Chondromyxoid fibroma

WikiDoc Resources for Chondromyxoid fibroma
Articles
Most recent articles on Chondromyxoid fibroma Most cited articles on Chondromyxoid fibroma Review articles on Chondromyxoid fibroma Articles on Chondromyxoid fibroma in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Chondromyxoid fibroma Images of Chondromyxoid fibroma Photos of Chondromyxoid fibroma Podcasts & MP3s on Chondromyxoid fibroma Videos on Chondromyxoid fibroma
Evidence Based Medicine
Cochrane Collaboration on Chondromyxoid fibroma Bandolier on Chondromyxoid fibroma TRIP on Chondromyxoid fibroma
Clinical Trials
Ongoing Trials on Chondromyxoid fibroma at Clinical Trials.gov Trial results on Chondromyxoid fibroma Clinical Trials on Chondromyxoid fibroma at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Chondromyxoid fibroma NICE Guidance on Chondromyxoid fibroma NHS PRODIGY Guidance FDA on Chondromyxoid fibroma CDC on Chondromyxoid fibroma
Books
Books on Chondromyxoid fibroma
News
Chondromyxoid fibroma in the news Be alerted to news on Chondromyxoid fibroma News trends on Chondromyxoid fibroma
Commentary
Blogs on Chondromyxoid fibroma
Definitions
Definitions of Chondromyxoid fibroma
Patient Resources / Community
Patient resources on Chondromyxoid fibroma Discussion groups on Chondromyxoid fibroma Patient Handouts on Chondromyxoid fibroma Directions to Hospitals Treating Chondromyxoid fibroma Risk calculators and risk factors for Chondromyxoid fibroma
Healthcare Provider Resources
Symptoms of Chondromyxoid fibroma Causes & Risk Factors for Chondromyxoid fibroma Diagnostic studies for Chondromyxoid fibroma Treatment of Chondromyxoid fibroma
Continuing Medical Education (CME)
CME Programs on Chondromyxoid fibroma
International
Chondromyxoid fibroma en Espanol Chondromyxoid fibroma en Francais
Business
Chondromyxoid fibroma in the Marketplace Patents on Chondromyxoid fibroma
Experimental / Informatics
List of terms related to Chondromyxoid fibroma

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:

Overview

Chondromyxoid fibroma is a rare benign bone tumor accounting for approximately 1% of all benign bone tumors. Chondromyxoid fibroma affects all age groups. It is more commonly seen during the second and third decades of life. Men are more commonly affected than women. Chondromyxoid fibroma arising from the physeal remanant is the commonly proposed theory concerning the pathogenesis of chondromyxoid fibroma. It is predominantly found in the metaphyses of the long bones. The bones involved by chondromyxoid fibroma includes tibia, distal femur, pelvis, feet and hands. Chromosome 6 appears to be involved with chondromyxoid fibroma. Over expression of the glutamate receptor gene GRM1 in patients with chondromyxoid fibroma via promoter swapping and gene fusion has been observed. The hallmark of chondromyxoid fibroma is pain, swelling and pathological fracture. The mainstay of treatment for chondromyxoid fibroma is surgery in form of curettage and bone grafting.

Historical Perspective

In 1948, Jaffe and Lichtenstein described chondromyxoid fibroma for the first time.

Classification

Chondromyxoid fibromacan 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 etiology of chondromyxoid fibroma is unknown.
• Chondromyxoid fibroma arising from the physeal remanant is the commonly proposed theory concerning the pathogenesis of chondromyxoid fibroma.
• It is predominantly found in the metaphyses of the long bones.
• The bones involved by chondromyxoid fibroma includes tibia, distal femur, pelvis, feet and hands.

Genetics

• Chromosome 6 appears to be involved with chondromyxoid fibroma.
• The non-random clonal abnormalities involves 6p25, 6q13 and 6q23.
• whole-genome mate-pair sequencing and RNA sequencing showed over expression of the glutamate receptor gene GRM1 in patients with chondromyxoid fibroma via promoter swapping and gene fusion.

Causes

There are no established causes for chondromyxoid fibroma.

Differentiating Chondromyxoid Fibroma from Other Diseases

Chondromyxoid 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
Chondromyxoid Fibroma	-	-	Radiology and biopsy	+
Unicameral bone cyst	+	-	Radiology and biopsy	-
Non ossifying fibroma	+	-	Radiology and biopsy	-
Giant cell tumor	-	-	Radiology and Biopsy	+
Chondroblastoma	-	-	Biopsy	+
Aneurysmal bone cyst	+	+	Radiology and biopsy	+
Osteoblastoma	-	-	Radiology and biopsy	+
Telangiectatic osteosarcoma	-	+	Radiology and biopsy	-

Epidemiology and Demographics

• Chondromyxoid fibroma is a rare benign bone tumor accounting for approximately 1% of all benign bone tumors.
• Chondromyxoid fibroma affects all age groups.
• It is more commonly seen during the second and third decades of life.
• Men are more commonly affected than women.
• There is no racial predilection to chondromyxoid fibroma.

Risk Factors

There are no established risk factors for chondromyxoid fibroma.

Screening

There is insufficient evidence to recommend routine screening for chondromyxoid fibroma.

Natural History, Complications, and Prognosis

• Common complications of chondromyxoid fibroma include:
  • Pathological fracture
  • Premature epiphyseal closure
    • Limb-length discrepancy
    • Angular deformity
• Prognosis is generally good for chondromyxoid fibroma.
• Prognostic variables include:
  • Children
  • Tumor is more lobulated with abundant myxoid material

Diagnosis

Histology of chondromyxoid fibroma showing stellate cells in a myxoid stroma..Source: Case courtesy of Sarahkayb [CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0), from Wikimedia Commons]

Diagnostic Study of Choice

• Biopsy is the diagnostic study of choice for the diagnosis of chondromyxoid fibroma.
• Biopsy findings include:
  • Under low power field:
    • It has a biphasic appearance
    • It has lobules and pseudolobules of fibromyxoid tissue with hypercellular area.
    • The cells are spindle-shaped or stellate-shaped and contain hyperchromatic nuclei.
    • Multinucleated giant cells and fibrovascular tissue may be present between the lobules.
  • Hypocellular area with chondroid material is seen.
  • Under high power field:
    • The tumor shows myxoid stroma mixed with stellate cells
    • In addition, an area of pleiomorphic cells with bizarre nuclei may be seen.

History and Symptoms

• The majority of patients with chondromyxoid fibroma have a positive history of:
  • Pain
  • Swelling
  • Stiffness
  • Pathological fracture

X-ray of ulna showing chondromyxoid fibroma.Source: Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 6172

Physical Examination

• Common physical examination findings of chondromyxoid fibroma include:
  • Deformity
  • Swelling
  • Decreased range of motion
  • Warmth over the affected area

Laboratory Findings

There are no diagnostic laboratory findings associated with chondromyxoid fibroma.

Electrocardiogram

There are no ECG findings associated with chondromyxoid fibroma.

X-ray

• X-ray is helpful in diagnosis of chondromyxoid fibroma.
• X-ray findings include:
  • The tumor appear as a lytic, eccentric metaphyseal lesion.
  • The lesion size may vary from 2 to 10 cm.
  • It has a sharply demarcated border from the adjacent bone.

CT of proximal ulna showing chondromyxoid fibroma.Source: Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 6172

• • It may show scalloped and sclerotic rim
  • Calcification is usually not seen
  • The tumor may show cortical expansion.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with chondromyxoid fibroma.

CT scan

• CT scan confirms the x-ray findings.
• CT is superior to x-ray for analyzing the expansion of the lesion and cortical breach.

MRI

• MRI findings of chondromyxoid fibroma include:

MRI of proximal ulna showing chondromyxoid fibroma.Source: Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 6172

• T1 image: Chondroid and myxoid tissues produce low signals.
• T2 image: Chondroid and myxoid tissues produce high signals.

Bone Scan

• Increased uptake is seen in chondromyxoid fibroma.

Other Diagnostic Studies

There are no other diagnostic studies associated with chondromyxoid fibroma.

Treatment

Medical Therapy

There is no treatment for chondromyxoid fibroma; the mainstay of therapy is surgery.

Surgery

Surgery is the mainstay of treatment for chondromyxoid fibroma.

Types

• Intralesional curettage and bone grafting or cement
• En bloc excision

Intralesional curettage

• It is the mainstay of treatment for chondromyxoid fibroma.
• The defect is filled with bone graft or bone cement in form of polymethlymethacrylate(PMMA).

En Bloc Incision

• It usually reserved for recurrent cases.

Recurrence Rate

• The recurrence rate is about 25% for chondromyxoid fibroma.

Primary Prevention

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

Secondary Prevention

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

References

Template:WikiDoc Sources