CASE REPORT

Psammomatoid juvenile ossifying fibroma: case studyand a review

Leonardo Morais Godoy Figueiredo & Thaís Feitosa Leitão de Oliveira &

Gardênia Matos Paraguassú & Rômulo Oliveira de Hollanda Valente &

Wilson Rodrigo Muniz da Costa & Viviane Almeida Sarmento

Received: 30 September 2012 /Accepted: 11 February 2013 /Published online: 24 February 2013# Springer-Verlag Berlin Heidelberg 2013

AbstractBackground The group of fibro-osseous lesions from themaxillofacial region is very heterogeneous, but what theyall have in common is the substitution of normal bone byfibroblasts with the consequent formation of collagen fibers,as well as substitution by different types of mineralizedtissues which may be similar to bone or cement. Within thisgroup of lesions, the juvenile ossifying fibroma is found,considered a rare and recurrent benign fibro-osseous neopla-sia. The term juvenile ossifying fibroma has been used in theliterature to describe two histopathological variations of con-ventional ossifying fibroma. These variations are trabecularjuvenile ossifying fibroma and psammomatoid juvenile ossi-fying fibroma. Psammomatoid juvenile ossifying fibroma isan uncommon bone-forming neoplasm with aggressive localgrowth. Diagnostic of this lesion occurs after the correlation ofclinical, imaging, and histopathological findings. Proposed

treatments range from enucleation and curettage to resectionof the tumor.Objectives The present article has as its objectives to reportan uncommon case of a 4-year-old male patient treated byconservative approach and revise the literature on juvenileossifying fibroma.Conclusions Psammomatoid juvenile ossifying fibroma, forits being very uncommon, warrants further investigation inorder to establish the best treatment, principally in children,with a view to minimizing mutilating treatments. In the caseexamined, a conservative treatment was chosen, where thesurgeon established curette and cryotherapy, and the reinte-gration of the child in his social environment, without re-lapse during the first year of therapy.

Keywords Neoplasms . Bone tissue . Fibrous tissue .

Fibroma . Ossifying

Background

The group of fibro-osseous lesions of the head and neckregion is rather heterogeneous, but what the lesions all havein common is the substitution of normal bone by fibroblastswith the consequent formation of collagen fibers, as well asthe substitution by different types of mineralized tissues,which may be similar to bone or cement. As time haspassed, lesions with this morphology have come to bedescribed as juvenile ossifying fibroma (JOF), active JOF,aggressive ossifying fibroma, fibrous displasia, cherubism,and osteoblastoma [1–3].

Fibro-osseous lesions of the head and neck were firstreported in 1938 by Benjamins, who reported a psammomatoidJOF. However, he designated the lesion as atypical osteoidfibroma with ossification in the front sinus. In 1949, Gögl

L. M. G. Figueiredo (*)Department of Oral and Maxillofacial Surgery,Federal University of Bahia, Salvador,Bahia, Brazile-mail: leo_m_godoy@hotmail.com

T. F. L. de OliveiraDepartment of Oral Medicine, University of São Paulo,Bauru School of Dentistry, Bauru, São Paulo, Brazil

G. M. ParaguassúDepartment of Oral Medicine, Federal University of Bahia,Salvador, Bahia, Brazil

R. O. de Hollanda Valente :W. R. M. da CostaDepartment of Oral and Maxillofacial Surgery,Institute of Integral Medicine, Recife, Pernambuco, Brazil

V. A. SarmentoFederal University of Bahia,Salvador, Bahia, Brazil

Oral Maxillofac Surg (2014) 18:87–93DOI 10.1007/s10006-013-0400-y

renamed this lesion as psammomatoid ossifying fibroma of theparanasal sinuses and the nose, reporting two paranasal sinuscases in children. In 1952, Johnson denominated this samelesion as active JOF. He described the lesion as being a formof cellular and aggressive ossifying fibroma that normallyaffects children [4–7].

The World Health Organization in 2005 proposed classi-fication for odontogenic tumors: the concept of JOF wasdivided into psammomatoid JOF and trabecular JOF [8].The present article has as its objectives to report on a casestudy and revise the literature on psammomatoid JOF.

Case report

A 4-year-old male patient had his first treatment in pediatricemergency care, 3 weeks after falling from a bicycle, for rigidpersistent edema in the left hemiface and an absence of cuta-neous alteration or fluctuations (Fig. 1). A complementarypanoramic radiograph was done, and through this imaging,osteolytic lesions in the body and left mandibular angle weredetected (Fig. 2).

An incisional biopsy was performed and forwarded to theanatomic pathology service at the Oncology Clinic, thecondition being diagnosed as fibrous dysplasia. The patientwas then admitted for surgery for curettage of the lesion bymeans of intraoral access through which the pathologicaltissue was removed (Fig. 3). In the second histopathologicalexamination, a uniform cellular proliferation was observedin the presence of fibroblast fusiform cells. Among thefibroblast cells were found numerous irregular and sphericalossicles interspersed, exhibiting differing degrees of calcifi-cation (Fig. 4). No endothelial lining was found in thelesion; however, there were large sinusoid and vascularspaces full of red blood cells. In the histomorphological,clinical, and radiograph analyses of the lesion, the clinicalsuspect, psammomatoid JOF, was confirmed.

After 8 months of the curettage, the patient returnedpresenting a progressive new growth in the region, whichwas causing accentuated facial asymmetry. The panoramicradiograph showed a multilocular radiolucent area, infiltra-tive, cortical destructive aspect, in the left side of the man-dible (Fig. 5). At that time, a multislice computedtomography showed in the axial and coronal views anextensive and mixed hypodense/hyperdense aspect of thelesion, involving the bony cortex (Fig. 6). Clinical andradiological examination determined that the condition wasaggressive, indicating a new surgical approach, and thus, abasal mandibular corticomy with a central curette to theregion was performed (Fig. 7).

After 6 months, a region of trabecular bone compatiblewith the areas of relapse was noticed in imaginologic exams.

Fig. 1 Initial appearance of patient’s lesion. Left side in the face ofslow growth and painless

Fig. 2 Panoramic radiography showing the radiolucent area, poorlydefined in the left mandibular body, causing cortical expansion andtooth displacement

Fig. 3 First surgery with intraoral access and lesion curettage

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A new approach with extra-oral access was chosen, in whichosteotomy and cryotherapy played a role (Fig. 8). In an 18-month follow-up, a new irregular bone formation was notedin the operated area, as well as persistence in an increase involume (Fig. 9). The patient is being monitored and a futuresurgery with osteoplasty has been planned.

Discussion

JOF is a benign tumor in the bone. It is an uncommoncondition, representing around 2 % of the mouth tumorsthat occur in children and adolescents. It is distinct fromother fibro-osseous lesions in terms of the age at which itbegins the clinical framework, and the potential for growth.Generally, the tumors occur between ages 5 and 15 years.JOF has a greater prevalence in males. In areas with con-genitally absent teeth, the tumor grows rapidly and in anasymptomatic way in the anatomic regions concerned. It islocally aggressive and tends to recur, leading to the possi-bility of facial asymmetry [9–14]. Relapse is principally dueto incomplete removal of the tumor, which happens in 30 to58 % of these cases [15, 16]. What we have observed in the

case studied, where we have the admission of a 4-year-oldmale, is that the lesion showed rapid and aggressive growth,followed by two relapses.

Some cases of fibro-osseous lesions, including JOF, canbe associated with the brown tumors of hyperparathyroid-ism when they present as main symptoms of primary hyper-parathyroidism and kidney lesions. The diagnosis of thissyndrome is of the utmost importance because it can occurin other members of the family, besides increasing themalign potential of these fibro-osseous lesions [11, 17].However, in the case cited there was no correlation betweenthe brown tumors syndrome and hyperparathyroidism.

The literature relates JOF occurrence in regions such asthe maxilla bone, paranasal sinuses, and the front-ethmoidalorbital complex, but in few cases the jaw was involved. Inaccordance with the mineralization pattern, there exist con-ventional ossifying fibroma and JOF. The latter has beendescribed in the literature as having two distinct histopath-ological variations of ossifying fibroma that occur in thecraniofacial region: psammomatoid JOF and trabecular JOF[6, 13, 18–26]. The psammomatoid variety was observed inthe object of study.

Zama et al., Slootweg and Muller, and Sun et al. [24, 26,27] reaffirmed in their studies how difficult it is to establishcriteria for the definitive diagnosis of JOF. Toro et al. andSlootweg et al. [2, 21] described the maxilla as the regionwhere tumors occur the most. For Brannon and Fowler andfor Cuellar et al. [10, 28], the mandible was the predominantsite. However, Johnson et al. [15] affirm that 90 % of thelesions occur in the paranasal sinuses and 10 % in themandible. In our case, we observed aggravation of themandible. El-Mofty et al., Dominguete et al., and Rinaggioet al. [4, 11, 13] agree in their studies that JOF displays anaggressive behavior with rapid and painless growth in thebone that is attacked. The tumor presents specific character-istics according to the affected region, like exophthalmosand bulbar dislocation where the orbital cavity is involved,

Fig. 4 Photomicrograph of thehematoxylin–eosin specimenwith the presence of a histologicpattern of psammomatoidossicles set in cellularfibroblastic tissue (originalmagnification, ×10)

Fig. 5 Panoramic radiography after 8 months. Increase in the radiolu-cent area and expansion of the cortical bone

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nasal obstruction where the paranasal sinuses are concerned,and facial asymmetry when the mandible or maxilla isinvolved. In the cases where the jaw is involved, there is apainless swelling with expansion of the vestibular andtongue cortical areas. The tumor occurs more commonlyin the branch than in the body of the mandible, this beingcompatible with reports by Zama et al., Sun et al., Patil etal., and Sarode et al. [24, 27, 29, 30], as well as with whatwe have observed.

Radiographically, the manifestation is unilocular ormultilocular with radiopaque areas that are well defined,with possible central opacification and the presence of aline between the tumor and the healthy bone tissue. Onecan observe that in the most aggressive cases there are

perforations of the cortical bone. In the tomography, onewill note well-defined frontiers with a fine sclerotic bed ordamages to the cortical bone with the involvement of sur-rounding anatomical structures. The lesion’s consistency ispredominantly of soft tissue with a variable quantity ofinternal calcifications. Toro et al., Leimola-Virtanen et al.,and Papadaki et al. [2, 6, 31] agreed that JOF does not havespecific radiographic aspects, being possibly unilocular ormultilocular; however, it is radiography that makes it possi-ble to differentiate the diagnosis of JOF from fibrous dys-plasia, since fibrous dysplasia is less confined and notusually surrounded by a fibrous capsule.

Diagnosis occurs after a correlation is made betweenclinical, imaginological, and histopathological findings[12, 22, 31–33]. Histologically, JOF is characterized byhaving material rich in fibrous connective cellular tissue,not encapsulated but well contained by surrounding bone,and possessing a greater quantity of osteoblasts and a scar-city of osteoclasts. Myxomatous compositions can be found,which are frequently associated with pseudocystic degener-ation. Hemorrhage areas and small groups of multinucleargiant cells are generally observed. The mineralized compo-nent in the two patterns is very different. The trabecularvariety possesses irregular agglomerates of highly cellularosteoid, which contain voluminous and irregular osteocytes.In contrast, the psammomatoid pattern forms concentriclamellas and spherical ossicles that vary in form and presentbasophilic centers with an osteoid eosinophyllic peripheral

Fig. 6 a–d Computedtomography showing a largehypodense area in the leftmandibular body, causingexpansion of the cortical bone.Note the formation of discreteareas inside hyperdense. aAxial cut with a window forsoft tissues. b Axial cut withbone window. c, d Coronal cutwith a window for bone

Fig. 7 Second surgical intervention for curettage by extraoral access

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margin [17, 22]. These aspects were observed in the presentcase. For Smith et al. [10], the average age is 20 forpsammomatoid JOF and 8.5 to 12 years for trabecularJOF. In disagreement with this, El-Mofty and Papadaki etal. [4, 31] confirm in their studies that the two histopatho-logical variations of JOF present themselves with greaterprevalence in individuals between ages 2 and 15 years. Thecase presented shows a prevalence compatible with thatdemonstrated by El-Mofty and Papadaki et al. [4, 31].

The treatment for JOF can vary according to the aggres-siveness of the tumor. In the less aggressive cases, a con-servative surgery is chosen that includes curette and localexcision of the abnormality. This will bring more benefits tothe patients in the long term than a radical resection [5, 31].The conservative treatment may preserve the function of theinferior alveolar nerve, the masticatory function, and thenormal growth of the bone attacked—be it the mandible ormaxilla—besides esthetic preservation [5, 6, 31]. On theother hand, the JOF, which promotes a high rate of its owngrowth, fragility in the cortical bone, dental dislocation, orradicular resorption, requires en bloc resection within con-servative margins to prevent recurrence.

Kaban et al. [34] proposed an alternative treatment forchildren with aggressive giant cell tumor, based on curettageor enucleation, in combination with adjuvant interferonalpha therapy for 6 to 8 months. The authors obtained ahigh rate of tumor control with decreased operative morbid-ity compared with conventional treatment, although thepatients presented some collateral effects [34].

After resection, the surgeon must wait a while to decidewhether or not the bone margins have become free of thetumor in order to exclude the possibility of relapse. After aperiod of time given to patient follow-up, a reconstructionwith bone graft is performed [2, 5, 22, 24, 31, 35–38]. In somecases of JOF, with aggression toward the maxilla, orbit, andparanasal sinus, it was suggested, as an alternative, to intensifythe curette or enucleation treatment and provide interferonalpha chemotherapy for 1 year [31, 34, 37].

Leimola-Virtanen et al. and Tanaka et al. [6, 39] agree ona more conservative treatment for JOF, given that the greaternumber of oral and maxillofacial tumors in children arebenign. In contrast, Toro et al., El-Mofty et al., Smith etal., Sun et al., and Partridges et al. [2, 4, 10, 27, 35]preferred a more radical treatment, consisting of en blocresection with rigid internal fixation by reconstructionplaques, bone grafts, integrated bone implants, and prosthet-ic rehabilitation, understanding that one is dealing with atumor that is 30 to 58 % likely to recur. Troulis et al. [37]agreed with this, also performing the resection of the lesion,but with their own procedure comprising four phases thatinclude, besides resection, bone reconstruction, the insertionof implants, and prosthetic rehabilitation when possible. Bycontrast, Uckanet et al. and Rudolf [40, 41] suggested that inchildren and adolescents, the surgeon should take out theplaques of rigid internal fixation in a second surgical mo-ment after confirming bone consolidation. Brahim andCarmichael et al. [42, 43] complemented this by findingthe need for extreme caution when using implants in children,

Fig. 8 a, b Third surgery. aAppearance during surgery. bUse of cryocautery

Fig. 9 a–c Clinical appearance after surgery. Persistent deformation of the face. a After 4 months. b, c After 12 months

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due to possible alterations in the growth of maxillas anddentition, and, when possible, the insertion of implants shouldbe postponed until the age of 15 years for girls and 18 years forboys. In cases where it is not possible to delay these dentalimplants, the patient should be accompanied periodically. In arelated case, a conservative treatment was chosen in favor abetter long-term prognosis for the patient, being a child with abenign tumor. Hemi-mandibulectomy would be a mutilatingtreatment, and reconstruction would be complex in this casedue to the child’s stage of growth. Cryotherapy favored ne-crosis of areas adjacent to the lesion, offering safety in relationto the margins of the lesion and reduction in the chances ofrelapse.

Conclusions

Psammomatoid JOF, for its being very uncommon, warrantsfurther investigation in order to establish the best treatment,principally in children, with a view to minimizing mutilatingtreatments. In the case examined, a conservative treatmentwas chosen, where the surgeon established curette and cryo-therapy, and the reintegration of the child in his social envi-ronment, without relapse during the first year of therapy.

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