Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Journal of Medical Case Reports
Published in: Journal of Medical Case Reports 1/2016

Open Access 01-12-2016 | Case report

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

Authors: Hidetaka Miyashita, Seiji Asoda, Tomoya Soma, Kanako Munakata, Masaki Yazawa, Taneaki Nakagawa, Hiromasa Kawana

Published in: Journal of Medical Case Reports | Issue 1/2016

Login to get access

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.
Literature
1.
Barnes L, Eveson JW, Reichart P, Sidransky D. World Health Organization classification of tumors. Pathology and genetics of head and neck tumors. Lyon: IARC; 2005.
2.
Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg. 1986;151:230–7.CrossRefPubMed
3.
Baumert BG, Spahr MO, Von Hochstetter A, et al. The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network. Radiat Oncol. 2007;2:12.CrossRefPubMedPubMedCentral
4.
Tse GM, Chan KF, Ahuja AT, King AD, Pang PC, To EW. Fibromatosis of the head and neck region. Otolaryngol Head Neck Surg. 2001;125:516–9.PubMed
5.
Kasper B, Baumgarten C, Bonvalot S, et al. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise – a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer. 2015;51:127–36.CrossRefPubMed
6.
de Bree E, Zoras O, Hunt JL, et al. Desmoid tumors of the head and neck: a therapeutic challenge. Head Neck. 2014;36:1517–26.PubMed
7.
Humar A, Chou S, Carpenter B. Fibromatosis in infancy and childhood: the spectrum. J Pediatr Surg. 1993;28:1446–50.CrossRefPubMed
8.
Woltsche N, Gilg MM, Fraissler L, et al. Is wide resection obsolete for desmoid tumors in children and adolescents? Evaluation of histological margins, immunohistochemical markers, and review of literature. Pediatr Hematol Oncol. 2015;32:60–9.CrossRefPubMed
9.
Gronchi A, Casali PG, Mariani L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21:1390–7.CrossRefPubMed
10.
Honeyman JN, Theilen TM, Knowles MA, et al. Desmoid fibromatosis in children and adolescents: a conservative approach to management. J Pediatr Surg. 2013;48:62–6.CrossRefPubMed
11.
Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25:1785–91.CrossRefPubMed
12.
Wang CP, Chang YL, Ko JY, Cheng CH, Yeh CF, Lou PJ. Desmoid tumor of the head and neck. Head Neck. 2006;28:1008–13.CrossRefPubMed
13.
Alebouyeh M, Moussavi F, Tabari AK, Vossough P. Aggressive intra-abdominal fibromatosis in children and response to chemotherapy. Pediatr Hematol Oncol. 2005;22:447–51.CrossRefPubMed
14.
Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G. Desmoid fibromatosis: a clinicopathologic study of 25 children. Semin Diagn Pathol. 1986;3:138–50.PubMed
15.
Bisogno G, Tagarelli A, Stramare R, Beltrame V, Carli M. Hydroxyurea treatment can avoid the need for aggressive surgery in pediatric fibromatosis. J Pediatr Hematol Oncol. 2013;35:e171–3.CrossRefPubMed
16.
Buitendijk S, van de Ven CP, Dumans TG, et al. Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 2005;104:1090–9.CrossRefPubMed
17.
Burlini D, Conti G, Bardellini E, Amadori F. Rare case of desmoid-type fibromatosis of the mandibular region in a child: diagnosis and surgical management. Eur J Paediatr Dent. 2013;14:333–4.PubMed
18.
Caglar K, Ozyonum H, Akalin M, Balci S. Effective treatment of multifocal aggressive fibromatosis with low-dose chemotherapy. Turk J Pediatr. 2006;48:365–8.PubMed
19.
Carr RJ, Zaki GA, Leader MB, Langdon JD. Infantile fibromatosis with involvement of the mandible. Br J Oral Maxillofac Surg. 1992;30:257–62.CrossRefPubMed
20.
Crawley MB, Anand SM, Clain JB, Scherl S, Buchbinder D, Urken ML. Trismus release in a pediatric patient using a parascapular free flap reconstruction following desmoid tumor resection. Laryngoscope. 2013;123:1451–4.CrossRefPubMed
21.
De Corti F, Avanzini S, Cecchetto G, et al. The surgical approach for cervicothoracic masses in children. J Pediatr Surg. 2012;47:1662–8.CrossRefPubMed
22.
De Santis D. Fibromatosis of the mandible: case report and review of previous publications. Br J Oral Maxillofac Surg. 1998;36:384–8.CrossRefPubMed
23.
Ekinci S, Karnak I, Tanyel FC. Infantile fibromatosis of the sternocleidomastoid muscle mimicking muscular torticollis. J Pediatr Surg. 2004;39:1424–5.CrossRefPubMed
24.
Falco NA, Upton J. Infantile digital fibromas. J Hand Surg [Am]. 1995;20:1014–20.CrossRef
25.
Federici S, Mordenti M, Domenichelli V, Pelusi G, Straziuso S, Pericoli R. Successful combined treatment for giant mesenteric desmoid tumor: case report and review of the literature. J Pediatr Surg. 2012;47:e25–30.CrossRefPubMed
26.
Flacke S, Pauleit D, Keller E, et al. Infantile fibromatosis of the neck with intracranial involvement: MR and CT findings. AJNR Am J Neuroradiol. 1999;20:923–5.PubMed
27.
Fowler CB, Hartman KS, Brannon RB. Fibromatosis of the oral and paraoral region. Oral Surg Oral Med Oral Pathol. 1994;77:373–86.CrossRefPubMed
28.
Geist ET, Kent JN, Carr RF, Super S. Case 52: Multiloculated radiolucency of the left mandible. J Oral Maxillofac Surg. 1985;43:205–9.CrossRefPubMed
29.
Goepfert H, Cangir A, Ayala AG, Eftekhari F. Chemotherapy of locally aggressive head and neck tumors in the pediatric age group. Desmoid fibromatosis and nasopharyngeal angiofibroma. Am J Surg. 1982;144:437–44.CrossRefPubMed
30.
Gomez FM, Patel PA, Stuart S, Roebuck DJ. Systematic review of ablation techniques for the treatment of malignant or aggressive benign lesions in children. Pediatr Radiol. 2014;44:1281–9.CrossRefPubMed
31.
Hartstein ME, Thomas SM, Ellis LS. Orbital desmoid tumor in a pediatric patient. Ophthal Plast Reconstr Surg. 2006;22:139–41.CrossRefPubMed
32.
Hoffman CD, Levant BA, Hall RK. Aggressive infantile fibromatosis: report of a case undergoing spontaneous regression. J Oral Maxillofac Surg. 1993;51:1043–7.CrossRefPubMed
33.
Jabbari S, Andolino D, Weinberg V, et al. Successful treatment of high risk and recurrent pediatric desmoids using radiation as a component of multimodality therapy. Int J Radiat Oncol Biol Phys. 2009;75:177–82.CrossRefPubMed
34.
Jacob CI, Kumm RC. Benign anteromedial plantar nodules of childhood: a distinct form of plantar fibromatosis. Pediatr Dermatol. 2000;17:472–4.CrossRefPubMed
35.
Kau CH, Kamel SG, Wilson J, Wong ME. New method for analysis of facial growth in a pediatric reconstructed mandible. Am J Orthod Dentofacial Orthop. 2011;139:e285–90.CrossRefPubMed
36.
Koyluoglu G, Yildiz E, Koyuncu A, Atalar M. Management of an esophagogastric fibromatosis in a child: a case report. J Pediatr Surg. 2004;39:640–2.CrossRefPubMed
37.
Kraft SM, Singh V, Sykes KJ, Gamis A, Manalang MA, Wei JL. Differentiating between congenital rhabdomyosarcoma versus fibromatosis of the pediatric tongue. Int J Pediatr Otorhinolaryngol. 2010;74:781–5.CrossRefPubMed
38.
Kriss TC, Warf BC. Cervical paraspinous desmoid tumor in a child: case report. Neurosurgery. 1994;35:956–9. discussion 959.CrossRefPubMed
39.
Lackner H, Urban C, Benesch M, et al. Multimodal treatment of children with unresectable or recurrent desmoid tumors: an 11-year longitudinal observational study. J Pediatr Hematol Oncol. 2004;26:518–22.CrossRefPubMed
40.
Lackner H, Urban C, Kerbl R, Schwinger W, Beham A. Noncytotoxic drug therapy in children with unresectable desmoid tumors. Cancer. 1997;80:334–40.CrossRefPubMed
41.
42.
Maillard AA, Kountakis SE. Pediatric sino-orbital desmoid fibromatosis. Ann Otol Rhinol Laryngol. 1996;105:463–6.CrossRefPubMed
43.
Mannan AA, Ray R, Sharma SC, Hatimota P. Infantile fibromatosis of the nose and paranasal sinuses: report of a rare case and brief review of the literature. Ear Nose Throat J. 2004;83:481–4.PubMed
44.
Meng J, Zhuang Q, Tian Z, Fu H, He Y. Infantile fibromatosis of the pterygomandibular space. J Craniofac Surg. 2012;23:e542–4.CrossRefPubMed
45.
Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE, Rao BN. Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys. 2000;47:1267–71.CrossRefPubMed
46.
Neri HA, Villagra EJ, Alvarez AC, et al. Ethmoidal desmoid tumor in a pediatric patient. Otolaryngol Head Neck Surg. 2007;136:137–8.CrossRefPubMed
47.
Otmani N, Lamalmi N, Khattab M. Treatment of submandibular fibromatosis with chemotherapy and surgery. A case report. Minerva Stomatol. 2008;57:133–7.PubMed
48.
Pena S, Brickman T, StHilaire H, Jeyakumar A. Aggressive fibromatosis of the head and neck in the pediatric population. Int J Pediatr Otorhinolaryngol. 2014;78:1–4.CrossRefPubMed
49.
Perez-Cruet MJ, Burke JM, Weber R, DeMonte F. Aggressive fibromatosis involving the cranial base in children. Neurosurgery. 1998;43:1096–102.CrossRefPubMed
50.
Ramirez RN, Otsuka NY, Apel DM, Bowen RE. Desmoid tumor in the pediatric population: a report of two cases. J Pediatr Orthop B. 2009;18:141–4.CrossRefPubMed
51.
Reich S, Overberg-Schmidt US, Buhrer C, Henze G. Low-dose chemotherapy with vinblastine and methotrexate in childhood desmoid tumors. J Clin Oncol. 1999;17:1086.PubMed
52.
Roychoudhury A, Parkash H, Kumar S, Chopra P. Infantile desmoid fibromatosis of the submandibular region. J Oral Maxillofac Surg. 2002;60:1198–202.CrossRefPubMed
53.
Rutenberg MS, Indelicato DJ, Knapik JA, et al. External-beam radiotherapy for pediatric and young adult desmoid tumors. Pediatr Blood Cancer. 2011;57:435–42.CrossRefPubMed
54.
55.
Sato K, Kawana M, Nonomura N, Takahashi S. Desmoid-type infantile fibromatosis in the mandible: a case report. Am J Otolaryngol. 2000;21:207–12.CrossRefPubMed
56.
Seper L, Burger H, Vormoor J, Joos U, Kleinheinz J. Agressive fibromatosis involving the mandible – case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;99:30–8.CrossRefPubMed
57.
Sharma A, Ngan BY, Sandor GK, Campisi P, Forte V. Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review. J Pediatr Surg. 2008;43:1596–604.CrossRefPubMed
58.
Shindle MK, Khanna AJ, McCarthy EF, O’Neill PJ, Sponseller PD. Desmoid tumor of the spinal canal causing scoliosis and paralysis. Spine (Phila Pa 1976). 2002;27:E304–7.CrossRef
59.
Sinno H, Zadeh T. Desmoid tumors of the pediatric mandible: case report and review. Ann Plast Surg. 2009;62:213–9.CrossRefPubMed
60.
Spiegel DA, Dormans JP, Meyer JS, et al. Aggressive fibromatosis from infancy to adolescence. J Pediatr Orthop. 1999;19:776–84.PubMed
61.
Steuer KS. Pediatric fibromatosis requiring mandibular resection and reconstruction. AORN J. 1995;62:212. 215-224, 226.CrossRefPubMed
62.
Styczynski J, Lasek W, Wysocki M. Calcified fibromatosis of the neck in 4-year old girl: rapid growth, rapid therapy. Int J Pediatr Otorhinolaryngol. 2005;69:847–52.CrossRefPubMed
63.
Tandon S, Garg RK. Intraoral desmoplastic fibroma: a manifestation of tuberous sclerosis. Fetal Pediatr Pathol. 2012;31:195–201.CrossRefPubMed
64.
Tostevin PM, Wyatt M, Hosni A. Six cases of fibromatosis of the head and neck in children. Int J Pediatr Otorhinolaryngol. 2000;53:235–44.CrossRefPubMed
65.
Tzakis AG, Tryphonopoulos P, De Faria W, et al. Partial abdominal evisceration, ex vivo resection, and intestinal autotransplantation for the treatment of pathologic lesions of the root of the mesentery. J Am Coll Surg. 2003;197:770–6.CrossRefPubMed
66.
Wang B, Ma J, Jin H. Infantile intracranial aggressive fibromatosis: report of two cases with a review of the literature. Pediatr Neurosurg. 2012;48:181–6.CrossRefPubMed
67.
Wang W, Koirala U, Ma S, et al. Age-based treatment of aggressive fibromatosis in the head and neck region. J Oral Maxillofac Surg. 2014;72:311–21.CrossRefPubMed
68.
Watzinger F, Turhani D, Wutzl A, Fock N, Sinko K, Sulzbacher I. Aggressive fibromatosis of the mandible: a case report. Int J Oral Maxillofac Surg. 2005;34:211–3.CrossRefPubMed
69.
Wehl G, Rossler J, Otten JE, et al. Response of progressive fibromatosis to therapy with liposomal doxorubicin. Onkologie. 2004;27:552–6.PubMed
70.
Yazici N, Yalcin B, Soylemezoglu F, et al. Intracranial desmoid tumor with familial adenomatous polyposis coli. Pediatr Neurosurg. 2008;44:140–3.CrossRefPubMed
71.
Zampieri N, Cecchetto M, Zorzi MG, Pietrobelli A, Ottolenghi A, Camoglio F. An unusual case of extra-abdominal desmoid tumour. Eur J Cancer Care (Engl). 2010;19:410–2.CrossRef
72.
Zhou Y, Zhang Z, Fu H, Qiu W, Wang L, He Y. Clinical management of pediatric aggressive fibromatosis involving the mandible. Pediatr Blood Cancer. 2012;59:648–51.CrossRefPubMed
73.
Arnold DJ, Wax MK. Microvascular Committee of the American Academy of Otolaryngology – Head and Neck Surgery. Pediatric microvascular reconstruction: a report from the Microvascular Committee. Otolaryngol Head Neck Surg. 2007;136:848–51.CrossRefPubMed
74.
Bilkay U, Tiftikcioglu YO, Temiz G, Ozek C, Akin Y. Free-tissue transfers for reconstruction of oromandibular area in children. Microsurgery. 2008;28:91–8.CrossRefPubMed
75.
Skapek SX, Anderson JR, Hill DA, et al. Safety and efficacy of high-dose tamoxifen and sulindac for desmoid tumor in children: results of a Children’s Oncology Group (COG) phase II study. Pediatr Blood Cancer. 2013;60:1108–12.CrossRefPubMed
76.
Skapek SX, Ferguson WS, Granowetter L, et al. Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 2007;25:501–6.CrossRefPubMed
77.
Constantinidou A, Jones RL, Scurr M, Al-Muderis O, Judson I. Pegylated liposomal doxorubicin, an effective, well-tolerated treatment for refractory aggressive fibromatosis. Eur J Cancer. 2009;45:2930–4.CrossRefPubMed
78.
Garbay D, Le Cesne A, Penel N, et al. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol. 2012;23:182–6.CrossRefPubMed
79.
80.
Penel N, Le Cesne A, Bui BN, et al. Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up. Ann Oncol. 2011;22:452–7.CrossRefPubMed
81.
Nuyttens JJ, Rust PF, Thomas Jr CR, Turrisi 3rd AT. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer. 2000;88:1517–23.CrossRefPubMed
82.
Guadagnolo BA, Zagars GK, Ballo MT. Long-term outcomes for desmoid tumors treated with radiation therapy. Int J Radiat Oncol Biol Phys. 2008;71:441–7.CrossRefPubMed
Metadata
Title
Desmoid-type fibromatosis of the head and neck in children: a case report and review of the literature
Authors
Hidetaka Miyashita
Seiji Asoda
Tomoya Soma
Kanako Munakata
Masaki Yazawa
Taneaki Nakagawa
Hiromasa Kawana
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2016
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-016-0949-9

Other articles of this Issue 1/2016

Journal of Medical Case Reports 1/2016 Go to the issue

Case report

Management of severe dengue hemorrhagic fever and bleeding complications in a primigravida patient: a case report

Case report

Mechanical aortic valve without anticoagulation for 33 years in a Yemeni man: a case report

Case report

Salvage of bleeding renal allograft following biopsy, with suture technique: a case report

Editorial

Case reports in medical education: a platform for training medical students, residents, and fellows in scientific writing and critical thinking

Case report

Cholangiocarcinoma associated with limbic encephalitis and early cerebral abnormalities detected by 2-deoxy-2-[fluorine-18]fluoro-d-glucose integrated with computed tomography-positron emission tomography: a case report

Case report

Endometrial tuberculosis compounding polycystic ovary syndrome in a subfertile woman: a case report