Juvenile Ossifying Fibroma

Juvenile Ossifying Fibroma

Article
Bone, Muscle, & Joint
Kids' Zone
+3
Contributed byKrish Tangella MD, MBAJul 10, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • JOF (Juvenile Ossifying Fibroma)
  • Juvenile Fibro-Osseous Lesion

What is Juvenile Ossifying Fibroma? (Definition/Background Information)

  • Juvenile Ossifying Fibroma (JOF) is rare, benign tumor of the craniofacial (skull and face) bones. It is considered a "fibro-osseous neoplasm" because it is characterized by an overgrowth of bone
  • Affected people generally experience a gradual or rapid, painless expansion of the affected bone or region
  • Other symptoms such as exophthalmos or nasal blockage can rarely be associated with the tumor depending on its exact location. In some cases, the condition can be particularly aggressive with rapid growth and significant facial disfigurement
  • Although the condition can affect people of all ages, it is most commonly diagnosed between the ages of 5 and 15 years
  • The exact underlying cause of Juvenile Ossifying Fibroma is currently unknown; however, most cases occur sporadically in people with no family history of the condition
  • Juvenile Ossifying Fibroma is usually treated with surgery. Because the recurrence rate of JOF ranges from 30% to 58%, continued follow-up is essential

(Source: Juvenile Ossifying Fibroma; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Juvenile Ossifying Fibroma can be classified into two subtypes, based on microscopic differences in the affected cell types:

  • Juvenile psammomatoid ossifying fibroma
  • Juvenile trabecular ossifying fibroma

Who gets Juvenile Ossifying Fibroma? (Age and Sex Distribution)

  • Juvenile Ossifying Fibroma is a rare disorder. The presentation of symptoms most often occurs between the age of 5 and 15 years
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Juvenile Ossifying Fibroma? (Predisposing Factors)

  • Currently, no risk factors have been clearly identified for Juvenile Ossifying Fibroma

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Juvenile Ossifying Fibroma? (Etiology)

  • The exact cause of Juvenile Ossifying Fibroma is currently not known
  • Most cases occur sporadically, with no family history of the condition

What are the Signs and Symptoms of Juvenile Ossifying Fibroma?

The signs and symptoms of Juvenile Ossifying Fibroma may vary among affected individuals in severity and location of tumor formation, and may include:

  • Lesion in the face or head
  • Nasal blockage due to bone growth

How is Juvenile Ossifying Fibroma Diagnosed?

Juvenile Ossifying Fibroma is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Juvenile Ossifying Fibroma?

The complications of Juvenile Ossifying Fibroma may include:

  • Complete obstruction of nostrils
  • Recurrent sinus infections
  • Obstruction of eyes causing blurred vision
  • Severe facial disfigurement
  • Weight loss

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Juvenile Ossifying Fibroma Treated?

Juvenile Ossifying Fibroma is generally treated with surgical excision of the lesion followed by frequent and regular monitoring.

How can Juvenile Ossifying Fibroma be Prevented?

  • The exact cause of Juvenile Ossifying Fibroma is not known at the present time, and no guidelines or methods are available for its prevention
  • Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as JOF
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended, since the condition is known to recur when the surgical excision is incomplete

What is the Prognosis of Juvenile Ossifying Fibroma? (Outcomes/Resolutions)

  • Juvenile Ossifying Fibroma is a benign condition, and the prognosis is considered to be excellent with complete surgical excision of the tumor 
  • However, the condition is known to recur in 30-58% of affected individuals, if the excision is incomplete. This may necessitate routine monitoring in affected individuals

Additional and Relevant Useful Information for Juvenile Ossifying Fibroma:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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