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Benign odontogenic tumors

Cemento-ossifying fibroma / ossifying fibroma

Authors: Saira Javeed, M.B.B.S., M.Phil., Saba Anjum, M.B.B.S., Arsalan Ahmed, M.D.

Editorial Board Member: Molly Housley Smith, D.M.D.

Deputy Editor-in-Chief: Kelly Magliocca, D.D.S., M.P.H.

Last author update: 27 November 2023

Last staff update: 26 March 2024

Copyright: 2003-2024, PathologyOutlines.com, Inc.

PubMed Search: Cemento-ossifying fibroma / ossifying fibroma

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Table of Contents

Cite this page: Javeed S, Anjum S, Ahmed A. Cemento-ossifying fibroma / ossifying fibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillaossifyingfibroma.html. Accessed April 23rd, 2024.

Definition / general

Benign fibro-osseous neoplasm (J Maxillofac Oral Surg 2021;20:240, Oral Dis 2017;23:440, Head Neck Pathol 2020;14:70, J Stomatol Oral Maxillofac Surg 2022;123:364)
Origin is controversial but is mostly odontogenic in origin and usually arises in jaw (J Maxillofac Oral Surg 2021;20:240, Head Neck Pathol 2022;16:248)
Characterized by production of bone and cementum-like calcifications in a fibrous stroma
Usually located in the mandible during third to fourth decade of life (Oral Dis 2017;23:440, Head Neck Pathol 2020;14:70, J Stomatol Oral Maxillofac Surg 2022;123:364)

Essential features

Cemento-ossifying fibroma (COF) is a fibro-osseous lesion characterized by varied patterns of bone formation in a fibroblastic stroma (Head Neck Pathol 2022;16:991)
Putatively benign lesion with very rare malignant transformation or metastasis (Head Neck Pathol 2022;16:991, Ear Nose Throat J 2023;102:24)
Cemento-ossifying fibroma is named because of its usual location in the tooth bearing region of the jaw

Terminology

Ossifying fibroma, conventional type; cemento-ossifying fibroma; ossifying fibroma; conventional ossifying fibroma; cementifying fibroma

ICD coding

ICD-O: 9274/0 - cemento-ossifying fibroma

Epidemiology

Rare lesion that occurs over a broad age range, with peak incidence in third to fourth decade of life (Oral Dis 2017;23:440, Head Neck Pathol 2020;14:70, J Stomatol Oral Maxillofac Surg 2022;123:364)
Has female predilection (F:M = 5:1)
Tumor is more prevalent in White people than Black people (Oral Dis 2017;23:440)

Sites

Mandible is most common, followed by the maxilla
Mandibular premolar and molar area have more predilection (Head Neck Pathol 2020;14:70)
Maxillary lesions tend to involve antrum and canine fossa
Cemento-ossifying fibroma can be classified according to site of origin as (Int J Surg Case Rep 2020;68:257)
- Soft tissue counterpart is referred to as peripheral cemento-ossifying fibroma (PCOF) (Int J Surg Case Rep 2020;68:257)
- Central cemento-ossifying fibroma (CCOF) arises from cells of periodontal ligament in the apical area (Int J Surg Case Rep 2020;68:257)

Pathophysiology

Multiple cemento-ossifying fibromas rarely show syndromic / inherited association and usually present at a younger age (Case Rep Dent 2023;2023:4664619)
Hyperparathyroidism jaw tumor syndrome (HPT JT): rare autosomal dominant disease caused by mutation of the tumor suppressor gene CDC73 that encodes for parafibromin resulting in premature truncation of parafibromin protein product; the syndrome predisposes to a triad occurrence: multiple maxillary or mandibular cemento-ossifying fibroma, parathyroid adenoma or carcinoma and renal and uterine tumors (Int J Surg Case Rep 2020;68:257)

Etiology

Origin of cemento-ossifying fibroma has been suggested as odontogenic or from periodontal ligament progenitor cells (J Maxillofac Oral Surg 2021;20:240)
Origin of nonodontogenic lesions of similar histology arising from craniofacial bone outside the jaw is unclear (Head Neck Pathol 2022;16:257)
Periodontal membrane has multipotent cells capable of forming cementum, lamellar bone and fibrous tissue (J Maxillofac Oral Surg 2021;20:240)
Lesion can present as a component of hyperparathyroidism jaw tumor syndrome; 25 - 50% of patients of this syndrome develop cemento-ossifying fibroma of the jaws with the mandible as a favored site (Case Rep Dent 2023;2023:4664619, J Dent Sci 2020;15:426)

Clinical features

Smaller lesions are asymptomatic
Larger / solitary lesions present as painless swelling that causes jaw expansion, facial asymmetry, tooth divergence (Heliyon 2021;7:e07594)
Grows slowly but can reach a considerable size if left untreated (Head Neck Pathol 2020;14:70)
Extension to the nasal septum, orbital floor and infraorbital foramen can be seen in larger lesions; rarely associated with the destruction of extraosseous soft tissue components (Heliyon 2021;7:e07594)
Juvenile variant of lesion occurring at a young age is clinically aggressive and highly recurrent (Heliyon 2021;7:e07594, J Maxillofac Oral Surg 2021;20:240)

Diagnosis

Diagnosis dependent on clinical, radiologic and pathologic correlation (J Maxillofac Oral Surg 2021;20:240)

Laboratory

No specific laboratory tests are available

Radiology description

Radiological findings vary according to maturity of lesion; early lesions show well defined corticated radiolucency and over time, lesion becomes progressively more radiopaque (Heliyon 2021;7:e07594, BMJ Case Rep 2020;13:e239286)
Well circumscribed, unilocular lesion with centrifugal pattern of growth (Heliyon 2021;7:e07594)
Can present as lesion with well defined sclerotic borders and is predominantly unilocular (BMJ Case Rep 2020;13:e239286)
Larger lesions show expansion, thinning and perforation of buccal and lingual cortex and involvement of the lower border of mandible (BMJ Case Rep 2020;13:e239286)

Radiology images

Contributed by Molly Housley Smith, D.M.D., Thomas W. Fonner, D.M.D., Carlton Taylor, D.M.D., Firas Katabi, D.D.S. and Nish Shah, D.M.D., M.D.

Radiolucency in anterior mandible

Expansile lesion of mandible

Cortical destruction

Root divergence

Prognostic factors

Slow growing, benign neoplasm with no recurrence in majority of cases
Malignant transformation has been reported in a single case (Ear Nose Throat J 2023;102:24)

Case reports

19 year old woman with a large maxillary cemento-ossifying fibroma superimposed with solitary bone cyst (Int J Surg Case Rep 2020;68:257)
30 year old man with multiple cemento-ossifying fibromas (Case Rep Dent 2023;2023:4664619)
45 year old man with large cemento-ossifying fibroma of the mandible involving the infratemporal and parapharyngeal spaces (Heliyon 2021;7:e07594)

Treatment

Treated by enucleation and curettage as it shells out easily and recurs rarely (J Maxillofac Oral Surg 2021;20:240)
Untreated tumors can attain a massive size and rarely require en bloc resection (J Maxillofac Oral Surg 2021;20:240)

Clinical images

Images hosted on other servers:

Large solitary swelling obliterating buccal vestibule

Bony mass in maxilla

Intraoperative exposure of COF showing clear distinction between the lesion and healthy bone

Distinction between lesion and healthy bone

Gross description

Well circumscribed tumor that can be shelled out easily in one piece

Gross images

Images hosted on other servers:

Excision

Microscopic (histologic) description

Well defined lesion; may have thin fibrous capsule
Well demarcated margin from surrounding normal bone
Lesion consists of variable proportion of fibrous and mineralized tissue, more heavily mineralized centrally; it shows variation in the amount and type of mineralization, even within a single lesion
Osteoblastic rimming of bone trabeculae is frequent (J Stomatol Oral Maxillofac Surg 2022;123:364)
Stroma is fibroblastic with areas of hypercellularity and nuclear hyperchromasia
No significant atypia and mitoses are infrequent (Head Neck Pathol 2020;14:70)
Woven to lamellar bone, osteoid and dense acellular or paucicellular basophilic rounded cementum-like calcifications may all be present (Head Neck Pathol 2020;14:70)
Bony trabeculae may form thick anastomosing strands or fuse into large sheets centrally
Hemorrhagic cystic degeneration, resembling aneurysmal bone cyst formation, is more often found in juvenile active ossifying fibromas but has been reported in rare COFs (J Stomatol Oral Maxillofac Surg 2022;123:364, Head Neck Pathol 2022;16:248)
Areas resembling cemento-ossifying fibroma may be seen in psammomatoid and trabecular ossifying fibroma; cemento-ossifying fibroma may contain ossicles like psammomatoid ossifying fibroma
Cemento-ossifying fibroma with syndromic association has histology similar to that of nonsyndromic cemento-ossifying fibroma
Cemento-ossifying fibroma in gnathodiaphyseal dysplasia is at the more fibrous end of spectrum with predominantly basophilic rounded islands and droplets of acellular bone (Head Neck Pathol 2014;8:432)

Microscopic (histologic) images

Contributed by Saira Javeed, M.B.B.S., M.Phil., Kelly Magliocca, D.D.S., M.P.H. and Molly Housley Smith, D.M.D.

Well circumscribed solid tumor mass

Rounded to spherical variable sized cementum-like ossicles

Benign fibro-osseous proliferation

Rounded mineralized droplets

Fibro-osseous proliferation

Woven bone

Brush borders

Positive stains

Cemento-ossifying fibromas showed significant immune reactivity for keratan sulphate (IHC is not necessary for diagnosis) (J Maxillofac Oral Surg 2021;20:240)

Negative stains

p53 (J Clin Exp Dent 2022;14:e27)
MDM2 (25%) and CDK4 (2%) can be seen in a subset of cases but is typically focal (≤ 10% of nuclear staining) (Head Neck Pathol 2022;16:991)

Molecular / cytogenetics description

None for sporadic cemento-ossifying fibroma
Syndromic cemento-ossifying fibroma can be identified clinically or by genetic mutations in CDC73 (also known as HRPT2) (J Dent Sci 2020;15:426, Case Rep Dent 2023;2023:4664619)
Multiple ossifying fibromas can be associated with hyperparathyroidism jaw tumor syndrome (Case Rep Dent 2023;2023:4664619, J Dent Sci 2020;15:426)
Amplification of MDM2 gene by fluorescence in situ hybridization is rarely (3%) seen in ossifying fibroma (Head Neck Pathol 2022;16:991)
Positive MDM2 amplification in craniofacial fibro-osseous lesion does not completely exclude ossifying fibroma; however, it should still raise concern for low grade osteosarcoma because this genetic event is much more common in the latter (Head Neck Pathol 2022;16:991)

Molecular / cytogenetics images

Images hosted on other servers:

MDM2 amplification by FISH

CDC73 mutations in sporadic tumors

Sample pathology report

Mandible, enucleation and curettage:
- Benign fibro-osseous neoplasm consistent with cemento-ossifying fibroma / ossifying fibroma (see comment)
- Comment: This is a benign fibro-osseous neoplasm that requires clinical and radiological correlation.

Differential diagnosis

Peripheral ossifying fibroma (Int J Health Sci (Qassim) 2019;13:63, Med Oral Patol Oral Cir Bucal 2022;27:e460):
- Reactive, inflammatory, hyperplastic growth of the gingiva
- It is the soft tissue counterpart to COF; microscopically, it is lined by mucosa and can have identical microscopic appearance
Cemento-osseous dysplasia:
- Nonneoplastic fibro-osseous lesion of tooth bearing regions
- Most common benign fibro-osseous lesion of jaws, affects middle aged women
- Patients can be managed with follow up and symptomatic treatment
- Radiology: radiolucent, radiodense or mixed, as the lesion matures becomes more radiodense and calcified
- Grossly usually received in multiple gritty fragments with tan brown color
- Microscopically identical to COF
  - Unencapsulated with variable cellular fibrous stroma and areas of loose collagen
  - Stroma can display osteoid, bone and cementum-like material
  - Mature lesion shows more calcification
- Clinical and radiographic correlation is essential to differentiate the two, especially on incisional biopsies
- An expansile variant of cemento-osseous dysplasia exists and can mimic COFs
Chronic osteomyelitis (Head Neck Pathol 2020;14:842):
- Inflammatory process with a history of trauma and sinus formation
- No age or gender preference
- Treatment includes surgery to remove portions of bone that are infected or dead, followed by intravenous antibiotics given in the hospital
- Microscopically: findings are nonspecific, such as chronic inflammatory cell infiltration, bone marrow fibrosis and sclerotic bone formation
Desmoplastic fibroma:
- Locally aggressive fibroblastic lesion of bone
- Mostly affects mandible in people younger than 30 (mean: 16 years), no gender predilection
- Excision, recurrence is common
- Radiology: well defined without mineralization
- Grossly: firm, tan-white with rough cut surface, myxoid areas can be seen
- Microscopically
  - Infiltrative lesion comprising fascicles of uniform myofibroblasts / fibroblasts having tapering nuclei in a collagenous to myxoid stroma
  - Perivascular edema and mitotic figures are present
  - Overt bony trabeculae or droplets absent
- IHC: alpha smooth muscle actin (ASMA) and beta catenin positive in some cases
- Associated with activating CTNNB1 gene / APC mutation
Fibrous dysplasia:
- Skeletal anomaly, a disorder of growing bones
- Monostotic or polyostotic lesion, affects craniofacial bones and femur in children and adolescents, no gender predilection
- Lesions stabilize with skeletal maturation, surgical intervention in younger patients is delayed
- Radiology: radiolucent to sclerotic, ground glass appearance with indistinct borders
- Grossly: affected bone is rubbery, compressible with gritty texture
- Microscopically
  - Has bland hypocellular appearance if the lesion is mature; can be hypercellular when the lesion is in actively growing phase
  - Lesion fuses with adjacent normal bone
  - Irregularly shaped trabeculae of bone in a background fibrous stroma
  - Trabeculae of woven bone are elongated and curvilinear, described as Chinese characters or letters in alphabet soup
  - Osteoblastic rimming absent or minimal
  - Retraction artifact / peritrabecular clefting often present
- Associated with GNAS gene mutation and McCune-Albright syndrome
Intraosseous meningioma (Surg Neurol Int 2021;12:485):
- Most common benign brain / intracranial tumors, however, intraosseous meningiomas are rare lesions accounting for < 1% of intracranial meningiomas
- Common locations are frontoparietal and orbital regions
- Surgical removal though challenging is curative
- Radiology: depending upon location, usually circumscribed with surrounding bone shell
- Microscopically: morphology is similar to dura based lesions
Osteoblastoma:
- Benign bone forming tumor
- Rare lesion, mostly affects craniofacial bones specially mandible in people in second to third decade with slight male predominance
- Can be excised however recurrences are reported after incomplete removal
- Radiology: circumscribed rounded of usually > 20 mm in size, radiolucent to radiopaque, sclerotic border; can mimic malignancy
- Grossly: multiple firm tan-white fragments
- Microscopically
  - Lesion is composed of haphazard mineralized trabeculae of bone showing prominent osteoblastic rimming, lying in cellular fibrous stroma
  - Rare mitotic figures
Osteosarcoma (J Cancer Res Ther 2018;14:471):
- Malignant bone tumor
- Greatest predilection for the metaphysis (femur and tibia), osteosarcoma of craniofacial bones is infrequent
- Osteosarcoma of the jaws are rare and comprise ~7% of all osteosarcoma cases
- Radiology: irregular mixed radiolucent radiopaque pattern with infiltrative margins
- Microscopically: displays features commonly found in various types of malignant neoplasms
Reference: J Maxillofac Oral Surg 2021;20:240

Additional references

Neville: Oral and Maxillofacial Pathology, 5th Edition, 2022, WHO Classification of Tumours Editorial Board: Head and Neck Tumours, 5th Edition, 2024

Board review style question #1

The histologic pattern shown above is from a 32 year old woman who has had small swelling in the mandible for the last 2 weeks. On radiology, the lesion was found to be well circumscribed. What is the most expected behavior of this lesion?

Benign
Intermediate, locally aggressive
Intermediate, rarely metastasizing
Malignant

Board review style answer #1

A. Benign. This is a slow growing, benign neoplasm with no recurrence in most cases (J Maxillofac Oral Surg 2021;20:240, Oral Dis 2017;23:440, Head Neck Pathol 2020;14:70, J Stomatol Oral Maxillofac Surg 2022;123:364). Answers B, C and D are incorrect because this neoplasm rarely demonstrates malignant transformation or metastasis (Head Neck Pathol 2022;16:991, Ear Nose Throat J 2023;102:24).

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Reference: Cemento-ossifying fibroma / ossifying fibroma

Board review style question #2

Which of the following conditions is typically associated with hyperparathyroidism jaw tumor syndrome (HPT JT)?

Cemento-ossifying fibroma
Odontogenic keratocyst of jaw
Ovarian fibromas
Pits in palms and soles

Board review style answer #2

A. Cemento-ossifying fibroma is associated with hyperparathyroidism jaw tumor syndrome. The syndrome predisposes to a triad occurrence: multiple maxillary or mandibular cemento-ossifying fibroma, parathyroid adenoma or carcinoma and renal and uterine tumors (Int J Surg Case Rep 2020;68:257). Answers B, C and D are incorrect because they are usually associated with basal cell nevus syndrome.

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Reference: Cemento-ossifying fibroma / ossifying fibroma

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