Desmoid-type fibromatosis of the head and neck in children: a case report and review of the literature

Abstract

BackgroundDesmoid-type fibromatosis is defined as an intermediate tumor that rarely occurs in the head and neck of children. There is no doubt as to the value of complete surgical excision for desmoid-type fibromatosis. However, in pediatric patients, surgeons may often be concerned about making a wide excision because of the potential for functional morbidity. Some studies have reported a lack of correlation between margin status and recurrence. Therefore, we discussed our findings with a focus on the state of surgical margins.Case presentationWe report an unusual case of a 9-month-old Japanese girl who prior to presenting at our hospital underwent debulking surgery twice with chemotherapy for desmoid-type fibromatosis of the tongue at another hospital. We performed a partial glossectomy and simultaneous reconstruction with local flap and achieved microscopic complete resection. We also reviewed available literature of pediatric desmoid-type fibromatosis in the head and neck.ConclusionsWe described successful treatment for the refractory case of pediatric desmoid-type fibromatosis. The review results showed that some microscopic incomplete resections of tumors in pediatric patients with desmoid-type fibromatosis tended to be acceptable with surgical treatment.

Desmoid Fibromatosis of the Maxilla

“…1,2 These lesions typically occur in a bimodal distribution in infants and young adults without a gender predilection. 3,4 The etiology of DF is unknown, but trauma and hormonal influences have often been cited as possible causative factors. 4 The DF has also been associated with hereditary genetic syndromes such as familial adenomatous polyposis and hereditary desmoid disease.…”

“…Traditional treatment has been wide local excision with negative surgical margins to decrease recurrence. 1,2,3,[9][10][11] Meazza et al reported a 22% versus 76% recurrence rate with DF with negative compared to positive surgical margins. 10 This has led to the use of adjuvant chemotherapy when positive margins are reported or recurrences occur, for tumor stabilization and regression.…”

“…2,7 Other case series have reported resolution of DF after incomplete resection with positive margins and have not advocated adjuvant treatment with positive margins. 3,11,12 Instead, they recommend salvage surgery for clinical recurrences and chemotherapy only for unresectable primary or progressive recurrent disease.…”

Sonography used in the infantile desmoid fibromatosis of postcricoid area

“…[ 3 ] It is defined as an intermediate tumor that rarely occurs in the head and neck of children. [ 4 ] Lesions in the head and neck are reported in 12% of all cases, and 71% occur in the first decade of life. [ 2 , 5 ] There has been challenge to the management of fibromatosis in these pivotal anatomic areas because complete excision with a negative margin is usually impossible, and thus frequent local recurrence is common in many cases.…”

Aggressive Resection of Cervical Desmoid Tumor Invading the Paraspinal Muscles without Recurrence at Eight Year Followup: A Case Report and Review of the Literature

“…When recurrence rates were compared in patients with grossly negative surgical margins and grossly positive surgical margins for head, neck, and spine DTs, two studies showed statistical significance (p < 0.05) for lower recurrence rate after gross total resection, and seven showed a trend toward lower recurrence rates in patients with gross total resection. A summary of the recurrence rates following grossly complete resections compared to grossly incomplete excisions in case series with DTs of the head and neck is presented [3,[22][23][24][25][26][27][28][29][30] (Table 1).…”