The Desmoid Tumor Research Foundation

November 2020

Desmoid tumors of the head and neck in the pediatric population: Has anything changed? (FULL ARTICLE)

Author Information

Zhao CX(1), Dombrowski ND(1), Perez-Atayde AR(2,3), Robson CD(4,5), Afshar S(6,7), Janeway KA(8,9), Rahbar R(1,10).

(1)Dept of Otolaryngology & Communication Enhancement, Boston Children's Hospital, Boston, MA, United States. (2)Dept of Pathology, Boston Children's Hospital, Boston, MA, United States. (3)Dept of Pathology, Harvard Medical School, Boston, MA, United States. (4)Dept of Radiology, Boston Children's Hospital, MA, United States. (5)Dept of Radiology, Harvard Medical School, Boston, MA, United States. (6)Dept of Plastic & Oral Surgery, Boston Children's Hospital, Boston, MA, United States. (7)Dept of Oral and Maxillofacial Surgery, Harvard Medical School, Boston, MA, United States. (8)Pediatric Hematology/Oncology, Dana-Farber Cancer Institute, Boston, MA, United States. (9)Dept of Pediatrics, Harvard Medical School, Boston, MA, United States. (10)Dept of Otolaryngology, Harvard Medical School, Boston, MA, United States.

Publication

I J Ped Otor

Summary

Pediatric head and neck desmoid tumors are rare neoplasms that can cause significant morbidity due to infiltration of vital anatomic structures. The goal of this study is to review presentation, evaluation, and management of these tumors. Retrospective study of children with head and neck desmoid tumors treated from 1999 to 2018 and literature review. 11 patients (5 boys, 6 girls) were included. Presentation included firm neck mass (n = 8), trismus (n = 2) and tongue lesion (n = 1). All patients had preoperative imaging with CT (n = 2), MRI (n = 1) or both (n = 8). Five patients underwent needle biopsy, five had open biopsy and one was diagnosed on pathology from primary excision. Seven patients were treated by primary surgical resection, with positive surgical margins in six cases due to proximity to vital neurovascular structures. None needed chemotherapy, had disease recurrence or progression. Three patients with unresectable disease were treated with chemotherapy. One patient was monitored with imaging without any treatment and did not have disease progression. Follow-up ranged from 6 months to 6 years (median 21 months). Ten patients (7 surgical, 2 chemotherapy, 1 observation) were either disease-free or had stable disease at last follow-up.

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