Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review

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1 /jp-journals JIAOMR Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review CASE REPORT Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review 1 Preeti Nair, 2 Anil Kumar, 3 Karthik Hegde, 4 Shiba Neelakantan 1 Professor and Head, Department of Oral Medicine and Radiology, Peoples College of Dental Sciences and Research Center Bhopal, Madhya Pradesh, India 2 Reader, Department of Oral Medicine and Radiology, Peoples College of Dental Sciences and Research Center, Bhopal Madhya Pradesh, India 3 Senior Lecturer, Department of Oral Medicine and Radiology, Peoples College of Dental Sciences and Research Center Bhopal, Madhya Pradesh, India 4 Postgraduate Student, Department of Oral Medicine and Radiology, Peoples College of Dental Sciences and Research Center, Bhopal, Madhya Pradesh, India Correspondence: Shiba Neelakantan, Postgraduate Student, Department of Oral Medicine and Radiology, Peoples College of Dental Sciences and Research Center, Bhanpur Bypass Road, Bhopal, Madhya Pradesh, India, shiba.roby@gmail.com ABSTRACT Psammomatoid Juvenile Ossifying Fibroma (PJOF) is an uncommon neoplasm that is distinguished from the adult variant of ossifying fibromas on the basis of age, site, clinical behavior and microscopic appearance. It is seen in children younger than 15 years of age, and the maxilla is more commonly involved than the mandible. PJOF may exhibit erosion and invasion of the surrounding bone accompanied by rapid enlargement. PJOF can be distinguished from other maxillofacial fibro-osseous lesions by its tendency to recur and its clinical mimicry of malignant bone tumors, but some clinical and histological features of PJOF overlap with the other fibro-osseous lesions as well. We report a case of a 15-year-old female patient with a painless apparently slow growing tumor in the left malar region, which occupied almost the whole of the left maxillary sinus, eroding the orbital border of the sinus. Keywords: Psammomatoid, Juvenile ossifying fibroma, Psammoma bodies. INTRODUCTION Maxillofacial fibro-osseous lesions are a generic designation of a group of jaw disorders characterized by the replacement of bone by a connective tissue matrix, which displays varying degrees of mineralization in the form of woven bone or of cementum-like structures indistinguishable from cementicles. 1 These lesions are of particular interest to the maxillofacial radiologist because they emphasize the role of radiologist in the diagnostic process. In general, fibro-osseous lesions of the maxillofacial region present a diagnostic dilemma for the clinician and pathologist owing to the overlapping clinical and histomorphologic findings. Juvenile ossifying fibroma, like ossifying fibroma, is a central lesion of bone composed of variable amount of fibrous tissue proliferation and foci of calcification. 2 Grossly the juvenile ossifying fibroma appears as a discrete mass well demarcated from the surrounding bone. In the second edition of WHO classification of odontogenic tumors 1992, psammomatoid juvenile ossifying fibroma is classified under Neoplasms and other lesions related to bone. 1 The prefix juvenile indicates its occurrence in younger individuals. The term active refers to clinical aggressive behavior, such as bone erosion and soft tissue invasion. The most common clinical manifestation is proptosis. Other symptoms include nasal obstruction, headache, swelling and rarely epistaxis. Two histological patterns are classically described: (1) trabecular and (2) psammomatoid. 2-4 Psammomatoid juvenile ossifying fibroma is an aggressive variant of ossifying fibroma usually seen in the pediatric age group that is histologically characterized by the formation of psammoma body islands. 2 The word psammos is derived from the Greek word meaning sand. 5 The most distinctive component in these lesions is the presence of calcified ossicles containing osteocytes known as psammomatoid bodies. Psammomatoid juvenile ossifying fibroma (PJOF) has its peak incidence in the first and second decades of life with a slight male predilection. The preferred sites are paranasal sinuses, maxilla, ethmoid and frontal bones. 3 CASE HISTORY A 15-year-old female patient reported to the Department of Oral Medicine and Radiology with a painless swelling on left side of the face since one year. Patient noticed a small swelling of the face which gradually increased in size over a period of one year and the swelling was asymptomatic. On consulting the local dentist, she was advised antibiotics and analgesics. Patient did not report any reduction in the swelling. Later on, her left upper first molar was removed suspecting it to be a causative factor. Past medical and family history was noncontributory. On general physical examination, patient was conscious, cooperative, healthy, moderately built and well nourished. All vital signs were in the normal range. She had attained menarche at the age of 12 years. Extraoral examination showed facial asymmetry due to swelling over the left malar area. The left eye appeared smaller in size. Local examination of the area revealed a single diffuse swelling present on left malar area measuring approximately 2 3 cm size. Anteriorly, the swelling extended 1 cm from the ala of the nose to posteriorly 2 cm in front of the tragus of left ear. Superioinferiorly, it extended from the left orbit S53

Preeti Nair et al to 3 cm short of inferior border of mandible. Skin over the swelling and the surrounding area appeared normal.

Intraoral examination revealed obliteration of the vestibule in relation to the maxillary left canine and premolars. On inspection a single diffuse swelling was present in the vestibule sulcus.

On palpation swelling was tender and inspectory findings were confirmed. Swelling was hard in consistency and expansion of the buccal cortical plate was present.

2 Preeti Nair et al to 3 cm short of inferior border of mandible. Skin over the swelling and the surrounding area appeared normal. On palpation temperature of the swelling was not raised, but swelling was tender and all inspectory findings were confirmed. Swelling was hard in consistency. Intraoral examination revealed obliteration of the vestibule in relation to the maxillary left canine and premolars. On inspection a single diffuse swelling was present in the vestibule sulcus. Anteriorly, the swelling extended from the maxillary left canine to the second premolar posteriorly. Superioinferior borders of the swelling could not be evaluated (Fig. 1). On palpation swelling was tender and inspectory findings were confirmed. Swelling was hard in consistency and expansion of the buccal cortical plate was present. No expansion was noticed on the palatal aspect. Fig. 2: Circumscribed radiopacity in the left sinus region Fig. 1: Obliteration of the vestibular sulcus in relation to the maxillary left canine and premolars A provisional diagnosis of monostotic fibrous dysplasia involving the left maxilla was made. Differential diagnosis included ossifying fibroma and benign odontogenic tumor. Radiographic investigations included, maxillary lateral topographic occlusal view, which showed expansion of the buccal cortical plate and diffuse radiopacity in the molar region. PNS view showed circumscribed radiopacity occupying almost the whole of the maxillary sinus with well-defined borders (Fig. 2). Serum alkaline phosphatase was found to be within normal limits. CT examination revealed sclerosis, bone expansion, softening and bone ossification noted extensively in the left maxilla and maxillary sinus, which measures cm (Fig. 3). Nasal septum was mildly deviated to the right. Axial section CT showed the lesion destroying orbital margin of the sinus. CT findings were suggestive of either an ossifying fibroma or fibrous dysplasia involving the left maxilla. A more probable diagnosis of juvenile ossifying fibroma was considered because of the age of the patient. An incisional biopsy was done from the left maxilla. Microscopic examination of specimen revealed highly fibro Fig. 3: Sclerosis, bone expansion, softening and bone ossification extensively in the left maxilla and maxillary sinus cellular mesenchymal tissue containing abundant spherical and globular calcified masses or ossicles. The mesenchymal cells are mostly plump and spindle shaped. Upon correlating with clinical and radiographic findings, a final diagnosis of psammomatoid juvenile ossifying fibroma was established (Fig. 4). S54 JAYPEE

3 Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review Fig. 4: Photomicrograph showing highly fibrocellular plump and spindle shaped mesenchymal tissue containing abundant spherical and globular calcified masses or ossicles The lesion was surgically excised and microscopic examination of specimen revealed psammomatoid bodies with osteoid rims, embedded in a cellular stroma composed of uniform stellate and spindle shaped cells with little collagen. Immature osteoids with some showing irregular mineralization in the center were also seen. Excisional biopsy report confirmed the diagnosis of psammomatoid juvenile ossifying fibroma. Postoperative healing was uneventful and patient was reviewed after one month and has been advised to report every six months for review, keeping in mind the high recurrence rates of this particular neoplasm. REVIEW OF LITERATURE Psammomatoid juvenile ossifying fibroma is a very uncommon lesion. In an extensive review by El Mofty in 2002, 230 cases were reported till date. 3 The term psammomatoid ossifying fibroma was originally used by Gogl in 1949 to describe two cases, one affecting the frontal sinus in a 5-year-old boy and the other, the ethmoid sinus in a 9-year-old girl. 3,6 Before this Benjamins in 1938 reported a lesion of the frontal sinus, which he termed osteoid fibroma with atypical calcification that was histologically similar to Gogl s cases. He cited earlier references by Moser in 1899, Fetissof in 1929, and Ringertz in 1938, who had described similar pathological characteristics, but assigned different nomenclature. 7 Moser called the lesion a sarcoma, Fetissof called it spongy osteoma, and Ringertz called it juvenile basal fibroma. Although, Benjamins identified these lesions as the same entity, his nomenclature is no longer applied. In the interim, however, other synonymous nomenclature had been used, including least differentiated form of fibro-osteoma, young ossifying fibroma, cementifying fibroma, psammo-osteoid-fibroma, and psammomatoid ossifying fibroma. 7 Johnson et al in 1952 was the first to use the term juvenile active ossifying fibroma. 3 Makeke in 1983 reviewed 86 cases and considered the lesion to be a variant of osteoblastoma and termed it psammous desmo-osteoblastoma and he called the trabecular variant as trabecular desmoosteoblastoma. 3,8 In a later review of 112 cases, Johnson et al in 1991 described PJOF as a cellular mass, which generates innumerable small uniform sized osteoid bodies. 3 It has been proposed that the mesenchymal cells of the periodontal membrane serve as multipotential precursor cells capable of differentiation into cementum, osteoid, or fibrous tissue and give rise to a spectrum of fibro-osseous lesions. 2,9 According to some investigators, they perhaps originate from maldevelopment of the tissue generating the bony septa between the roots of molar teeth. 3 Johnson et al hypothesized that the lesion arose from the overproduction of myxofibrous cellular stroma normally involved in the development of septa in the paranasal sinuses as they enlarge and pneumatize. 10 Recent studies also demonstrate the presence of nonrandom chromosome break points at Xq26 and 2q33 resulting in (X; 2) translocation. 3,4 In reviews published by Hamner et al and Slootweg et al the mean age of onset was 11.5 and 11.8 years old respectively. 10,11 Bruce M Wenig et al in 1995 in a published report mentioned the youngest age in their study as five years, but also had patients in the fourth and sixth decade. 12 The onset of psammomatoid ossifying fibroma is often in the mid-teenage years, and the average duration of symptoms before presentation is 4 to 5 years. 5 Johnson et al suggested that these lesions may be present for extended periods of time, beginning in adolescence, but only manifest in adulthood once the lesion has gained an appreciable size, thus still qualifying as a juvenile lesion. 3 In general, PJOF commonly occurs in patients between the ages of 5 and 15 years and is more often seen in males than females. Johnson et al reviewed 3000 fibro-osseous lesions and found that 112 met the criteria for the juvenile active ossifying fibroma. Among the facial tumors, 90% arise from the paranasal sinuses and the remaining 10% involve the mandible. 3 Cases involving the temporal bone have also been reported. Faizan et al reported that the maxillary tumors often fill and obliterate the maxillary sinus whereas mandibular tumors usually involve the ramus and angle. 13 In 84% of the patients with PJOF, the tumors are located in the facial bones, but calvaria (12%) and extracranial sites (4%) can be involved 3. Slootweg et al reviewed cases from the university hospitals at Groningen and Utrecht and noted that psammomatoid type, termed juvenile ossifying fibroma WHO [World Health Organization] type in the study, had a predilection for the maxilla. Lehrer HZ in 1969 and Johnson LC et al in 1991 reported a predilection towards supraorbital locations (frontal and ethmoid bones), the ethmoid sinuses and the maxillary sinuses. 12 Margo et al reviewed 21 cases involving the orbit and found 10 cases involving the orbital plate of the frontal bone (supraorbital) and nine cases centered in the ethmoid sinus. 3 Lawton et al reports on ethmoid sinuses are the most commonly involved, followed by the frontal sinuses, maxillary sinuses, and sphenoid sinus. 7 Johnson et al in 1952 described PJOF as a cellular and aggressive form ossifying fibroma usually affecting children as the lesion proliferates, grows, invades and destroys tissue until the eyes are extruded and cerebrospinal fluid is reached. 3 The first clinical manifestation is swelling of the maxilla. When the orbital bone and paranasal sinuses are involved, the patients may develop exophthalmos, bulbar displacement and nasal obstruction. 11 S55

4 Preeti Nair et al Clinically, PJOF presents with proptosis, visual disturbances, blindness, ptosis, disturbances in ocular mobility, papilledema, recurrent headaches, and nasal obstruction. 8 Aggressive growth occurs in some but not all cases. Such behavior may be related to younger patient age and the concurrent development of aneurysmal bone cysts, which is seen more frequently in PJOF. 3 Lawton et al report that tumor erodes bone partitions and encroaches on adjacent orbital, nasal, and cranial compartments, distorting the face, displacing orbital contents, and blocking normal sinus drainage to form mucoceles. 1,7 Visual disturbances or blindness can occur from compression of the optic nerves and orbit. 14 Intracranial extension develops slowly with few neurological signs or symptoms, even though the lesions may be large enough to fill the anterior cranial fossa 7. The dura maintains an effective barrier to invasion of brain. Occasionally, patients will present with meningitis caused by communication between paranasal sinuses and subarachnoid space. 7 The radiographic appearance manifests as well-demarcated, unilocular or multilocular radiolucencies with a variable amount of radiopacity, usually manifesting as fine specks or as groundglass opacification. 2,13,15 It reveals an expansile well-circumscribed radiolucent or mixed radiolucent/radiopaque lesion surrounded by a thick bony wall. 16,17 PJOFs are classically described as circumscribed unilocular lesions that are often surrounded by an eggshell-thin rim of bone. 1,18 Aggressive forms tend to show more of an expansile growth pattern than the typical variety. However, they still tend to exhibit sharp demarcation from the normal adjacent bone and this occasionally reflected by an abrupt loss of the overlying cortex. 6 On gross inspection, PJOF appears as a yellow-white, homogeneous, and firm lobulated mass with gritty consistency. 2 Cystic spaces, although rare, can be present. 3 The most characteristic histologic feature of psammomatoid ossifying fibroma is the presence of numerous small, round ossicles or psammomatoid bodies that are embedded in a cellular fibrous stroma. 2-4,8,10 The ossicles are mineralized collagenous foci that vary from small, smoothly contoured round-to-oval patterns to larger, irregularly shaped ossicle-like patterns with concentric layering similar to that of psammoma bodies. 2-4,19,20 The ossicles are identified within the bony trabeculae as well as within the adjacent cellular stroma. A prominent marginal osteoid rim surrounds the ossicles. 3 The number of ossicles varies from only a few to a dense population of innumerable spherical bodies. DISCUSSION Juvenile ossifying fibroma has been considered as a distinct disease entity from conventional ossifying fibroma and the other fibroosseous lesions because of its tendency to occur at a young age and its locally aggressive behavior. Moreover, JOF may clinically manifest with rapid painless expansion of the affected bone as an aggressive lesion mimicking malignancy such as osteosarcoma. So, it is important to accurately recognize JOF for making the diagnosis and managing this disease. The age and site of PJOF in our case are consistent with other reports, which state it as being common in the second decade of life and common in the paranasal sinuses. The literature reports a slight male predominance, ours being a female patient. The clinical S56 features in our case were slightly different from the literature as the patient did not give a history of nasal obstruction or a feeling of heaviness in the sinus region. Radiographic and histological features are also similar to the reported cases. PJOF needs to be distinguished from malignant bone tumors; in that there is a similarity of clinical manifestations, but it can be easily excluded from malignant bone tumors on histological examination. The differential diagnosis in conjunction with radiographic findings in this patient initially included aneurysmal bone cyst, central giant cell granuloma, osteogenic sarcoma, and progressive monostotic fibrous dysplasia and nonodontogenic primary tumors of bone, such as osteoblastoma. In addition, less likely consideration included locally aggressive odontogenic lesions, such as ameloblastic fibro-odontoma, as well as other entities consisting of calcifying odontogenic cyst (Gorlin cyst), adenomatoid odontogenic tumor, and primordial cysts (keratocyst). Vascular tumors can also have rapid growth and should be considered in the differential diagnosis. For example, central hemangioma grows rapidly and commonly presents as a radiolucent mandibular mass in children and young adults. Arteriovenous malformations also exhibit rapid growth, but usually display thrills or bruits on examination. 2 It is important to distinguish PJOF from central cementifying fibroma (ossifying fibroma, cementifying fibroma, cementoossifying fibroma), which is a benign jaw lesion of odontogenic origin arising in the molar and premolar regions of the maxilla and, most commonly, the mandible. 2,3 Patients with central cementifying fibroma are in the third or fourth decade of life with a distinctly female predilection. 3,8,16 The main presenting symptom is painless swelling of the involved bone. Radiographically, central cementifying fibroma is a well-marginated, unilocular, radiolucent or variably opacified lesion. 8 Microscopically, central cementifying fibromas consist of fibrous stroma with dense cellularity and small, spherical calcifications cementicles (osseous tissue related to dental cementum). Unlike PJOF, central cementifying fibromas rarely recur after removal. 2,8 Treatment of PJOF has been controversial. Complete removal by enucleation alone or with curettage as well as en block resections are common therapeutic options. Mass, as much as possible, must be removed while protecting adjacent vital structures to prevent recurrences. Our case required curetting of adjacent normalappearing surrounding tissue to ensure total excision of the mass. The reported recurrence rate ranges between 30 and 58%. 3,7,11,17 Radiotherapy has been proven ineffective and contraindicated due to an increased incidence of malignant transformation ranging from 0.4 to 40%. Despite the aggressive behavior, no metastasis has been reported. 20 Therefore, conservative treatment of the jaw lesions by enucleation and curettage has been reported to be successful. CONCLUSION PJOF is a rapidly growing benign but aggressive neoplasm that is encountered most commonly in children and young adults. Also, it poses diagnostic challenges since its clinical features overlap with many commonly occurring bone neoplasms. Histologic criteria for accurate classification remain complicated and are often controversial, secondary to confusing terminology as well as JAYPEE

5 Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review overlapping morphologic features shared by several closely related entities. Consequently, accurate and reliable diagnosis of JPOF requires a multidisciplinary approach with close attention to pertinent clinical history, radiographic impression, and correlation with histomorphologic findings. The management of these lesions is directed at primarily surgical intervention and must consists of a complete resection, since partial removal is associated with the hazard of recurrence. REFERENCES 1. MacDonald DS, Jankowski. Fibro-osseous lesions of the face and jaws. Clinical Radiol 2004;59: Neville, Damm, Allen, Bouquot. Oral and maxillofacial pathology (2nd ed) Mofty ES. Psammomatoid trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod Mar 2002;93(3): Park S, Jae B, L Lee JH, Cho KJ. Juvenile Ossifying Fibroma: A Clinicopathologic study of 8 cases and comparison with craniofacial fibro-osseous lesions. The Kor J of Pathol 2007;41: Vandekerckhove K,Van-den AK,Van-den BC, Verstraete K, Meire F. Psammomatoid ossifying fibroma of the ethmoid. Bull Soc belge Ophtalmol 2003;287: Yonetsu K, Nakamura T. CT of calcifying jaw bone diseases. Am J Res 2001;177: Michael TL, Heiserman JE, Coons SW, Ragsdale BD, Spetzler RF. Juvenile active ossifying fibroma. J Neurosurg 1997;86: Liliya A, Mark F, Tabor H. Pathologic quiz case: A sino-orbital mass in a 13 years old adolescent girl. Arc of pathol and lab med 2003; 127(7): Alper S. Juvenile ossifying fibroma. Report of a case. Gazi medical journal 2001;12: Singh I, Ghimire A, Bhadani P, Nepal A, Maharjan M, Gauum D, et al. Proptosis in a young child. Indian journal of pediatrics 2006;73: Sun G, Chen X, Tang E, Li Z, Li J. Juvenile ossifying fibroma of the maxilla. Int J Oral Maxillofac Surg 2007;36: Kumar SD, Ashraf M, Sharma SC, Maheshwari V. Ossifying fibroma of nose in a 2 years old child. Int Jour of otolaryngol and head and neck surg 2004;56(1): Faizan A. Benign fibro-osseous diseases of the maxillofacial bones. Am J Clin Pathol 2002;118: Offiah C. Rapidly enlarging chin mass. The British J of Radiol 2005; 78: Waldrom CA. Fibro-osseous lesion of the Jaws. J Oral Maxillofac Surg 1993;51: Bruce MW, Tuyethoa NV, James GS, Craig BF, Glen DH, Dennis KH. Aggressive psammomatoid ossifying fibromas of the sinonasal region a clinicopathologic study of a distinct group of fibro-osseous lesions. Cancer 1995;76(7): Noeke CEE. Juvenile ossifying fibroma of the mandible. An 8 years radiological follow-up. DMFR 1998;27: Scholl RJ, Kellett HM, Neumann DP, Lurie AG. Cysts and cystic lesions of the mandible: Clinical and radiologic-histopathologic review radiographics 1999;19: Ross RD. Benign fibroosseous lesions and bone tumours of the jaws. Oral and maxillofacial pathology. Lecture Handouts & References/Foss2005/BFOL%20Lecture-Foss.pdf 20. Martin Hasselblatt, et al. Juvenile psammomatoid ossifying fibroma of the neurocranium. Report of four cases. J Neurosurg 2005;102: S57

Aggressive Juvenile Ossifying Fibroma of the Anterior Mandible

Case Report Aggressive Juvenile Ossifying Fibroma of the Anterior Mandible Dr.Ravikumar.R 1, Dr.Raghavendra.K 2, Dr.Santhosh Kumar 3 1 Senior Lecturer, 2 Reader Department of Oral Surgery, Sri Siddhartha