Dr James Wittig, MD Orthopedic Oncologist
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Bone Tumors
Chondromyxofibroma (CMF)

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James C. Wittig, MD
Sarcoma Surgeon
Orthopedic Oncologist



GENERAL INFORMATION

Chondromyxofibroma

Rare, least common benign cartilage tumor
Chondromyxofibroma consists of lobules of spindle or stellate cells in an abundant myxoid and chondroid stroma. Giant cells may be present. The lobules are separated by fibrous type tissue containing spindle type cells and giant cells.
Most commonly arises in the lower extremity
Proximal Tibia is most common site
Ilium is second most common site
Hands and Feet (3rd most common site)
Young adults: 60% of patients are < 30 yrs. old
Rarely malignant transformation to Chondrosarcoma
Benign aggressive tumor with high propensity for local recurrence following intralesional curettage. Tumor actively grows and destroys bone.



CLINICAL PRESENTATION

Signs/Symptoms:
Usually painful and may have mild swelling
A tumor mass is rare

Prevalence:
Slight to moderate male predilection (2:1 male to female ratio)
CMF constitutes less than 1% of all bone tumors

Age:
Occurs in all ages
Most common between 10 and 30 years of age

Sites:
Usually in long bones
Proximal tibia is most common site
Flat bones (approximately 25% of cases)
Ilium is the most commonly affected flat bone
May also occur in tubular bones of hands, feet, vertebrae, ribs, femur
Radiology of Chondromyxoid Fibroma (CMF)

Imaging:
Geographic, Well Circumscribed Lesion with IA-IC margin
Eccentric metaphyseal location; can be cortical
Expansile remodeling of bone
Rare matrix mineralization requires CT/Tomography usually for detection
          30% of cases show matrix mineralization/calcification
Often has an indolent, sclerotic medullary border and expansive blown out exterior border
Often has Internal Trabeculations
CT Scan: Useful for detecting minor calcifications and egg shell rim of calcification around soft tissue mass (periosteal reaction)
MRI:
TW1 images (similar or slightly lower intensity than T1W of muscle)
TW2 images (high signal intensity)


RADIOGRAPHIC PRESENTATION


Chondromyxofibroma of Proximal Tibia
Geographic, Eccentril Lesion
(Top arrow) Expansile Blown Out Border
(center)  Indolent, Sclerotic Medullary Border
(Right arrow) Subtle Internal Trabeculations


Plain X-ray: Chondromyxofibroma of Right Posterior Ilium



Plain X-Ray: CMF of Posterior Ilium: Geographic, Expansile Lesion




MRI T2 CMF of Right Posterior Ilium


MRI T2: CMF of Right Posterior Ilium (High Signal Intensity)




CT Scan of Chondromyxofibroma of Right Posterior Ilium

(Top arrow)  Subtle Mineralization
(Bottom arrow)  Geographic Expansile Lesion



Plain X-ray: Chondromyxofibroma of Calcaneus




Plain X-ray: Chondromyxofibroma of Calcaneus


Typically an eccentrically located, metaphyseal lesion
May extend into epiphysis
Long axes parallel to bone
Usually sharply demarcated
Scalloped margins
Intralesional calcified matrix rare


MRI T1 Chondromyxofibroma of Calcaneus
Intermediate Signal Similar to Muscle


(Left arrow)  Expansile Outer Border, Expanding Cortex
(Right arrow)  Indolent Border, Sharp Zone of Transition


MRI T2: Chondromyxofibroma of Calcaneus (Cartilage Tumors are Often High Signal on T2 Weighted Images)


Plain X-ray: CMF of Distal Femur

 


MRI T1: CMF of Distal Femur (Eccentric, Expansile with Indolent Medullary Border)
Intermediate Signal




MRI T2: CMF of Distal Femur
High Signal




Plain X-ray: Chondromyxofibroma of Right Femoral Neck




Xray: Chondromyxofibroma of Right Femoral Neck




Plain X-ray: CMF of Femoral Neck




CT: CMF of Right Femoral Neck




CT: CMF of Femoral Neck


(Left arrow)  Expansile Outer Border with Egg Shell Rim of Calcification
(Right arrow)  Indolent Medullary Border Sharply Circumscribed



Plain X-ray: Chondromyxofibroma of Proximal Phalanx of Toe


(Left arrow)  Expansile Lesion, Well Circumscribed, Geographic, No Mineralization Detected (Mineralization is not always detected in CMF)
(Right arrow)  Sclerotic Medullary Border


CT Scan (sagittal): CMF of Proximal Phalanx of Big Toe




CT Scan (coronal): CMF of Proximal Phalanx of Big Toe




MRI T2: CMF of Proximal Phalanx of Big Toe (High Signal)




MRI T2: CMF of Proximal Phalanx of Big Toe




Specimen: Curettings




Tumor Cavity of Proximal Phalanx after Curettage




X-ray and CT Scan of CMF of Distal Humerus
(Elbow)

  




MRI T1 and T2 of CMF of Distal Humerus 
   


Plain X-Rays of CMF of Proximal Right Tibia


(arrows)  Sclerotic/Indolent Medullary Border


CT Scan of CMF of Proximal Tibia
Calcifications within Lesion are Demonstrated




MRI T1 and T2 of CMF of Proximal Tibia

   

 T1 Weighted MRI T2 Weighted MRI


CMF of Radius

Xray T1 Weighted MRI T2 Weighted MRI

MRI of CMF of Proximal Tibia

   

T1 Weighted MRI

T2 Weighted MRI


CT Scan of Proximal Tibia CMF


(Left arrow) Expansile Outer Border, Benign Appearance
(Center arrow)  Minor Calcifications, Thin Internal Trabeculations
(Right arrow)  Indolent Medullary Border


Chondromyxoid Fibroma (CMF)

Pathology:
Myxoid, fibrous and chondroid tissue in various proportions
Myxoid areas - central
Cellular areas - peripheral
Foci of calcifications 5-27%

Lobulated tumor mass


GROSS PATHOLOGY

 

Typically sharply circumscribed
Lobulated and firm
Gray-white or blue-gray
If cortex is eroded, intact periosteum is still evident
Soft tissue implants are grossly identical

(Top) Curettings
(Bottom) Tumor Cavity of Proximal Phalanx after Curettage


 


MICROSCOPIC PATHOLOGY

Chondroid, Myxoid and Fibrous Areas with Lobular Growth Pattern
Sharply demarcated margins
          Either by bone or connective tissue/periosteum
Very nodular/lobular architecture of myxoid lobules/chondroid tissue
          Periphery is typically hypercellular
          Cells in this area usually resemble chondroblasts
Fibrous component
          Accounts for small portion of tumor
          Consists mainly of narrow fibrous septa separating the lobules
                  Contain large venules, muscular arteries, multinucleated giant cells, and occasionally, 
                  osteoid
Myxoid areas
         Highly variable cellularity
                  Range
                          Closely packed fibroblasts
                                       Small, narrow nuclei
                          Large cells with nuclear aberrations
Distinctive Stellate Chondroid cells
        Eosinophilic cytoplasm
        One or more long cytoplasmic processes
Polynucleated cells frequent
Mitotic figures are usually absent


Microscopic Pathology CMF

Microscopic Pathology CMF

(Top arrows)  Chondromyxoid Area
(Bottom arrows)  Fibrous Area



Microscopic Pathology

 


Microscopic Pathology


Fibrous Areas Around Periphery of Mysoid Chondroid Areas
Myxoid Area with Chondroid Tissue with Stellate Types of Cells


Giant Cells are Common in CMF
Fibrous Area with a Giant Cell




High Power of Chondromyxoid Area with Stellate Types of Cells
Stellate Cells have Long Cytoplasmic Processes



Low Power of Chondromyxoid Area



High Power of Chondromyxoid Area and Stellate Type Cells


Microscopic Pathology CMF - High Power of Fibrous Area
 



BIOLOGICAL BEHAVIOR

Chondromyxoid fibroma is a benign but aggressive tumor.
The tumor grows aggressively in the bone.
              Grows progressively and destroys bone
CMF does not metastasize
Malignant change to chondrosarcoma is possible, but extremely rare (only a few reported cases)
These tumors notoriously recur locally within the bone or soft tissue where they grow from.
May infiltrate nearby soft tissue
             Also might infiltrate periosteum


TREATMENT

CMF is a benign aggressive tumor. It recurs 30%-60% of the time after intralesional curettage. It grows aggressively and destroys bone.

Treatment: Intralesional curettage and cemented internal fixation and/or  bone grafting.

Local adjuvants such as cryosurgery (liquid nitrogen application) should be considered to reduce the risk of local recurrence. It is the authors preference in selected cases to utilize curettage and cryosurgery combined with cement/internal fixation in most cases. Bone grafting may be considered for selected cases.

En bloc resection may need to be considered for extremely large tumors and/or tumors that have recurred after being treated with previous intralesional surgeries.


PROGNOSIS

Prognosis is excellent in most cases
          Even with recurrence
Curettage with bone grafting has been associated with recurrence rates as high as 30% as reported in the literature
         Most recurrences are seen within 2 years
         Local recurrences can result in further bone destruction
Patients under the age of 15 are especially prone to recurrence
Soft tissue implantation is rare but can occur and result in local recurrence
Rarely local recurrences can be aggressive and require an amputation for treatment


 

 
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