Mandible & maxilla

Benign odontogenic tumors

Cemento-ossifying fibroma / ossifying fibroma



Last author update: 27 November 2023
Last staff update: 26 March 2024

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PubMed Search: Cemento-ossifying fibroma / ossifying fibroma

Saira Javeed, M.B.B.S., M.Phil.
Saba Anjum, M.B.B.S.
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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 March 29th, 2024.
Definition / general
Essential features
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
Sites
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
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
Laboratory
  • No specific laboratory tests are available
Radiology description
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

Radiolucency in anterior mandible

Expansile lesion of mandible

Expansile lesion of mandible

Cortical destruction Cortical destruction

Cortical destruction

Root Divergence

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
Treatment
Clinical images

Images hosted on other servers:
Large solitary swelling obliterating buccal vestibule

Large solitary swelling obliterating buccal vestibule

Bony mass in maxilla

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

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

Well circumscribed solid tumor mass

Rounded to spherical variable sized cementum-like ossicles Rounded to spherical variable sized cementum-like ossicles

Rounded to spherical variable sized cementum-like ossicles

Benign fibro-osseous proliferation

Benign fibro-osseous proliferation


Rounded mineralized droplets

Rounded mineralized droplets

Fibro-osseous proliferation

Fibro-osseous proliferation

Woven Bone

Woven bone

Brush borders

Brush borders

Positive stains
Negative stains
Molecular / cytogenetics description
Molecular / cytogenetics images

Images hosted on other servers:
MDM2 amplification by FISH

MDM2 amplification by FISH

Missing Image

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
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?

  1. Benign
  2. Intermediate, locally aggressive
  3. Intermediate, rarely metastasizing
  4. 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).

Comment Here

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)?

  1. Cemento-ossifying fibroma
  2. Odontogenic keratocyst of jaw
  3. Ovarian fibromas
  4. 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.

Comment Here

Reference: Cemento-ossifying fibroma / ossifying fibroma
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