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The Desmoid Tumor Research Foundation

August 2019

DESMOID-TYPE fibromatosis of the head and neck in children: A Changing situation

Author Information

Paul A(1), Blouin MJ(1), Minard-Colin V(2), Galmiche L(3), Coulomb A(4), Corradini N(5), Boutroux H(6), Van den Abbeele T(7), Leboulanger N(1), Denoyelle F(1), Garabedian EN(1), Couloigner V(1), Orbach D(8).

(1)Pediatric Otolaryngology Dept, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Paris, France. (2)Pediatric Oncology Unit, Gustave-Roussy Institute, 94800, Villejuif. (3)Pathology Dept, Necker-Enfants Malades Hospital, Public Assistance-Hospitals of Paris, Universite Sorbonne Paris Cite, 75015, Paris, France. (4)Dept of Pathology, Armand Trousseau Hospital, Public Assistance-Hospitals of Paris, Paris, France. (5)Institut DÂ’Hematologie et DÂ’Oncologie Pediatrique, Centre Leon Berard, Lyon, France. (6)Hematology and Oncology Dept, Armand Trousseau Hospital, Public Assistance-Hospitals of Paris, Paris, France. (7)Pediatric Ear, Nose, and Throat Dept, Robert Debre Hospital, Public Assistance-Hospitals of Paris, Paris, France. (8)SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL Univ, Paris, France.

Publication

Int J Ped Otor

Summary

Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies.This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

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