Vol. 101 No. 1 January 2006 ORAL AND MAXILLOFACIAL RADIOLOGY Editor: Allan G. FarmanDesmoplastic ﬁbroma of the jaw: A case report and review of literature Nasser Said-Al-Naief, DDS, MS,a Rui Fernandes, DMD, MD,b Patrick Louis, DMD, MD,c Walter Bell, MD,d and Gene P. Siegal, MD, PhD,e Birmingham, Ala THE UNIVERSITY OF ALABAMA AT BIRMINGHAM Desmoplastic ﬁbroma is a benign intraosseous neoplasm that is recognized as the intraosseous counterpart ofsoft tissue ﬁbromatosis in both gnathic and extragnathic sites. It has a propensity for locally aggressive behavior and localrecurrence. In the present report, we deﬁne the clinicopathological and radiographic features of a desmoplastic ﬁbroma of themandible in an 8-year-old white boy who initially presented with a 2-month history of a rapidly expanding, painlessmass along the right inferior border of his mandible. A critical and comprehensive review of the English language literatureis also provided. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:82-94)Desmoplastic ﬁbroma (DF) is a rare, myoﬁbroblastic radiographic features of previously documented gnathictumor, comprising far less than 1% of all bone tumors DF emphasizing the outcome of various treatmentincluding benign bone neoplasms.1,2 It represents the modalities as implemented in clinical practice.osseous manifestation of aggressive ﬁbromatosis and wasﬁrst reported by Jaffe in 19583 who described multiple CASE REPORTsites of involvement in the tibia, scapula, and femur. The Initial presentationﬁrst report of gnathic involvement with DF is attributed The patient was an 8-year-old white boy who presented to the Oral and Maxillofacial Surgery Clinic at our institutionto Grifﬁth and Irby in 1965,4 and since then numerous with a 2-month history of a rapidly expanding, painless masssimilar cases have been described.4-54 The surgical along the right inferior border of his mandible. The patientapproach to the tumor has been a source of controversy, initially saw his pediatrician who referred him to an oral andand variable treatment modalities, with different out- maxillofacial surgeon. This surgeon initially felt that the childcomes, have been implemented. The following is a report most likely had a reactive process within the bone, and aof a case of desmoplastic ﬁbroma of the mandible follow-up appointment was scheduled for repeat examinationsupplemented by analysis of clinicopathological and and radiographic evaluation. The patient’s mother sought a second opinion from another oral and maxillofacial surgeona and the child was subsequently referred to us. The child and Assistant Professor, Department of Pathology, University of his mother denied any history of trauma. The patient’s pastAlabama at Birmingham; Associate Scientist, University of Alabama medical history was noncontributory. Clinical examinationat Birmingham Center for Metabolic Bone Disease ComprehensiveCancer Center. revealed a slight expansion of the right inferior border of theb Chief Resident, School of Dentistry, Department of Oral and mandible as well as soft tissue fullness, directly overlying theMaxillofacial Surgery, University of Alabama at Birmingham. bony lesion. The remainder of the head and neck examinationc Associate Professor and Residency Director, Department of Oral was within normal limits. A panoramic radiograph showedand Maxillofacial Surgery, University of Alabama at Birmingham. a disruption of the right inferior cortex of the mandible. Thed Assistant Professor, Department of Pathology, University of lesion extended from the inferior border in the premolarAlabama at Birmingham. region. It had a smooth border and was slightly radiopaquee Professor and Director, Division of Anatomic Pathology, Depart- (Fig. 1). A computerized tomography (CT) scan demonstratedment of Pathology, University of Alabama at Birmingham; Senior an 8-mm right mandibular body mass destroying the outerScientist, University of Alabama at Birmingham Center for Meta- cortex (Fig. 2). Under local anesthesia, a biopsy of the lesionbolic Bone Disease.Received for publication Aug 16, 2004; returned for revision Mar 22, was obtained and submitted for histological evaluation.2005; accepted for publication Mar 27, 2005.1079-2104/$ - see front matter HistopathologyÓ 2006 Mosby, Inc. All rights reserved. Microscopic examination revealed an ill-deﬁned spindledoi:10.1016/j.tripleo.2005.03.034 cell proliferation in a collagenous background with varying82
OOOOEVolume 101, Number 1 Said-Al-Naief et al. 83Fig. 1. Preoperative panoramic radiograph depicting a radi-olucent process at the inferior border of the right mandible(arrows). Fig. 3. A medium power photomicrograph showing the tumor arranged in spindle cell pattern forming short fascicles and bundles with mild swirling (hematoxylin and eosin stain, original magniﬁcation 3100). Insert: The spindle cell prolif- eration displayed less than 1 mitotic ﬁgure/10 high-power ﬁelds (arrow) (hematoxylin and eosin stain, original magni- ﬁcation 3400). appearance, with rare mitoses (less than 1/10 high-power ﬁelds), none were atypical (Fig. 3, insert). Residual bony fragments were noted at the periphery of the section. Immunohistochemical staining Parafﬁn-embedded tissue sections, ﬁxed in formalin, were stained with the following antibodies obtained from Dako Corporation (Carpinteria, CA), according to manufacturer recommendations: (1) polyclonal rabbit anti S-100 protein, used at a dilution of 1:400; (2) monoclonal mouse antihuman smooth muscle actin (clone 1A4), used at a dilution of 1:50; (3) monoclonal mouse antivimentin antibodies used at a dilutionFig. 2. A preoperative CT scan showing a lesion of the right of 1:50; (4) monoclonal mouse antihuman muscle-speciﬁcinferior border of the mandible. Note that the lesion has actin used at a dilution of 1:50; and (5) monoclonal mouseeroded the cortex and is expanding into the soft tissue antihuman Ki-67 antigen (clone MIB-1), an excellent marker(arrows). of proliferative activity in soft tissue sarcomas.1 Immunohis- tochemistry was performed as previously outlined by us.55 Brieﬂy, 5-mm sections were placed on Plus slides and bakeddegrees of myxoid changes. The architectural arrangement of overnight. Before staining, heat-induced epitope retrieval bythe spindle cells varied with groups of cells arranged in parallel microwaving the specimen under standard conditions, in thefashion mixed with short interlacing fascicles and intertwining presence of the Dako Target Retrieval System, was performedstrands (Fig. 3). The spindle cells were generally uniform in in all but the vimentin-stained sections. Slides were then
OOOOE84 Said-Al-Naief et al. January 2006 Fig. 5. The tumor cells showed strong and diffuse reactivityFig. 4. The tumor cells reacted focally with anti-SMA for antivimentin (original magniﬁcation, ABC, 3200).(smooth muscle actin) antibodies. Note the positive internalcontrol (vessel walls) at the edge of the section (white arrows) tooth development with remodeling of the inferior border and(original magniﬁcation, ABC, 3100). In the low-power no neurosensory deﬁcits (Fig. 6).photomicrograph, the black arrow highlights the immunore-activity to anti-SMA. This is more clearly seen in the higher Review of the literaturepower insert. Insert: Higher power photomicrograph demon- Clinical features. A detailed review of the Englishstrating focal staining with SMA (black arrow) (original literature in the past 40 years has generated a total of 74 casesmagniﬁcation, ABC, 3200). Nonspeciﬁc background staining of gnathic desmoplastic ﬁbromas, as presented in Table I.4-54deposits can be seen in the upper left corner of the There was a modest difference in gender distribution (45% inphotomicrograph. males vs 54% in females). Twenty-one percent of patients were white, 9% were Hispanics, 5% were Asian, and 4% were African American, whereas race was not documented in 39%stained using the Ventania ES Systems (Ventania Medical of the reported cases. The neoplasm occurred in a wide ageSystems, Inc, Tucson, AZ), although the primary antibodies range, extending from the ﬁrst to the sixth decades of life, withwere applied manually. This system used 3-amino, a-ethyl- 84% of patients being younger than 30 years of age atcarbazole (AEC) as the chromogen for detection of the avidin- diagnosis. The majority of cases (84%) involved the mandiblebiotin-antibody complex (ABC method). Negative controls and the balance (16%) occurred in the maxilla. Approximatelywere prepared in parallel by the use of an irrelevant primary 70% of the mandibular cases and 83% of the maxillary tumorsantibody or mouse serum as a substitute for the antibody of were located posteriorly.interest. Signs and symptoms reported also varied. Sixty-ﬁve percent The tumor cells showed focal reactivity for smooth muscle of patients presented with asymptomatic swelling on initialactin (Fig. 4), and strong and diffuse immunoreactivity for presentation. Pain, in or around area of involvement, wasvimentin (Fig. 5). Tumor cells were nonreactive for S-100 reported in 15% of patients, limited mouth opening/trismusprotein and muscle-speciﬁc actin and the MIB-1 (Ki67) index with or without malocclusion in 11%, tooth mobility in 7%,was low (3%). Based on the clinical, radiological, and proptosis in only 2.6%, the presence of infection (with orpathological appearance, a diagnosis of desmoplastic ﬁbroma without adenopathy) in 2.6%, and an elevation of an ear lobe inwas rendered. 1.3% of patients. Dysesthesia and bleeding were reported separately in 2.6% of patients. In 19.4% of patients, signs and symptoms were not documented. One patient8 was excludedSubsequent course from the data analysis since no information was provided. The patient underwent a resection with wide margins of the Radiographic features. Conventional radiographicright inferior border of his mandible. This was performed features were reported for 91% of the 74 cases. In general,through a neck incision. The resection was carried down to the 18% of patients had an ill-deﬁned appearance with an identicalinferior alveolar neurovascular bundle. The bundle, as well as number of cases showing well-deﬁned borders. However thisthe developing dentition, was preserved. No grafting was feature was not emphasized in 61% of cases. Thirty-six percentperformed. Microscopic ﬁndings for the resection specimen of patients displayed a multilocular appearance while only 6%were in agreement with the ﬁndings in the initial biopsy and showed a unilocular pattern. Only 4% of patients displayed awere conﬁrmatory of the diagnosis. The patient tolerated the mixed radiolucent/radipaque pattern or a radiopaque pattern.procedure well and was discharged on postoperative day 2. He The radiographic features were not further deﬁned in 39% ofhas been followed for the past 4.5 years without any clinical or cases. One patient was excluded (the last case in reference 32).radiographic signs of recurrence. The patient has had excellent Where information was available, cortical perforation was
OOOOEVolume 101, Number 1 Said-Al-Naief et al. 85Fig. 6. One-year postoperative panoramic radiograph without evidence of recurrence.identiﬁed in 30% of cases, cortical expansion in 10% of cases, bland, and monomorphic cells, supported by prominentcortical perforation accompanied by fracture in another 3%, collagenized to hyalinized connective tissue stroma. It isand cortical erosion without perforation in only 1 patient. The noteworthy to mention that scarce reports12,20,21,33 havetumors caused displacement of teeth, with or without resorp- alluded to the presence of focal hyperchromasia and raretion, in 18% of cases. It was also interesting to note that the mitoses (normal) similar to what we observed.neoplasm was reported to occur in multiple other locations andthus have overlapping radiographic features with other lesionsnative to the gnathic bones. DISCUSSION Management. Patient outcomes were documented in The World Health Organization deﬁnes the histolog-93.2% of cases. Five cases were excluded due to incomplete ical criteria for desmoplastic ﬁbroma as that of a benignremoval of the tumor initially,4,20,23 patient refusal of treat- tumor of low to variable cellularity, whose cells can bement,22 or if the treatment rendered was unclear.54 ovoid or elongated with uniform nuclei that lack atypia, Eleven percent of cases were managed by curettage with or pleomorphism and mitotic activity.56 The tumor cellswithout enucleation. Follow-up periods ranged from 5 months are supported by a matrix of collagenized, variablyto 5 years. Sixty-two percent of this cohort showed no evi- hyalinized ﬁbrous connective tissue.56,57 The etiologydence of disease while 31% of patients exhibited recurrence. of DF remains unknown, however trauma3,58 and endo-Information was not available on 1 patient. Fourteen percent of crine58 and genetic factors59 have all been suggestedcases were managed by excision. The follow-up period ranged as possible etiologic agents. While most investigatorsfrom 1 to 7 years, during which 10.5% of patients recurred and31.5% of cases did not. No information was available on 2 believe that DF represents the osseous counterpart ofremaining cases. A total of 5.7% of patients were treated by soft tissue ﬁbromatosis,16,19,25-27 others have classiﬁedenucleation. The follow-up period ranged from 3.25 years to 20 it as a variant of nonossifying ﬁbroma of bone,28 andyears. Seventy-ﬁve percent of cases from this cohort exhibited considered it to be biologically intermediate betweenno evidence of recurrent disease, while no information was benign ﬁbrous lesions and ﬁbrosarcoma.60available on the remaining case. Our review of the literature revealed a wide age- The majority of patients (51%) were resected, with 74% range distribution as previously reported2,61 but only ashowing no evidence of disease during a follow-up period that modest female predilection,31,58 arguing for equal genderranged from 3 months to 12 years. Only 4.3% of cases recurred distribution.38,56 Signs and symptoms are generallywhile no information was available on 6 patients. A minority nonpathognomonic and onset of symptoms is oftenof cases (10.1%) were managed with chemotherapy with or insidious, with major destructive lesions often discov-without other additional treatment. No recurrence wasexhibited in 85.7% during a follow-up period that ranged ered at time of initial presentation. On the other hand,from 9 months to 9 years. During that period 1 patient was pain and swelling are frequently mentioned.62-64presumed dead of disease. Any bone may be involved with DF,56 but the Almost all cases demonstrated a classical histological mandible is the most common site of involvement,pattern, identical to that of extragnathic ﬁbromatosis, ie, a whereas in extragnathic sites the metaphyseal region ofnonencapsulated tumor composed of uniformly distributed, femur, tibia, humerus, or radius, as well as the pelvic
OOOOE86 Said-Al-Naief et al. January 2006Table I. Clinopathological features of gnathic desmoplastic ﬁbroma Age/ Gender/ Radiographic Follow-upReference race Site pattern History Initial treatment period CommentGrifﬁth and 8 y/$/ L mandibular MLRL, displacement 8-year history of Curettage 6m Tumor was Irby 19654 white body and of unerupted teeth, inability to open incompletely ramus cortical perforation mouth completely, removed. and pathological swelling of L fracture mandibleMarlette and 21 y/#/ L mandibular MLRL, cortical 3-week history of Curettage 5y NED Gerhard NA angle expansion asymptomatic 19685 hard swelling of L mandibleRabhan and 13 y/#/ Mandible NA 2- to 3-week history of Excision 2y Suspicious for Rosai, NA asymptomatic mass in recurrence? 19686 mandibleHinds et al. 15 y/#/ L mandibular MLRL, cortical 1-year history of painful Excision, then 9m NED 19697 LA ramus, Inﬁlt perforation swelling in L mandible, radical resection ration to decrease in opening and surrounding slight deviation of structures mandible to the L on opening, history of extraction of mandibular L 3rd molar 3 months priorSchmaman 46 y/#/ Mandible NA NA Excision 5y Recurrence treated with et al. 19708 white hemi-mandibulectomyGosserez 1 y/$/ L mandibular Ill-deﬁned RL, 4-month history of Horizontal 1 y and Recurrence treated et al. 19709 NA body and cortical asymptomatic hard cervicotomy. 8m by surgical angle destruction swelling in L excision, NED submandibular region after 3-month follow-upMartis and 30 y/$/ L mandibular MLRL 4-year history of facial Radical resection/ NA NA Karakasis NA body and deformity and hemi- 197210 ramus asymptomatic mandibulectomy progressive swelling in L mandible with intraoral swellingDehner 197311 13 y/#/ L mandibular RL 3-week history of Excision 7y NED NA body asymptomatic ﬁrm swelling in L mandible 13 y/#/ L mandibular RL 1-year history of Resection 1y NED NA body and asymptomatic ﬁrm angle, soft swelling in L mandible tissue extension 13 y/$/ L maxilla, palate Ill-deﬁned RO 3-month history of Resection/subtotal 2y NED NA and maxillary occupying asymptomatic mass maxillectomy sinus maxillary protruding through socket sinus, erosion of previously extracted of inferior maxillary molar orbital rimBear 197312 26 y/$/ L mandibular MLRL extending 2-year history of facial Curettage NA NA NA body and from L bicuspid asymmetry and ramus to 3rd molar asymptomatic area, cortical progressive swelling of expansion mandible extending from mental foramen area to mandibular angle, tooth mobility in the area, obliteration of L buccal vestibule intraorallyHoving a and 15 y/$/ L anterior Well-deﬁned MLRL Few year history of Resection 3m NED Ingenhoes NA mandibular occupying asymptomatic cherry-size 197413 body interproximal area hard swelling in between R mandibular R mandibular alveolar lateral incisor and ridge, history of extraction canine, teeth separated of a supernumerary tooth apart in the area
OOOOEVolume 101, Number 1 Said-Al-Naief et al. 87Table I. Continued Age/ Gender/ Follow-upReference race Site Radiographic pattern History Initial treatment period CommentFerguson 5 y/$/ R mandibular Ill-deﬁned MLRL at Asymptomatic swelling Excision 6y NED 197414 NA angle mandibular angle, in R mandibular cortical perforation, angle reactive bone formation 38 y/#/ R mandibular ULRL, cortical History of pain in R Enucleation 8y NED NA ramus expansion mandibular quadrant, caused by broken, carious, wisdom tooth, tumor was incidentally discoveredBadger et al. 13 y/#/ L mandibular Irregular MLRL, 4- to 6-year history Excision 1 y and NED 197415 white ramus and cortical erosion, of asymptomatic 8m angle reactive slowly progressive subperiosteal bone swelling of L deposition mandible, history of trauma to area 6 to 8 years priorCunningham 4 y/#/ R mandibular MLRL extended Several month history of Resection/R hemi- 1 y1 NED et al. white body/ from 2nd occasional tooth ache, mandibulectomy 197516 submandibular deciduous molar 3-month history of area up to the hard swelling of condyle and R mandible, intraoral coronoid swelling processes, cortex thinned but intactSood and 21 y/#/ R posterior RO of R maxillary 4-year history of gradually Aggressive curett-age/ 2 y NED Chatterjee In maxilla/ sinus, thickening progressive swelling in peripheral 197517 maxillary of R orbital ﬂoor, R maxilla and inferior ostectomy sinus thickening of roof orbital margin started and lateral wall of asymptomatic but R maxillary sinus, developed vague pain enlargement of 6 months after zygomaCalatrava and 2 y/$/ L mandibular Ill-deﬁned RL 2-month history of diffuse Chemotherapy, then 2 y? NED. A diagnosis of Donado SP ramus and symptomatic swelling hemi-mandibulectomy low-grade ﬁbrosarcoma 197618 angle at L pre-auricular was rendered at ﬁrst region and then a deﬁnitive mandibular angle, diagnosis of intraoral swelling desmoplastic ﬁbroma was establishedFisker and 27 y/$/ L mandibular MLRL, teeth 2-month history of Curettage 2y NED Philipsen NA body displaced facial asymmetry and 197619 swelling at L mandible, history of trauma to area 5 years previously, teeth mobility, intraoral expansionSummers and 21 y/$/ R posterior RO of R Asymptomatic right facial Enucleation/ NA NA Matz white maxilla/ maxillary swelling, expansion of curettage 197620 maxillary sinus R maxillary alveolar sinus, soft process, history of removal tissue of a similar lesion from extension R maxillary sinus 10 years ago 59 y/#/ L mandibular Well-deﬁned RL Painful intraoral swelling Enucleation? NA NA, the lesion was white ascending occupying mandibular over L mandibular ramus, incomplete associated with ramus and ramus and condyle asymptomatic swelling in removal of incidental traumatic condyle, soft with extension into the anterior aspect of lesion neuroma tissue L maxillary alveolar alveolar ridge, history of extension ridge removal of similar lesion from the area 10 years ago Continued
OOOOE88 Said-Al-Naief et al. January 2006Table I. Continued Age/ Gender/ Follow-upReference race Site Radiographic pattern History Initial treatment period CommentNussbaum 3 y/$/ L mandibular Large RL extending 3-month history of Resection/hemi- 1 y1 NED et al. white body from from parasymphysis asymptomatic progressive mandibulectomy 197621 parasymphysis to ramus L mandibular swelling, to the intraoral buccal and ramus lingual cortical expansionWagner et al. 25 y/#/ L mandibular MLRL extending from Facial asymmetry and Refused treatment N/A N/A 197722 LA body and premolar area to swelling at inferior ramus ramus, cortical border of mandible perforationFreedman 26 y/$/ L mandibular Well-deﬁned ULRL with 4-month history of Curettage 6m Residual tumor present, et al. AA body at interradicular asymptomatic L moderate patient remained 197823 premolar and extension at premolar mandibular swelling with asymptomatic 4 years molar area and molar area, root intraoral swelling in after, no further divergence and mandibular buccal treatment was resorption, inferior vestibule, teeth mobility rendered displacement of the inferior alveolar canal, focal cortical perforationTaguchi and 27 y/$/ L mandibular Ill-deﬁned RL extending History of L mandibular Hemi-mandibulectomy 1 y and NED Kaneda Or body, ramus, from lower R 1st swelling, deviation of 8 m? 198024 and condyle molar to the ascending mandible to the R on ramus, cortical opening, TMJ arthralgia destruction and crepitus with malocclusionOsguthorpe 41 y/#/ L posterior Large RL involving L 3-month history of Maxillectomy/ 9m NED et al. white maxilla/ maxillary sinus with progressive L infraorbital modiﬁed en 198125 maxillary sinus destruction of sinus hypoesthesia, nasal block resection and pterygoid walls erosion stiffness with ﬁrm plates, focal of pterygoid plate asymptomatic cheek soft tissue swelling, proptosis extensionGreen and 23 y/$/ L mandibular Well-deﬁned MLRL, 6-month history of Resection 1 y and NED Gaffney white body and teeth displacement asymptomatic cystic 8m 198126 ramus lesion of L mandible with expansion of L posterior mandibular alveolar ridgeSlootweg and 3 y/$/ Posterior Well-deﬁned ULRL 6-month history of Enucleation 20 y NED Muller white mandibular adjacent to an mandibular swelling 198327 inferior unerupted tooth. border 2 y/$/ Posterior Well-deﬁned ULRL with 3-month history of Excision with 2y Recurred, treated with white mandible suggestion of mandibular swelling curettage resection then 2nd pericoronal recurrence after 5 location years treated with block resection, NED after 15 years follow-upSiemssen and 18 y/$/ R mandibular Ill-deﬁned Asymptomatic lesion in Enucleation, then 1 y and NED Anagnostaki NA body and angle MLRL right mandible, wide excision. 4m 198428 discovered by routine radiographic examinationEisen and 46 y/#/ R posterior Ill-deﬁned mixed 4-year history of Excision 1y NED Butler AA maxilla/ RL/RO of the R asymptomatic R facial, 198429 maxillary maxillary sinus lateral nasal, and labial sinus vestibule swellingAddante and 6 y/#/ R mandibular Well deﬁned RL, cortical 1-month history En bloc resection/ 2y NED Laskin NA body and perforation of asymptomatic segmental 198530 submandibular R submandibular mandibulectomy area swelling extending to midline
OOOOEVolume 101, Number 1 Said-Al-Naief et al. 89Table I. Continued Age/ Gender/ Follow-upReference race Site Radiographic pattern History Initial treatment period CommentGeorge et al. 22 y/#/ R maxilla, Mixed MLRL/RO lesion 4-month history of Curettage 1y NED 198531 white beneath the in interradicular area asymptomatic slowly zygoma 2nd premolar/3rd enlarging swelling in R molar, loss of lamina maxilla including R dura, obliteration of maxillary alveolar ridge ﬂoor of maxillary and vestibule, teeth sinus, cortical mobility and displacement perforationAyala et al. 2 y and 3 L mandible NA 8-month history of slowly Chemotherapy with 4 y and NED 198632 m/#/ progressive swelling in partial response, 3m LA L mandible then excision, then chemotherapy again 3 y and L mandible Destructive RL 2-month history of Partial excision, then 9 y and NED 7 m/$/ asymptomatic mass chemotherapy with 5m white in L mandible good response, then additional excision and then more chemotherapy 2 y and R maxilla NA 2-week history of painful Total resolution of 6 y and NED 9 m/#/ mass in R maxillary molar tumor with 4m white area simulating an chemotherapy alone odontogenic infection 3 y & 7m/ L mandible NA 4-month history of Excision, then partial 7y NED $/LA asymptomatic mass in mandibulectomy L mandible. with positive margins, followed by total resolution of tumor with chemotherapy 4 y and Posterior NA 3-week history of swelling in Chemotherapy with 1y NED 9 m/$/ Mandible mandibular molar good response, then LA simulating an odontogenic conservative infection resection 9 y and R posterior CT: large mass, 4-month history of earache Chemotherapy for 7 9 m then 9 m alive with disease 11 m/$/ maxilla, involving R skull and difﬁculty in chewing, months with no lost to then lost to follow-up, LA extension into base, with mass in R maxilla response follow- presumed dead from skull base and nasopharyngeal discovered following tooth (uncontrolled up disease nasopharynx extension and extraction diseases) sphenoid destructionBertoni et al. 6 y /#/ R mandibular MLRL, cortical 1-month history of Aggressive 9m Recurrence, treated by 198633 white ramus perforation asymptomatic facial curettage excision/resection, swelling NED after 3 years. 1 y and R mandibular Well-deﬁned RL, cortical 1-month history of Marginal 2 y and NED 10 m/$/ body, condyle, perforation asymptomatic facial resection 10 m white ramus, and swelling angle 30 y/$/ R mandibular Ill-deﬁned RL, focal 1-year history of Aggressive curettage 6y NED white body, cortical perforation asymptomatic facial premolar/ swelling molar areaHietanen et al. 13 y/#/ R mandible, Ill-deﬁned ULRL in Asymptomatic swelling in Excision NA NA 198634 NA canine interradicular area, alveolar crest discovered premolar area teeth displacement during routine examination, teeth displacedMakek and 2 ½ y/#/ R mandibular MLRL 18-month history of Resection 4y Recurrence, treated by Lello NA body and asymptomatic R facial resection, 2nd 198635 ramus swelling from distal tooth recurrence after 4 germ to the ramus, R years treated with mandibular deviation and resection. NED after malocclusion 27 years follow-up, parasthesia present Continued
OOOOE90 Said-Al-Naief et al. January 2006Table I. Continued Age/ Gender/ Follow-upReference race Site Radiographic pattern History Initial treatment period Comment 33 y/$/ R mandibular Ill-deﬁned MLRL from R 2-month history of Enucleation and 3y NED, mild NA body, ramus premolar to sigmoid asymptomatic swelling in local excision hypoesthesia and angle. notch area, cortical R mandibular angle present. perforation 15 y/#/ L mandibular MLRL extending from 2-month history of Resection 11 y NED, anesthesia exists. NA ramus and 3rd molar to asymptomatic progressive angle mandibular ramus and swelling of L mandibular angle ascending ramus, intermittent pain, elevation of L ear lobe, intraoral expansionSchultz et al. 7 y/$/ L mandibular Well-deﬁned MLRL, Several weeks’ history of Curettage 5m Recurrence, 198636 NA ramus cortical destruction swelling in L mandible, treated by limited mouth opening excisionRubin et al. 7 y/$/ L mandibular Well-deﬁned MLRL 1-month history of moderate Aggressive 10 m Recurrence, treated by 198737 AA body and swelling in L mandibular curettage aggressive curettage, angle body and angle, trismus, recurred again after intraoral swelling in 1 year, treated by mandibular vestibule hemimandibulectomy, NED after 1-year follow-upKwon et al. 9 y/#/ R mandibular Large RL in the 3-week history of Curettage 9m Recurrence with 198938 NA body and mandibular body and asymptomatic R facial symptoms, treated ramus ramus in second swelling with partial premolar and molar hemimandibulectomy, areas, teeth displaced NED after approximately 4-year follow-upDe Vito et al. 16 m/$/ L mandibular Destructive RL, cortical 6-month history of Curettage 1y NED 198939 NA body/inferior perforation progressive L mandibular mandibular swelling and facial border asymmetryValente et al. 29 y/$/ R mandibular Irregular RL apical to 5-year history of a repeated En bloc 2y NED 198940 white body endodontically treated abscess like lesion apical resection lower R second to a tooth simulating an premolar, apical root odontogenic infection resorptionChristiansen 8 y/$/ L mandibular ULRL distal to Asymptomatic swelling of Resection 1y Recurrence treated with 199041 white body, ramus, unerupted 2nd molar, left mandible, lower left resection, recurred and condyle inferior alveolar nerve lip parasthesia, deviation again after 1 year involvement, cortical of mandible to the L on treated again with perforation opening resection, NED after 3 year follow-upBoon et al. 51 y/#/ R mandibular MLRL, cortical 6-month history of painful Resection 1y NED 199142 Ind. angle and perforation with progressive swelling in R ramus pathological fracture mandible, local of lower mandibular lymphadenopathy, border, root resorption intraoral swellingVally and NA/$/ Posterior RL Slow-growing asymptomatic Resection NA NA Altini white mandible hard swelling in posterior 199043 mandible 42 y/#/ Posterior RL Slow-growing asymptomatic Resection NA NA AA mandible hard swelling in posterior mandible 10 m/#/ Posterior RL Slow-growing asymptomatic Resection NA NA AA mandible hard swelling in posterior mandible 21 y/$/ Posterior RL Slow-growing asymptomatic Resection NA NA AA mandible hard swelling in posterior mandible 12 y/#/ Posterior RL Slow-growing asymptomatic Resection 1 y and NED AA mandible hard swelling in posterior 6m mandible
OOOOEVolume 101, Number 1 Said-Al-Naief et al. 91Table I. Continued Age/ Gender/ Follow-upReference race Site Radiographic pattern History Initial treatment period Comment 22 y/$/ Posterior RL Rapidly growing Resection 6 y and NED AA mandible. asymptomatic hard 6m swelling in posterior mandible 27 y/#/ Maxilla RL Slow-growing asymptomatic Resection 4y NED AA hard swelling in maxilla 32 y/$/ Posterior RL Slow-growing asymptomatic Excision 1y NED AA mandible hard swelling in posterior mandibleHashimoto 15 y/#/ L maxilla and RL, indistinct maxillary 1-year history of Resection 7y NED et al. Or maxillary sinus sinus ﬂoor and asymptomatic swelling, L 199144 posterior wall, cortical maxillary buccal vestibule perforation, root and molar region, displacement and infraorbital swelling, resorption palatal displacement and extrusion of L. maxillary molar, history of trauma to areaSleeman 29 y/#/ R posterior Ill-deﬁned RL in R 1-week history of En block resection 18 m NED et al. NA maxillary posterior maxilla, asymptomatic spontaneous 199345 alveolar ridge teeth destruction bleeding from R maxillary distal to quadrant, inﬂamed premolars attached gingiva, sinus tract was also presentCranin et al. 2 ½ y/#/ R mandibular MLRL, cortical 1-month history of Resection 12 y NED 199446 AA body from thickening asymptomatic ﬁrm molar to canine swelling of R mandible region 9 y/#/ L mandibular ULRL from molar area 2-year history of delayed en-block resection 2y NED white body and to the ramus, teeth eruption of L mandibular ramus displacement, stunted molar, otherwise negative growth of condyle ﬁndings, the tumor exists head for 5 or more years?Miyamoto 29 y/$/ L. mandibular Well deﬁned ULRL, 3-month history of facial Curettage with 3y NED, history of tuberous et al. Or body, incisors cortical thinning and asymmetry and peripheral sclerosis with mental 199547 and premolar expansion asymptomatic swelling in ostectomy retardation, area L mandibular incisors and angiomyolipoma of premolar area the kidney, cerebral calciﬁcation, facial adenoma sebaceumIwai et al. 3 y and 9 L mandibular MLRL extending from 2-week history of Resection 6y NED 199648 m/$/ body & area of 2nd primary asymptomatic ﬁrm Or ramus molar to ramus, swelling in L mandible cortical destruction, periosteal reaction with sun-ray like patternHopkins 13 y/#/ R posterior and Well-deﬁned ULRL, 3-month history of gradual Resection 10 m then NED et al. NA anterior cortical expansion swelling of R Mandible lost for 199649 mandibular long-term body follow-up 19 y/$/ R mandibular ULRL, slight cortical Asymptomatic swelling Partial resection 1 y and NED NA body and expansion discovered on routine 7m parasymphysis examinationTempleton 6 y/$/ R mandibular Ill-deﬁned MLRL, 10-month history of rapidly Continuity/segmental 2 y and NED et al. NA body and cortical perforation, progressive trismus resection 6m1 199750 ramus root resorption unresponsive to orthodontic management, mildly tender swelling of R mandibular ramus and diffuse ﬁrm mass in the R posterior mandibular vestibule, slight deviation of mandible to the R with opening Continued
OOOOE92 Said-Al-Naief et al. January 2006Table I . Continued Age/ Gender/ Follow-upReference race Site Radiographic pattern History Initial treatment period CommentBakaeen and 4 y/$/ R. mandibular Well deﬁned ULRL, A 3 month history of Aggressive curettage/ 3 y NED Rajab NA angle cortical destruction asymptomatic slowly peripheral 199951 enlarging hard swelling ostectomy at R. mandibular angleCupero et al. 14 y/$/ R posterior Homogeneous R palatal and maxillary R maxillectomy with 2 y1 NED. 200152 NA maxilla with destructive RL of R gingival- buccal sulcus orbital preservation orbital maxilla, maxillary swelling, proptosis involvement sinus, and R orbital/ displacement of R nasal ﬂoors, R hard posterior maxillary teeth palatal perforationHereford et al. 11 y/$/ R mandibular MLRL associated with 3 1-year history of progressive L marginal NA NA, this report may 200153 NA angle impacted teeth, well- asymptomatic swelling of mandibulectomy represent an example deﬁned mixed RL/RO L mandible, history of and excision of R of synchronous posterior to the ﬁrst desmoplastic ﬁbroma cutaneous nodule ﬁbromatosis? lesion removed with marginal mandibulectomy 5 years prior from contralateral side, a cutaneous nodule was also present in the R periauricular region biopsy proven to be ‘‘Desmoid tumor’’Kaplan and 3 y/#/ R anterior Destructive RL, lingual Several-month history of soft Incisional NA NA Torske NA mandible cortical perforation tissue mass in R anterior biopsy, resection 200254 with soft tissue mandible, history of visits later? extension into ﬂoor to ER prior to making the of mouth diagnosis, due to sudden R mandibular swelling, treated as an odontogenic infection by antibiotics#, Male; $, female; C, Caucasians; LA, Latin; Or, Oriental; In, Indian; AA, African/African American; L, left; R, right; RL, radiolucent; RO, radioopaque;MLRL, multilocular radiolucency; ULRL, unilocular radiolucency; RL/RO, mixed radiolucent and radioopaque; y, years; m, months; NA, not available;NED, no evidence of disease.bones, are well reported as other primary sites.62,65 In a be similar to that seen in a low-grade central osteosar-review of 184 cases, DF most commonly involved the coma,59 may further lead to an inaccurate diagnosis.mandible (22%), followed by the femur (15%), pelvic Magnetic resonance imaging (MRI) has been shown tobones (13%), radius (12%), and tibia (9%).2 contribute little to the differential diagnosis; however, In general, the radiographic features of DF are due to its clear separation of intraosseous tumors fromnonspeciﬁc. These include a unilocular or multilocular, normal bone marrow, MRI is most valuable in surgicalwell-demarcated or irregular radiolucency with variably planning,2 while CT is considered superior to MRI inexpressed marginal sclerosis.19 Therefore, DF often demonstrating the cortical breakthrough seen in 29% ofmimics other common as well as unusual pathologies patients.66of the jaws including ameloblastoma, odontogenic Immunohistochemical stains may not be alwaysmyxoma, aneurysmal bone cyst, chondromyxoid helpful in distinguishing this neoplasm from otherﬁbroma, central hemangioma, and eosinophilic gran- spindle cell tumors and tumor-like lesions that involveuloma.49 Other unusual patterns have been also described. the oral and maxillofacial bones. The tumor cells mayThese include periapical pathosis in association with an not be reactive with antibodies directed against smoothendodontically treated tooth,40 and in an interradicular muscle actin and muscle-speciﬁc actin,62 but immuno-position at presentation.31 reactivity with the vascular markers anti CD34 and A sunray radiographic presentation, mimicking CD31 has been occasionally reported.67-69 In the presentosteogenic sarcoma48 may be especially signiﬁcant case, the neoplasm did not stain with vascular markersbecause, although it has been previously described in (with well-delineated positive internal controls, high-benign intraosseous tumors,59 it may wrongly lead to a lighting the vascular structures in the section), whilediagnosis of malignancy. Further, the rapid growth and highlighting the low proliferative activity (MIB-1) ofbone destruction often seen in association with DF, the lesion making a malignant lesion such as ﬁbrosar-coupled with a spindle cell histological pattern that may coma less likely.
OOOOEVolume 101, Number 1 Said-Al-Naief et al. 93 Arguably, low-grade ﬁbrosarcoma is the most chal- follow-up period is no less than 3 years.59 Our reviewlenging differential diagnosis and the most important has conﬁrmed that the method of surgery affects thelesion to differentiate from DF. Unlike ﬁbrosarcoma recurrence rate. Resection and excision are superiorwhere cells typically assume fascicular growth pattern over curettage in minimizing postsurgical recurrence,and often produce a so called ‘‘herring bone’’ appear- which was signiﬁcantly lower in the resected cases,ance, DF favors a single cell orientation and may be seen when compared to ones treated by curettage. Interestingly,arranged in bundles. Additionally, overlapping of the our review of the literature has also alluded to thespindle cells, increased mitotic activity, pleomorphism, presence of subtle atypia or mitotic ﬁgures, which wasand paucity of collagenous background are character- also demonstrated in the present case. In the currentistic features of ﬁbrosarcoma, and not DF. Another case, we present the management of a moderatelyfeature that favors a diagnosis of desmoplastic ﬁbroma cellular DF, which was treated with a wide localis the presence of indistinct cell borders and a cytoplasm excision. The patient has been followed in our clinicthat merges with the supporting collagenous back- for the past 4.5 years without recurrence.ground. The cells also often contain multiple, yet smallnucleoli. In certain circumstances, the distinction REFERENCESbetween the 2 conditions may not be possible and all 1. Weiss S, Goldblum J. Enzinger and Weiss’s soft tissue tumors. 4th ed. 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Desmoplastic ﬁbroma of the mandible mimicking osteogenic sarcoma: report of a case. J Oral Department of Pathology, SDB 81 Maxillofac Surg 1996;54:1370-3. University of Alabama at Birmingham49. Hopkins KM, Huttula CS, Kahn MA, Albright JE. Desmoplastic 1530 3rd Avenue South ﬁbroma of the mandible: review and report of two cases. J Oral Birmingham, AL 35294-0007 Maxillofac Surg 1996;54:1249-54. email@example.com