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

Abstract

Background: Desmoid-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 presentation: We 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.

Conclusions: We 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.

Keywords: Case report; Children; Decision-making; Desmoid; Fibromatosis; Head and neck; Tongue.

Fig. 1

The patient could not close her mouth completely because of the presence of a part of the tumor outside her mouth. The maximum tumor size reached 5 cm

Fig. 2

a Magnetic resonance imaging (MRI) showing huge mass with contrast enhancement in the right side. b MRI of saggital plane did not show the posibility of infiltration into the root of the tongue. Arrows indicate the areas of tumor with contrast enhancement

Fig. 3

a A partial glossectomy with a 5-mm safety margin was performed. b We performed simultaneous reconstruction with a local flap and rotation of the anterior tongue on the unaffected side into the tongue defect on the affected side

Fig. 4

Intraoral findings after a year postoperatively

Fig. 5

Details of margin status and disease condition in recurrent patients. CR complete resection, DOD dead of disease, MIR microscopic incomplete resection, NED no evidence of disease, NR no report, PD progressive disease, Positive MIR or Residual, PR partial response, Residual gross incomplete resection, SD stable disease

Fig. 6

Disease condition in all patients (n=141). DOD dead of disease, NED no evidence of disease, NR no report, PD progressive disease, PR partial response, SD stable disease

Fig. 7

The number of recurrences for each margin status. CR complete resection, MIR microscopic incomplete resection, NR no report, Positive microscopic incomplete resection or residual gross incomplete resection, Residual gross incomplete resection

Fig. 8

Relationship between margin status and disease condition in the recurrent patients. CR complete resection, DOD dead of disease, MIR microscopic incomplete resection, NED no evidence of disease, PD progressive disease, Positive microscopic incomplete resection or residual gross incomplete resection, PR partial response, Residual gross incomplete resection, SD stable disease