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Pediatric Desmoid Tumor of the Head and Neck: A Systematic Review and Modified Framework for Management by Age Group
Bryan S Torres1, Hannah G Brown2, Julisa Nuñez3
1From the School of Medicine, Tulane University, New Orleans, La.
Insights
Pediatric head and neck desmoid tumors (DTs) often require intervention due to developmental interference. Achieving negative surgical margins is crucial to prevent recurrence, guiding future management strategies for these rare pediatric tumors.
Area of Science:
- Oncology
- Pediatric Surgery
- Tumor Biology
Background:
- Pediatric head and neck desmoid tumors (DTs) pose unique challenges due to their potential to disrupt normal anatomical development.
- Unlike in adults, early and aggressive management is essential for pediatric DTs.
- Defining optimal interventions and management strategies is critical for this population.
Purpose of the Study:
- To conduct a comprehensive systematic review of pediatric head and neck desmoid tumors (DTs).
- To assess lesion location, intervention, and management strategies.
- To examine age-related outcomes, including surgical margins, recurrence, and complications.
Main Methods:
- Systematic literature review from January 1990 to December 2023.
- Databases searched: PubMed, Scopus, and MEDLINE.
- Analysis of outcomes in pediatric patients aged 0-11 and 12-21 years.
Main Results:
- 121 patients from 44 studies were analyzed.
- Predominant locations included the mandible, cranium, and neck.
- Tumors occurred early (mean age 4.18 years) and were often resected with positive margins; age did not significantly impact recurrence or complications.
Conclusions:
- Recurrence of desmoid tumors (DTs) is associated with positive surgical margins.
- Surgical resection aiming for negative margins is recommended when feasible.
- Enhanced follow-up and data collection are needed to refine management practices for pediatric DTs.
Background:
Unlike in adults, pediatric head and neck desmoid tumors (DTs) have greater capacity to interfere with normal anatomical development. Therefore, adequate interventions and management must be defined. We aimed to provide the most comprehensive systematic review on pediatric head and neck DTs to date, including assessment of lesion location predominance, intervention, and management, and examination of any associations between age and outcomes like surgical margin status, recurrence, and complications.
Methods:
A systematic literature review was conducted between January 1990 and December 2023 using PubMed, Scopus, and MEDLINE databases following the Preferred Reporting Items for Systematic Review and Meta-Analyses 2020 guidelines. We aimed to elucidate intervention and management strategies by studying various outcomes in 0-11 and 12-21 year olds.
Results:
The literature search yielded 44 studies, totaling 121 patients. Most head and neck DTs localized to the mandible, cranium, and neck; occurred early (P = 4.18 years); and underwent local resection with positive margins. Older and younger patients shared no difference in complication or recurrence rates.
Conclusions:
We found recurrence is likely to occur with positive margins. Because standard treatment of DTs is surgical resection with negative margins, if technically feasible with reasonable associated morbidity, we suggest additional resection of the tumor to achieve negative margins. We also recommend more robust follow-up data collection, not only due to desmoid's high recurrence and data paucity in patients older than 12 years, but also, to better establish best management practices.
