Nonossifying fibroma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rohan A. Bhimani, M.B.B.S., D.N.B., M.Ch.[2]

Synonyms and keywords: Fibroxanthoma; Fibrous cortical defect

Overview

Historical Perspective

In 1929, Phemister first described the term non ossifying fibroma.^[1]^[2]
In 1941, Sontag and Pyle reported a radiologic description of non ossifying fibroma.
In 1942, Jaffe and Lichtenstein described clinical findings, anatomic aspects and the natural history.

Classification

Non ossifying fibroma can be classified based on imaging findings.

Enneking (MSTS) Staging System

The Enneking surgical staging system (also known as the MSTS system) for benign musculoskeletal tumors based on radiographic characteristics of the tumor host margin.^[3]
It is widely accepted and routinely used classification.

Stages	Description

1	Latent: Well demarcated borders
2	Active: Indistinct borders
3	Aggressive: Indistinct borders

Pathophysiology

The exact pathogenesis of non ossifying fibroma is not fully understood.
Various theories have been proposed concerning the pathogenesis of non ossifying fibroma:^[4]^[5]
- An abnormal development extending from the growth plate.
- An abnormal osteoclastic resorption at the subperiosteal level during remodeling of the metaphysis.
Non ossifying fibroma (NOF) typically occur in the metaphysis of the long bones.^[6]
The bones often involved are femur, tibia, and fibula.^[7]
Conditions associated:
- Jaffe-Campanacci syndrome
  - Congenital syndrome of multiple non-ossifying fibromas and cafe au lait pigmentation, mental retardation, heart, eyes, gonads involved
- Neurofibromatosis
- Familial multifocal NOF
- Aneurysmal bone cyst

Causes

There are no established causes for non ossifying fibroma.^[8]

Epidemiology and Demographics

The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.

OR

In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.

OR

In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.

Patients of all age groups may develop [disease name].

OR

The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.

OR

[Disease name] commonly affects individuals younger than/older than [number of years] years of age.

OR

[Chronic disease name] is usually first diagnosed among [age group].

OR

[Acute disease name] commonly affects [age group].

There is no racial predilection to [disease name].

OR

[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].

[Disease name] affects men and women equally.

OR

[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.

The majority of [disease name] cases are reported in [geographical region].

OR

[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].

Risk Factors

There are no established risk factors for non ossifying fibroma.

Screening

There is insufficient evidence to recommend routine screening for non ossifying fibroma.

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Diagnosis

Diagnostic Study of Choice

X-ray is the diagnostic study of choice for the diagnosis of non ossifying fibroma.
X-ray findings include:^[9]^[10]^[11]
- Metaphyseal eccentric "bubbly" lytic lesion surrounded by sclerotic rim
- Cortex may be expanded and thin
- As bone grows, it migrates to diaphysis and the lesions enlarge.
- Lesions become sclerotic as patient approaches skeletal maturity.

History and Symptoms

The majority of patients with non ossifying fibroma are asymptomatic.^[12]
Some patients with non ossifying fibroma have a positive history of:
- Pain
- Swelling
- Pathological fracture

Physical Examination

Patients with non ossifying fibroma usually appear well.
Common physical examination findings of non ossifying fibroma include:^[13]^[14]^[15]
- Asymptomatic
- Pain if associated with pathological fracture
Findings associated with Jaffe-Campanacci syndrome include:^[16]
- Café-au-lait spots
- Multiple nevi
- Hypogonadism
- Ocular abnormalities

Laboratory Findings

There are no diagnostic laboratory findings associated with non ossifying fibroma.

Electrocardiogram

There are no ECG findings associated with non ossifying fibroma.

X-ray

Three views of affected bone or joint are recommended.
X-ray findings include:^[17]^[18]^[19]
- Metaphyseal eccentric "bubbly" lytic lesion surrounded by sclerotic rim
- Cortex may be expanded and thin
- As bone grows, it migrates to diaphysis and the lesions enlarge.
- Lesions become sclerotic as patient approaches skeletal maturity.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with non ossifying fibroma.

CT scan

Computed tomography (CT) is useful in predicting the fracture risk.^[20]^[21]^[22]
CT scans may depict a central lucency.
CT may confirm a minimally displaced fracture.
It may also be helpful in preoperative planning in unusual locations such as the femoral neck.

MRI

Magnetic resonance imaging (MRI) are similar to those from CT scan.^[23]
MRI demonstrates a characteristic low signal on both T1 and T2 sequences without enhancement.
High signal on T2 can be seen with an associated stress fracture.

Other Imaging Findings

There are no other imaging findings associated with non ossifying fibroma.

Other Diagnostic Studies

Biopsy

Biopsy finding of non ossifying fibroma includes:^[24]
- Bland fibroblastic component with a few histiocytes, myofibroblast cells, and giant cells.
- Marked proliferations of spindle cells arranged in a storiform pattern.
- Hemosiderin deposits also are found.
- Some leukocyte infiltration may be present.

Treatment

Medical Therapy

Observation is the mainstay of treatment for non ossifying fibroma.^[25]

Observation

Indications

First line of treatment
Most lesions resolve spontaneously and progressively reossify as child enters 2nd and 3rd decade of life

Technique

Radiographs at 6, 12months, then annually until reossified

Casting

Indication

Pathologic fracture especially in the pediatric population.

Surgery