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

Abstract

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.

Fig. 1.

DT in the neck of a pediatric patient before resection. Intraoperative image of desmoid in the neck of a pediatric patient after lateral retraction of the sternocleidomastoid muscle and before resection. After total resection, the mass measured 6.2 × 6.0 × 4.0 cm.

Fig. 2.

Preferred reporting items for systematic reviews and meta-analyses flowchart detailing study inclusion.

Fig. 3.

The number of subjects included in the analysis by head and neck location group.