Ossifying Fibroma: Symptoms, Types, Causes and Treatment

Ossifying fibroma is a rare, benign bone tumor that primarily affects the craniofacial skeleton, especially the jaws. While it is generally considered non-cancerous, ossifying fibroma can behave aggressively, causing significant facial deformity and functional impairment if left untreated. Understanding its symptoms, types, causes, and treatment options is crucial for early diagnosis and effective management. This comprehensive guide synthesizes the latest evidence from clinical studies and reviews to offer a clear and practical overview for patients, caregivers, and healthcare professionals.

Symptoms of Ossifying Fibroma

Ossifying fibroma can be insidious in onset, often presenting with few or no symptoms in its early stages. As the lesion grows, however, it can lead to visible and sometimes painful changes, particularly in the jaw or facial bones.

Symptom	Description	Prevalence/Pattern	Source(s)
Swelling	Painless, slow-growing mass	Most common initial symptom	1 4 5 10
Facial Asymmetry	Noticeable unevenness or deformity	Especially in large lesions	1 4 10
Tooth Displacement	Shifting or loosening of teeth	Can affect bite or occlusion	1 4 10
Jaw Deformity	Bony expansion or contour change	More common in mandible	1 4 10
Root Resorption	Resorption of tooth roots	Less frequent	10

Table 1: Key Symptoms

Painless Swelling and Bony Expansion

One of the hallmark features of ossifying fibroma is a painless swelling in the jaw or facial bone. Most patients initially notice a slowly enlarging mass, which may go unrecognized until it causes visible facial asymmetry or discomfort. The mandible is more frequently affected than the maxilla, although both sites can be involved 4 10.

Facial Changes and Tooth Displacement

As the lesion increases in size, it can cause pronounced facial asymmetry and deformity. This is particularly distressing in younger patients, where rapid growth can alter normal facial development. Tooth displacement is another common sign, as the expanding mass pushes adjacent teeth out of alignment, leading to an abnormal bite or malocclusion 1 4 10.

Less Common Symptoms

In rare cases, ossifying fibroma can cause tooth root resorption, numbness, or pain, especially if the lesion compresses nearby nerves or becomes secondarily infected. Most cases, however, are painless until the lesion becomes quite large 10.

Radiological Findings

Radiographic examination often reveals a well-circumscribed lesion with mixed radiolucent and radiopaque areas, reflecting the presence of both fibrous tissue and calcified material. Over 50% of lesions exhibit jaw expansion, and the majority have well-defined, unilocular borders 4 10.

Types of Ossifying Fibroma

Not all ossifying fibromas are the same. The condition encompasses several subtypes, each with distinct clinical and histological characteristics. Understanding these differences aids in diagnosis and guides treatment strategies.

Type	Typical Age/Sex	Location	Aggressiveness	Source(s)
Cemento-ossifying Fibroma (COF)	Adults, F>M	Mandible	Slow-growing	3 5
Juvenile Trabecular OF (JTOF)	Children, M>F	Jaws (maxilla)	Aggressive	1 2 5 6
Juvenile Psammomatoid OF (JPOF)	Wide age range	Sinonasal/orbital	Aggressive	2 5 8

Table 2: Ossifying Fibroma Types

Cemento-ossifying Fibroma (COF)

COF is the most common variant, typically affecting women in their 20s and 30s. It usually presents as a slow-growing, well-circumscribed lesion in the mandible. The clinical course is often indolent, and these lesions are less likely to recur after conservative surgical removal 3 5 10.

Juvenile Ossifying Fibroma (JOF): Trabecular vs. Psammomatoid

Juvenile Trabecular Ossifying Fibroma (JTOF):

• Occurs mainly in children and adolescents, with a slight male predominance.
• Primarily affects the jaws, especially the maxilla.
• Characterized by aggressive growth and a high recurrence rate, making management challenging 1 2 5 6.

Juvenile Psammomatoid Ossifying Fibroma (JPOF):

• Can affect a broad age range (from infants to the elderly).
• More commonly found in the sinonasal and orbital bones, rather than the jaws.
• Also demonstrates aggressive behavior and a significant risk of recurrence 2 5 8.

Overlap and Diagnostic Challenges

Many ossifying fibromas exhibit overlapping histological features, and up to half of cases may show a mix of trabecular and psammomatoid patterns, making strict categorization difficult. Accurate diagnosis, therefore, depends on a combination of clinical, radiological, and histological findings 3.

Causes of Ossifying Fibroma

While the precise causes of ossifying fibroma are not fully understood, research has identified several contributing factors. An interplay between genetic predisposition and the local tissue environment seems to play a pivotal role.

Cause/Factor	Description	Evidence/Notes	Source(s)
Periodontal Origin	Arises from periodontal ligament	Explains jaw predilection	4
Genetic Mutations	CDC73/HRPT2 gene abnormalities	Associated with some cases	7
Syndromic Association	Hyperparathyroidism-jaw tumor syndrome	Rare familial cases	7
Unknown/Idiopathic	No clear cause in most cases	Sporadic majority	3 10

Table 3: Causes and Risk Factors

Periodontal Ligament Origin

Most ossifying fibromas are thought to originate from the periodontal ligament—the connective tissue that surrounds the tooth roots. This theory is supported by the tumor's close association with tooth-bearing areas of the jaws 4.

Genetic and Syndromic Associations

A subset of ossifying fibromas, especially those occurring as part of familial syndromes, have been linked to inactivating mutations in the CDC73 (also known as HRPT2) gene. This genetic defect is most famously associated with hyperparathyroidism-jaw tumor syndrome (HPT-JT), a rare inherited condition that predisposes individuals to both parathyroid tumors and ossifying fibromas of the jaw 7. Some sporadic, non-syndromic cases have also shown CDC73 mutations or abnormal expression of the parafibromin protein, suggesting a possible genetic role even outside familial syndromes 7.

Idiopathic Cases

Despite these associations, the majority of ossifying fibroma cases occur sporadically, with no identifiable cause or family history. Environmental factors, trauma, or local irritation have been suggested, but there is little concrete evidence to support these as significant contributors 3 10.

Treatment of Ossifying Fibroma

Treatment decisions for ossifying fibroma must be individualized, taking into account lesion size, location, patient age, and the risk of recurrence. The primary goal is complete excision of the tumor while minimizing functional and cosmetic impairment.

Treatment	Indication	Recurrence Risk	Source(s)
Enucleation/Curettage	Small, non-aggressive lesions	Higher (esp. JOF)	5 6 8 9
Peripheral Osteotomy	Adjunct to curettage	Reduced recurrence	5 8
Radical Resection	Large, aggressive, recurrent cases	Lowest recurrence	5 6 8
Regular Follow-up	All cases	Early detection	1 5 8

Table 4: Treatment Approaches and Outcomes

Conservative Surgery: Enucleation and Curettage

For most small and well-circumscribed ossifying fibromas, especially the cemento-ossifying subtype, enucleation (surgical removal of the lesion) and curettage (scraping of the cavity) are effective options. However, this approach carries a higher risk of recurrence in the more aggressive juvenile variants, particularly JTOF and JPOF 5 6 8.

Adjunctive Measures: Peripheral Osteotomy

Adding a peripheral osteotomy—that is, removing a thin margin of surrounding bone—significantly reduces recurrence rates in juvenile ossifying fibromas. This measure is often recommended after enucleation to help ensure complete removal of microscopic tumor cells 5 8.

Radical Resection

In cases of large, rapidly growing, or recurrent lesions, especially in young patients where preserving facial structure is critical, radical resection (removal of the tumor with a wide margin of healthy tissue) may be necessary. Although this approach offers the lowest chance of recurrence, it carries a higher risk of disfigurement and functional impairment. For this reason, radical surgery is usually reserved for the most aggressive or unmanageable cases 1 5 6 8.

Recurrence and Follow-up

Ossifying fibroma—particularly the juvenile forms—has a notable risk of recurrence, especially after conservative surgery. Long-term, regular follow-up with clinical and radiological assessment is essential to catch and treat any recurrence early 1 5 8.

Treatment Decision Factors

• Lesion size, location, and aggressiveness: Larger or more aggressive tumors require more extensive surgery 5 6 8 10.
• Patient age and growth potential: In children, preserving normal development is a priority 1 5.
• Morbidity and cosmetic concerns: Surgical options must balance recurrence risk against the potential for facial disfigurement 1 5 8.

Conclusion

Ossifying fibroma is a rare but important benign tumor of the craniofacial skeleton, notable for its variable presentation, subtypes, and treatment challenges. Early recognition and appropriately tailored management are crucial to minimize morbidity and maximize functional and cosmetic outcomes.

Key Points:

• Ossifying fibroma often presents as a painless swelling with facial asymmetry, jaw deformity, and tooth displacement 1 4 5 10.
• There are several subtypes, including cemento-ossifying fibroma (adults) and the more aggressive juvenile variants (JTOF and JPOF) 2 3 5.
• The exact cause is unclear, but some cases are linked to genetic mutations and syndromic associations 4 7.
• Treatment ranges from conservative surgery to radical resection, with the choice guided by lesion behavior and recurrence risk 5 6 8.
• Juvenile forms, especially JTOF and JPOF, have a higher recurrence risk and require diligent follow-up 1 5 8.
• Multidisciplinary care and patient-specific decision-making are vital for optimal outcomes.

With ongoing research and improved surgical techniques, the outlook for patients with ossifying fibroma continues to improve. Early diagnosis and expert management remain the cornerstones of successful treatment.