Join us in Philadelphia from Sept. 25-27th for our "Together We Will" Weekend: - Register today!

The Desmoid Tumor Research Foundation

September 2024

Pediatric Desmoid Tumor of the Head and Neck: A Systematic Review and Modified Framework for Management by Age Group

Author Information

Bryan S Torres 1, Hannah G Brown 2, Julisa Nuñez 3, Chenue Abongwa 4, Fouad M Hajjar 4, Rajendra F Sawh-Martinez 5, Joseph Lopez 5 6

1From the School of Medicine, Tulane University, New Orleans, La. 2School of Medicine, University of Central Florida, Orlando, Fla. 3School of Medicine, Georgetown University, Washington, D.C. 4Division of Medical Oncology, AdventHealth for Children, Orlando, Fla. 5Division of Pediatric Plastic and Reconstructive Surgery, Department of Pediatric Surgery, AdventHealth for Children, Orlando, Fla. 6Division of Pediatric Head and Neck Surgery, Department of Pediatric Surgery, AdventHealth for Children, Orlando, Fla.

Publication

Plast Reconstr Surg Glob Open

Summary

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.

View Abstract