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

Historical Perspective

• In 1929, Phemister first described the term non ossifying fibroma.
• 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.
• 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:
  • 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.
• The bones often involved are femur, tibia, and fibula.
• 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.

Differentiating Non Ossifying Fibroma from Other Diseases

Non ossifying fibroma must be differentiated from following bone disorders:

Disease	Bubbly lytic lesion on x-ray	Lakes of Blood on histology	Diagnosis	Treatment is curretage and bone grafting
Non ossifying fibroma	+	-	Radiology and biopsy	-
Unicameral bone cyst	+	-	Radiology and biopsy	-
Aneurysmal bone cyst	+	+	Radiology and biopsy	+
Giant cell tumor	-	-	Radiology and Biopsy	+
Chondroblastoma	-	-	Biopsy	+
Chondromyxoid Fibroma	-	-	Radiology and biopsy	+
Osteoblastoma	-	-	Radiology and biopsy	+
Telangiectatic osteosarcoma	-	+	Radiology and biopsy	-

Epidemiology and Demographics

• The incidence of Non ossifying fibroma is approximately 1000-2000 per 100,000 individuals worldwide.
• Adolescents and children are most affected by non ossifying fibroma.
• Non ossifying fibromas are present in about 30 % of children.
• The age distribution of Non ossifying fibroma is between 5-15 years.
• Men are more commonly affected than women, with a 1.9:1 ratio.

There is no racial predilection to non ossifying fibroma.

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

• Common complications of aneurysmal bone cyst include:
  • Pathological fracture
  • Premature epiphyseal closure
    • Limb-length discrepancy
    • Angular deformity
  • Rare malignant transformations
• Prognosis is generally excellent for non ossifying fibroma.
• Factors that influence the outcome of the non ossifying fibroma include:
  • Young age
  • Open growth plates
  • Metaphyseal location

Diagnosis

Diagnostic Study of Choice

X ray of Non ossifying fibroma in proximal humerus.Source: Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 12463

• X-ray is the diagnostic study of choice for the diagnosis of non ossifying fibroma.
• X-ray findings include:
  • 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.
• 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:
  • Asymptomatic
  • Pain if associated with pathological fracture
• Findings associated with Jaffe-Campanacci syndrome include:
  • 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:
  • 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.

CT of non ossifying fibroma in distal femur.Source: Case courtesy of Dr Roberto Schubert, Radiopaedia.org, rID: 14173

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.
• 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.
• 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.

MRI of non ossifying fibroma in proximal humerus.Source: Case courtesy of Dr Hani Salam, Radiopaedia.org, rID: 12463

Other Diagnostic Studies

Biopsy

• Biopsy finding of non ossifying fibroma includes:
  • 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.

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

Indication

• Unstable fractures
• High risk of pathologic fracture

Technique

• The surgical approach involves exposing the fracture site and developing a cortical window to curette the tumor.

Primary Prevention

There are no established measures for the primary prevention of non ossifying fibroma.

Secondary Prevention

There are no established measures for the secondary prevention of non ossifying fibroma.

References

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