone external beam radiotherapy was only 20%; however, in those aged 18 to 30 years, it was 63%.