Huge Facial Desmoid Tumors with Neck Extension: A Case Report

Document Type: Case Report

Authors

1 Department of Otorhinolaryngology, Jondishapour University of Medical Sciences, Ahwaz, Iran.

2 Department of Otorhinolaryngology, Damascus University of Medical Sciences, Damascus, Syria.

3 Department Oral and maxillofacial surgery, Damascus University of Medical Sciences, Damascus, Syria.

Abstract

Introduction:
Desmoid tumors are very rare, benign fibrous neoplasms arise from the musculoaponeurotic structures throughout the body.
 
Case Report:
The patient was a   seven-year old boy with a large mandibular mass growing over a period of six months. His CT-scan showed a large mass, 13 cm in diameter in the cheek area extending to the neck and trachea. Biopsy was compatible with desmoid fibromatosis.  He was given neoadjovant treatment with vinblastin and methotrexate. The patient underwent a tracheostomy. Then a complete hemimandibulectomy and submandibular gland excision was performed.  Finally reconstruction with latisimus dorsi free flap was performed.
 
Conclusion:
Despite rarity desmoid tumors should be kept in mind of an otorhinolaryngologist as a differential diagnosis in children with head and neck mass.
 

Keywords


1. ShieldsCJ,Winter DC, KirwanWO, Redmond HP. Desmoid tumors. Eur J SurgOncol2001; 27 (8):701–6.

2. Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome: mew aspects in the cause, pathogenesis and treatment ofthedesmoid tumor. Am J Surg1986; 151: 230–7.

3. Timothy Anderson, Gregory S. Weinstein, John Harwick et al. Hypopharyngealdesmoidtumor. Otolaryngology Head Neck Surg 2000; 123(3): 279-81.

4. Koeda S, Nagasaka H, Kumamoto H, Kawamura H. Extra-abdominal fibromatosis of the cheek: report ofa case. J Oral Maxillofac Surg 2005; 63(8): 1222–6.

5. Goy BW, Lee SP, Eilber F. The role of adjuvant radiotherapyin the treatment of resectabledesmo idtumors. Int J RadiatOncolBiolPhys 1997; 39:
659-65.

6. Buitendijk S, van de Ven CP, Dumans TG. Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer 2005; 104(5):1090-9.

7. Wever de I, Cin dal P, Fletcher CDM,  Mandahl N,Mertens F, Mitelman F, et al. Cytogenetic,clinical, and morphologic correlations in 78 cases of fibromatosis: a report from the CHAMP Study Group. Mod Pathol2000; 13 (10): 1080–5.

8. Lackner H, Urban C, Kerbl R, Schwinger W, et al. Noncytotoxicdrug therapy in children with unresectabledesmoidstumors. Cancer1997(2); 80:      334–40.

9. Faulkner LB, Hajdu SI, Kher U, Quaglia M La, Exelby P R, Heller G, et al. Pediatric desmoids tumor: retrospective analysis of 63 cases. J ClinOncol 1995; 13(11): 2813–18.

10. Acker JC, Bossen EH, Halperin EC. The management of desmoidtumors. Int J Radiat Oncol Biol Phys 1993; 26(5):851-8.

11. Buitendijk S, Van de Ven CP, Dumans TG. den Hollander JC, Nowak PJ, Tissing  WJ, et al. Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer 2005; 104(5):1090-9.