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CLINICAL DENTISTRY AND RESEARCH 2016; 40(2): 73-78 Case ReportCLINICAL DENTISTRY AND RESEARCH 2016; 40(2): 73-78 Olgu Bildirimi

CorrespondenceA. Pınar Sumer, DDS, PhD

Department of Dentomaxillofacial Radiology,

Faculty of Dentistry,

University of Ondokuz Mayıs,

55139 Kurupelit

Samsun, Turkey

Phone: +90 362 3121919-3290

Fax: +90 362 4576032

E-mail: psumer1970@yahoo.com

A. Pınar Sumer, DDS PhD Professor, Department of Dentomaxillofacial Radiology,

Faculty of Dentistry, University of Ondokuz Mayıs,

Samsun, Turkey

Armağan Çalışkan DDS Research Assistant, Department of Dentomaxillofacial Radiology,

Faculty of Dentistry, University of Ondokuz Mayıs,

Samsun, Turkey

Mahmut Sumer, DDS PhDProfessor, Department of Oral and Maxillofacial Surgery,

Faculty of Dentistry, University of Ondokuz Mayıs,

Samsun, Turkey

Ömer Günhan, MD, PhDProfessor, Department of Pathology,

Gülhane Military Medicine Academy,

Ankara, Turkey

DESMOPLASTIC FIBROMA OF THE MANDIBLE: A CASE REPORT

ABSTRACT

Desmoplastic fibroma of bone is an uncommon myofibroblastic

tumor characterized by local aggressive behavior that is associated

with a high-recurrence rate. The lesion most frequently occurs

in the mandibular posterior area in children or young adults.

Radiographically, it appears as a multilocular or occasionally

unilocular radiolucency with well-defined or ill-defined margins. The

treatment of patients with fibromatosis consists of wide surgical

excision. Radiation therapy sometimes has been used. This report

describes a case of desmoplastic fibroma in a 20 year old female

presenting as a firm, painless mass in the labial side of the left

mandibular lateral incisor with a radiolucency of adjacent bone on

radiographs.

Keywords: Desmoid Tumor, Fibromatosis, Mandible

Submitted for Publication: 01.23.2015

Accepted for Publication : 04.06.2015

Clin Dent Res 2016: 40(2): 73-78

CLINICAL DENTISTRY AND RESEARCH 2016; 40(2): 73-78 Olgu Bildirimi

Sorumlu YazarA. Pınar Sumer

Ondokuzmayıs Üniveristesi,

Diş Hekimliği Fakültesi,

Ağız Diş ve Çene Radyolojisi Anabilim Dalı,

55139 Kurupelit

Samsun, Türkiye

Telefon: +90 362 3121919-3290

Faks: +90 362 4576032

E-mail: psumer1970@yahoo.com

A. Pınar SumerProf. Dr. Ondokuzmayıs Üniveristesi, Diş Hekimliği Fakültesi,

Ağız Diş ve Çene Radyolojisi Anabilim Dalı,

Samsun, Türkiye

Armağan ÇalışkanAraş. Gör. Ondokuzmayıs Üniversitesi, Diş Hekimliği Fakültesi,

Ağız Diş ve Çene Radyolojisi Anabilim Dalı,

Samsun, Türkiye

Mahmut SumerProf. Dr. Ondokuzmayıs Üniveristesi, Diş Hekimliği Fakültesi,

Ağız Diş ve Çene Cerrahisi Anabilim Dalı,

Samsun, Türkiye

Ömer GünhanProf.Dr. Gülhane Askeri Tıp Akademisi,

Patoloji Anabilim Dalı,

Ankara, Türkiye

MANDİBULADA GÖRÜLEN DEZMOPLASTİK FİBROMA: VAKA RAPORU

ÖZ

Kemiğin dezmoplastik fibroması, yüksek rekürrens oranı ile ilişkili lokal agresif davranış ile karekterize nadir görülen bir miyofibroblastik tümördür. Radyografik olarak, iyi sınırlı veya sınırları belirsiz, multilokuler veya bazen unilokuler radyolusensi olarak izlenir. Tedavisi geniş cerrahi eksizyondur. Bazen radyasyon tedavisi de kullanılabilir. Bu raporda, 20 yaşında kadın hastada sol mandibular lateral kesici dişin labialinde sert, ağrısız bir şişlikle kendini gösteren ve ilgili kemikte radyolusensiye sebep olmuş bir dezmoplastik fibroma olgusu sunulmaktadır.

Anahtar Kelimeler: Dezmoid Tümor, Fibromatozis, Mandibula

Yayın Başvuru Tarihi : 23.01.2015

Yayına Kabul Tarihi : 06.04.2015

Clin Dent Res 2016: 40(2): 73-78

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Desmoplastic Fibroma

CLINICAL DENTISTRY AND RESEARCH 2016; 40(2): 73-78 Olgu BildirimiINTRODUCTION

The fibromatoses also called desmoids tumors are

rare neoplasms that develop from the tissue of the

musculoaponeurotic system.1 In the soft tissues of the head

and neck, these lesions are frequently called aggressive

fibromatoses or extraabdominal desmoids. Similar lesions

within the bone have been called desmoplastic fibromas.2

The lesion is characterized by local aggressive behavior but

it has no metastatic potential.1-3 It most frequently occurs

in children and young adults however it can affect any age

group.2,4 The age range of reported gnathic examples is

from 10 months to 59 years, with a mean of approximately

16 years.2 Although some studies have found a male

preponderance4,5, a female preponderance was found6,7

whereas other studies suggest no sex preponderance.8,9

The etiology remains unclear. However, a genetic

predisposition, trauma, hormonal factors, an association

with familiar adenomatous polyposis and Gardner’s

syndrome have been implicated.4 Aslan et al.10 reported a

case of fibromatosis that occurred after a trauma. Kındar11

described a case that occurred at the incision scar which is

extending to the superior peducular screw insertion point

after thoracal fracture stabilization. Most of desmoplastic

fibromas have occurred in the mandible, especially in the

molar angle-ascending ramus area. Patients usually complain

of facial swelling, rarely pain and dysfunction may be

observed.2,5 Said-Al-Naief 12 reported a case of desmoplastic

fibroma of the mandible in an 8-year-old boy who initially

presented with a 2-month history of a rapidly expanding,

painless mass along the right inferior border of his mandible.

Radiographically, the lesion appears as a multilocular or

occasionally unilocular destructive radiolucency with well-

defined or ill-defined margins.2 Because of lesion’s locally

aggressive nature, the preferred treatment is wide surgical

excision with a clear margin.2,13 Adjuvant treatments such

as radiotherapy, chemotherapy, hormonal therapy, and non-

steroidal anti-inflammatory drugs have also been used.4,5,7,14

In this article a case of desmoplastic fibroma in a 20 year old

female is presented.

CASE REPORT

A 20 year old female was referred with a one month history

of an expanding, firm, painless swelling of the left lower jaw.

The patient’s medical and family history was unremarkable.

Intraoral examination revealed a 0.5x0.5cm in size, hard,

non-tender mass on the labial side of the left mandibular

lateral incisor. No mucosal surface ulceration was present. Radiographically, a radiolucent destruction area was observed between the mandibular left lateral incisor and canine teeth in panoramic and periapical radiographs (Figure 1). As the lesion was smaller in size, conventional radiographs were sufficient. Computed tomography (CT), and magnetic resonance imaging (MRI) were not required. The lesion was removed surgically via an intraoral approach under local anesthesia and the surgical specimen was sent for histopathologic examination. In histopathologic examination, tumoral tissue parts were observed infiltrated irregularly into the striated muscle. The tumor was composed of ill-defined spindle fibroblastic cells and variable amount of collagen. No atypical mitosis was seen. The diagnosis of the lesion was fibromatosis (Figure 2). The patient was followed up for four years. Figure 3 shows the clinical appearance and radiological imaging of the lesion in four years time without any recurrence.

DISCUSSION

Fibromatosis is a rare benign tumor accounting for about 0.03% of all neoplasms and less than 3% of all soft tissue tumors. Head and neck desmoids tumors are estimated to constitute only 12% of all desmoids tumors throughout the body.9,13 The most common locations are the mandible, femur, pelvic bones, radius and tibia.2 In Sharma et al’s4 retrospective review, a total of 10 cases of fibromatosis of the head and neck were identified from a total of 340 cases of fibromatosis of the whole body. Pena et al.8 evaluated 97 cases of fibromatosis in the head and neck in the pediatric population and found that the majority of tumors were of the mandible (38%), and other locations included the submandibular area, neck, tongue, sinuses, upper lip and hard palate. In a literature review, 84% cases of desmoplastic fibromas involved the mandible and 70% of these cases were located posteriorly.12 Fibromatoses show an aggressive and infiltrative behavior5. The tumor does not have a capsule and its margins are often poorly defined so it tends to interdigitate with the underlying bone or soft tissue and invades adjacent neurovascular structures.4,5,8,13 A soft tissue mass will be occurred when the desmoplastic fibroma erodes through the cortex that may cause difficulty to determine whether the lesion is a desmoplastic fibroma of bone with soft tissue extension or a soft tissue fibromatosis with an extension into the bone.2 Seper et al5 evaluated 37 published cases of fibromatosis of the mandible and reported that most patients (89%) felt

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CLINICAL DENTISTRY AND RESEARCH

Figure 1. Intraoral photograph shows 0.5x0.5cm in size mass on the buccal side of the left mandibular lateral incisor (A), Panoramic (B) and periapical (C) radiographs show the destruction between the mandibular left lateral incisor and canine teeth

Figure 2. Intraoperative photograph showing the lesion being removed (A), Macroscopic appearance of the lesion (B), Histopathologic examination shows the desmoids tumor composed of ill-defined spindle fibroblastic cells infiltrated into the striated muscle (HEX100) (C)

Figure 3. Postoperative intraoral photograph (A), panoramic (B) and periapical (C) radiographs

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Desmoplastic Fibroma

no pain, and the signs such as tooth mobility, limitations of mouth opening were infrequent. A painless swelling of the affected area is the most common initial complaint.2 Tuncer et al15 reported a case of fibromatosis in an 11 year old girl noticed by an extraoral swelling. Fibromatosis should be considered in young patients submitting with a mass in head and neck region in early child-hood.16 In the present case the lesion was on the buccal side of the left mandibular lateral incisor and caused no pain or tooth mobility. X-ray examination, ultrasonography (US), CT, and MRI might be helpful for tumor management, preoperative planning and for follow-up.3 The radiographic features of desmoplastic fibromas are nonspecific. These include a unilocular or multilocular, well-defined or ill-defined radiolucency with variably expressed marginal sclerosis.2,12 Smaller lesions appear to be unilocular and when the lesion is large the internal aspect may be multilocular. Desmoplastic fibromas of bone can expand bone and often perforate the outer cortex.17 In Said-Al-Naief12 literature review about desmoplastic fibromas of the jaw, a 30% of cases had cortical perforation and 10% of cases had cortical expansion. MRI used for the assessment of the nature and size of the soft tissue lesion and involvement of surrounding structures. T1-weighted MRI, the internal structure has a low signal, which helps in determining intraosseous extent because of the contrast with the high signal from the bone marrow.17 CT is valuable in determining the tumor’s relation to bone and US will reveal a heterogenenous hypoechogenicity and increased surrounding vascularity.7,13 Usually CT scan and MRI are required to determine the exact soft tissue extent of the lesion.17 Especially in children, US or MRI was preferred to CT for the follow-up to prevent unnecessary radiation unless bone was involved.7 In the present case, panoramic and periapical radiographs showed a radiolucent destruction area of adjacent bone and because the lesion was small, there was no need for further radiologic examination. Differential diagnosis should be made with fibrosarcoma, fibroma, giant cell lesions and fibrous dysplasia.2,16 Radiographically, the periphery of the lesion most often is ill defined and has an invasive characteristic usually seen in malignant lesions.17 Distinguishing this neoplasm from a ill-defined fibrosarcoma may be difficult so all desmoplastic fibromas of bone should be considered potentially malignant.2,17

The management of fibromatosis is based on an accurate diagnosis by histologic and clinical examination.5 Because of its lack of capsule and infiltrative growth, a wide

excision of the tumor is advocated.13 Surgical resection was recommended for most of the cases1,5,8,13 similar to the present case and sometimes chemotherapy and radiotherapy was given.5,7,8,14 Wang et al.7 reported that a conservative resection with preservation of form and function should be given greater priority in all age groups for the treatment of aggressive fibromatosis. Additionally, postoperative adjuvant theraphy was vital for patients to obtain recurrence-free survival. A 23% recurrence rate has been reported for oral and paraoral fibromatosis but higher recurrence rate has been noted for other head and neck sites.2,13 Kruse et al.9 reported 30.07% recurrence of a total of 143 published cases of fibromatosis of the head and neck and found no correlation between recurrence and localization of the lesion. Küçük et al.18 reported a 29% recurrence rate for 31 cases diagnosed as aggressive fibromatoses and suggested adjuvant radiotherapy with surgical treatment for satisfactory clinical results. Close follow-up at least two but preferably more years because of the late relapses was necessary.1,7,13 Recurrences are treated essentially in the same way as primary tumors.13 The present case is different from other cases in the literature as it was smaller in size so surgical excision was used as treatment and no recurrence was observed for four years. Appropriate radiographic imaging and timing are important for the follow-up for satisfactory clinical results.

ACKNOWLEDGEMENT

The study was presented as a poster presentation in the 17th International Congress on Oral Pathology and Medicine at May 25-30, 2014 held in Istanbul, Turkey

REFERENCES

1. 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: 211-213.

2. Angela C. Chi. Bone Pathology. In: Neville BW, Damm DD, Allen CM, Bouquot JE. Editors. Oral and Maxillofacial pathology. St. Louis, Elsevier; 2009. p. 613-677

3. Castellazzi G, Vanel D, Le Cesne A, Le Pechoux C, Caillet H, Perona F et al. Can the MRI signal of aggressive fibromatosis be used to predict its behavior? Eur J Radiol 2009; 69: 222-229.

4. Seper L, Bürger H, Vormoor J, Joos U, Kleinheinz J. Aggressive fibromatosis involving the mandible-case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005; 99: 30-38.

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5. 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- 1604.

6. Stoeckle E, Coindre JM, Longy M, Binh MB, Kantor G, Kind M et al. A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases. Eur J Surg Oncol 2009; 35: 129-134.

7. Wang W, Koirala U, Ma S, Liu G, Ding M, Hu X et al. Age-based treatment of aggressive fibromatosis in the head and neck region. J Oral Maxillofac Surg 2014; 72: 311-321.

8. 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.

9. Kruse AI, Luebbers HT, Gratz KW, Obwegeser JA. Aggressive fibromatosis of the head and neck: a new classification based on a literature review over 40 years (1968-2008). Oral Maxillofac Surg 2010; 14: 227-232.

10. Arslan E, Işık AF, Şanlı M, Tunçözgür B, Elbeyli L. Toraks travması sonrası gelişen fibromatozis: Olgu sunumu. Türk Göğüs Kalp Damar Cerr Derg 2013; 21: 216-218.

11. Çeçen AD, Kibar Y, Süslü H, Tatarlın, Hiçdönmez T. Torakolomber Stabilizasyondan Sonra Gelişen Tekrarlayıcı Fibromatozis: Olgu Sunumu. Sinir Sistemi Cerrahisi Derg 2010; 3: 81-83.

12. Said-Al-Naief N, Fernandes R, Louis P, Bell W, Siegal GP. Desmoplastic fibroma of the jaw: A case report and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006; 101: 82-94.

13. de Bree E, Zoras O, Hunt JL, Takes RP, Suarez C, Mendenhall W et al. Desmoid tumors of the head and neck: a therapeutic challenge. Head Neck 2014; 36: 1517-1526.

14. Hu T, Jing G, Lv K. Aggressive fibromatosis in the maxilla. Br J Oral Maxillofac Surg 2009; 47: 129-131.

15. Tuncer N, Keskin A, Baykul T, Demiralp S. Fibromatosis: Vaka Raporu. T Klin Diş Hek Bil 1999; 5: 15-18.

16. Gözü A, Akpınar C, Olgaç V, Özsoy Z. Çocukluk Çağının Saldırgan Baş-Boyun Tümörü: Agresif Fibromatozis. Türkiye Klinikleri J Med Sci 2011; 31: 1559- 1562.

17. White SC, Pharoah MJ editors. Oral Radiology Principles and Interpretation 7thed. Canada: Mosby, Inc; 2014.

18. Küçük L, Keçeci B, Sabah D, Yücetürk G. Agresif fibromatozis: Prognostik faktörler ve cerrahi tedavi sonuçlarının değerlendirilmesi. Acta Orthop Traumatol Turc 2014; 48: 55-60.