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[Desmoid tumors in children: current strategy].
Caroline Oudot1, Anne-Sophie Defachelles, Véronique Minard-Colin
1CHU de Limoges, hôpital de la Mère et de l'Enfant, service d'oncologie pédiatrique, 8, avenue Dominique-Larrey, 87042 Limoges, France. oudot_caroline@yahoo.fr
Bulletin Du Cancer
|May 23, 2013
Summary
Desmoid tumors (DTs), or aggressive fibromatosis, are benign soft tissue neoplasms with a high recurrence rate. Management strategies for pediatric DTs prioritize observation or medical treatment over surgery due to unpredictable behavior and recurrence potential.
Area of Science:
- Oncology
- Pathology
Context:
- Desmoid tumors (DTs), also known as aggressive fibromatosis, are rare soft tissue neoplasms characterized by benign histology but aggressive local invasion and recurrence.
- DTs affect all age groups, including children, with an incidence of 2-4 cases per million annually.
- While the etiology is largely unknown, genetic predispositions like familial adenomatous polyposis and Gardner's syndrome are implicated in a small percentage of cases.
Purpose:
- To provide an accurate overview of desmoid tumors in children.
- To discuss the diagnostic criteria, clinical behavior, and current management strategies for pediatric desmoid tumors.
Summary:
- Desmoid tumors are histologically benign but locally invasive neoplasms with a significant potential for recurrence, making complete surgical resection challenging.
- Diagnosis requires biopsy to differentiate from sarcomas, and treatment decisions are guided by disease aggressiveness, often favoring a "wait-and-see" approach for asymptomatic cases.
- Medical therapies are considered when complete resection is not feasible without functional or cosmetic compromise, with radiation therapy being a less common option in pediatric cases.
Impact:
- This knowledge base aids clinicians in making informed decisions regarding the diagnosis and management of desmoid tumors in pediatric patients.
- Highlights the importance of considering conservative management strategies and non-surgical interventions for pediatric desmoid tumors.
- Contributes to a better understanding of the unpredictable clinical behavior and recurrence patterns of desmoid tumors in children.
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