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
Historical Perspective
In 1948, Jaffe and Lichtenstein described chondromyxoid fibroma for the first time.[1]
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.[2]
- 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.[3]
- Chondromyxoid fibroma arises from the physeal remanant is the commonly proposed theory concerning the pathogenesis of chondromyxoid fibroma.[4]
- It is predominantly found in the metaphyses of the long bones.[5]
- The bones involved by chondromyxoid fibroma includes tibia, distal femur, pelvis, feet and hands.[6]
Genetics
- Chromosome 6 appears to be involved with chondromyxoid fibroma.[5][7]
- The non-random clonal abnormalities involves 6p25, 6q13 and 6q23.[5]
- 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.[8]
Causes
There are no established causes for chondromyxoid fibroma.[9]
Differentiating Chondromyxoid Fibroma from Other Diseases
Aneurysmal bone cyst 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.[10]
- 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
Diagnostic Study of Choice
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
OR
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
OR
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
OR
There are no established criteria for the diagnosis of [disease name].
History and Symptoms
The majority of patients with [disease name] are asymptomatic.
OR
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
Physical Examination
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
OR
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
OR
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
Laboratory Findings
There are no diagnostic laboratory findings associated with chondromyxoid fibroma.
Electrocardiogram
There are no ECG findings associated with chondromyxoid fibroma.
X-ray
There are no x-ray findings associated with [disease name].
OR
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with [disease name].
OR
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
CT scan
There are no CT scan findings associated with [disease name].
OR
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
MRI
There are no MRI findings associated with [disease name].
OR
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Other Imaging Findings
There are no other imaging findings associated with 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
Surgical intervention is not recommended for the management of [disease name].
OR
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
OR
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
OR
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
OR
Surgery is the mainstay of treatment for [disease or malignancy].
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
- ↑ JAFFE HL, LICHTENSTEIN L (1948). "Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma". Arch Pathol (Chic). 45 (4): 541–51. PMID 18891025.
- ↑ Jawad MU, Scully SP (2010). "In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system". Clin Orthop Relat Res. 468 (7): 2000–2. doi:10.1007/s11999-010-1315-7. PMC 2882012. PMID 20333492.
- ↑ Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
- ↑ Brien EW, Mirra JM, Kerr R (1997). "Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors". Skeletal Radiol. 26 (6): 325–53. PMID 9229417.
- ↑ 5.0 5.1 5.2 Cuvelier CA, Roels HJ (1979). "Cytophotometric studies of the nuclear DNA content in cartilaginous tumors". Cancer. 44 (4): 1363–74. PMID 498016.
- ↑ Halbert AR, Harrison WR, Hicks MJ, Davino N, Cooley LD (1998). "Cytogenetic analysis of a scapular chondromyxoid fibroma". Cancer Genet Cytogenet. 104 (1): 52–6. PMID 9648559.
- ↑ Buddingh EP, Naumann S, Nelson M, Neffa JR, Birch N, Bridge JA (2003). "Cytogenetic findings in benign cartilaginous neoplasms". Cancer Genet Cytogenet. 141 (2): 164–8. PMID 12606137.
- ↑ Nord KH, Lilljebjörn H, Vezzi F, Nilsson J, Magnusson L, Tayebwa J; et al. (2014). "GRM1 is upregulated through gene fusion and promoter swapping in chondromyxoid fibroma". Nat Genet. 46 (5): 474–7. doi:10.1038/ng.2927. PMID 24658000.
- ↑ Gherlinzoni F, Rock M, Picci P (1983). "Chondromyxoid fibroma. The experience at the Istituto Ortopedico Rizzoli". J Bone Joint Surg Am. 65 (2): 198–204. PMID 6337162.
- ↑ Pintor F, Bahamondes C, Campos O, Zivov A (2015). "Chondromyxoid fibroma of zygoma in an elderly patient: A rare presentation". Ann Maxillofac Surg. 5 (2): 244–8. doi:10.4103/2231-0746.175755. PMC 4772571. PMID 26981481.