Years published: 2023
NORD gratefully acknowledges Kevin McMahon and Gregory Cichon, MD Candidates at Creighton School of Medicine and Nagamani Narayana, DMD, MS, Professor of Dentistry at University of Nebraska Medical Center for the preparation of this report.
Juvenile ossifying fibroma is a rare and non-cancerous overgrowth of bone in the face or jaw. There are two subtypes of juvenile ossifying fibroma called psammomatoid juvenile ossifying fibroma (PsJOF) and trabecular juvenile ossifying fibroma (TrJOF). Both subtypes primarily affect the bones of the face. PsJOF predominantly affects the bones of the eye and nose and mostly manifests in patients aged 16-33 years. TrJOF predominantly affects the bones of the upper and lower jaw and most commonly affects patients around the age of 8 years. Both forms affect males and females equally.
JOF is considered a “fibro-osseous lesion” as it is characterized by overgrowth of bone. Both forms of JOF present with slow or rapid, mostly painless, progressive bony overgrowth of the face or jaw. Symptoms may include nasal congestion/ obstruction, eye bulging or double vision. Regardless of type, surgical removal of affected bone is the primary method of treatment. Depending on the amount of bone removed, additional reconstructive or cosmetic surgeries may be required. Due to the high recurrence rate of JOF, continued post-surgical follow-up is essential. The exact cause of JOF is currently unknown and most cases occur sporadically in patients without any family history of the condition.
JOF and its subtypes are diagnosed based on the age of the patient affected and biopsy of the bony overgrowth and its location. JOF is not cancerous but may damage surrounding structures or cause significant disfigurement. Patients are often asymptomatic but due to the progressive nature of the lesion, early diagnosis allows for resection of a smaller lesion and less chance of damage to nearby structures.
Most patients with JOF are asymptomatic and have lesions identified incidentally through unrelated imaging such as dental x-rays.
As the fibroma expands either slowly or rapidly, patients may experience non-specific symptoms such as sinus infections (sinusitis), runny nose (rhinorrhea), nosebleeds (epistaxis), nasal obstruction, facial enlargement/changing shape, visual changes, blindness, eyelid drooping (ptosis), bulging of the eye(s) (proptosis/exophthalmos), pain/difficulty with eye movement or papilledema (optic disc bulging) depending on the bones affected. Pain and numbness are often absent but do rarely occur.
The exact cause of JOF is currently unknown, and most cases occur sporadically without any family history of the condition. Some studies suggest that JOF and aggressive behavior in JOF may be driven by a gene change (pathogenic variant or mutation) in a gene on chromosome 12, leading to amplification of genes such as MDM2 and RASAL1. Other studies on PsJOF have identified mutations at nonrandom chromosome breakpoints on the X chromosome at Xq26 and 2q33 leading to translocation of genes between the X chromosome and chromosome 2.
Juvenile ossifying fibroma mostly affects children and adolescents. Patients are generally younger than 15 with an average age of 8-12 years. Both males and females seem to be equally affected. Only 20% of patients are older than 15 at the age of onset.
For TrJOF, which mostly affects the bones of the upper and lower jaw (maxilla > mandible), patients on average are around 8 years old with a range of 2-12 years of age at onset.
For PsJOF, which mostly affects the bones of the eyes or nose (periorbital, ethmoid sinus bones, and frontal sinus bones), patients on average are 16-33 years old with a range of 3-72 years of age at onset.
A diagnosis of JOF may be suspected after imaging for another purpose, such as dental x-rays. The next step is referral to a specialist such as an ear-nose-and throat doctor (ENT) or maxillofacial surgeon who collects samples (biopsies) of the affected bone and may perform additional imaging tests. A pathology laboratory can identify the specific features of the fibroma biopsy sample under a microscope.
Fibromas of each type have a different appearance on imaging, under the microscope, and during surgery that help doctors properly diagnose each type.
Juvenile ossifying fibroma is curable through surgery but does have a high tendency to recur after treatment.
After identification, the patient is contacted and scheduled for surgery to remove the affected bone. If the fibroma is large enough, the patient may have additional surgeries for cosmetic purposes or removal or additional affected bone. Finally, patients are scheduled for regular follow-up due to the high risk of the fibroma recurring. Overall, patients generally live normal lives.
Both forms of juvenile ossifying fibromas are benign tumors that may be rapidly or slowly growing; thus both are treated exclusively with aggressive surgery and not with chemotherapy or radiation. JOF tumors are always progressive and will continue to grow without surgical removal. Early recognition of tumors when they are small and/or not invading local structures allows for surgical removal of a smaller area of bone. Tumors that are larger and with greater spread require more significant surgical excisions that remove larger areas of bone and tissue and may require cosmetic surgery to replace bone. Although the fibroma may return after surgery, there have been no reports of fibromas becoming cancerous and spreading to other parts of the body. Smaller tumors may be treated with curettage (scraping away of affected bone), moderate tumors may be treated with surgical excision (cutting away of affected bone) and large tumors may require en bloc resection (surgical removal of the entirety of a fibroma) or resection with reconstruction.
Fibroma recurrence after surgery is not uncommon, which means patients should follow up with their doctor consistently for screening. The more aggressive the original fibroma, the greater chance of it recurring after surgery. If a surgeon is unable to remove the entire fibroma or if the fibroma has grown into nearby structures, it is more likely to recur.
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