Chondromyxoid fibroma

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Chondromyxoid fibroma_table_infobox_0

Chondromyxoid fibromaChondromyxoid fibroma_header_cell_0_0_0
SpecialtyChondromyxoid fibroma_header_cell_0_1_0 OncologyChondromyxoid fibroma_cell_0_1_1

Chondromyxoid fibroma is a type of cartilaginous tumor. Chondromyxoid fibroma_sentence_0

Most cases are characterised by GRM1 gene fusion or promoter swapping. Chondromyxoid fibroma_sentence_1

It can be associated with a translocation at t(1;5)(p13;p13). Chondromyxoid fibroma_sentence_2

A chondromyxoid fibroma (CMF) is an extremely rare benign cartilaginous neoplasm which accounts for < 1% bone tumours. Chondromyxoid fibroma_sentence_3

Signs and symptoms Chondromyxoid fibroma_section_0

Typically people present with progressive pain, often long standing and/or bony swelling and restricted range of movement in affected limb 3,12. Chondromyxoid fibroma_sentence_4

The latter is most often the case in bones with little overlying soft tissues (e.g. short tubular bones of the hands and feet). Chondromyxoid fibroma_sentence_5

Most chondromyxoid fibromas are located in the metaphyseal region of long bones (60%), and may extend to the epiphyseal line and even rarely abut the articular surface 3,12. Chondromyxoid fibroma_sentence_6

They are almost never just epiphyseal 3. Chondromyxoid fibroma_sentence_7

The classical site is the upper 1/3rd of tibia ( which accounts for 25% of all cases) with the small tubular bones of the foot, the distal femur and pelvis being other relatively common locations 12. Chondromyxoid fibroma_sentence_8

Rarely occur in the skull or skull base. Chondromyxoid fibroma_sentence_9

Pathology Chondromyxoid fibroma_section_1

The tumor comprises a variable combination on chondroid, myxoid, and fibrous tissue components organized in a pseudolobulated architecture 20. Chondromyxoid fibroma_sentence_10

On gross examination they are typically seen as solid glistening tan-gray intraosseous masses. Chondromyxoid fibroma_sentence_11

Occasional osteoclast-like giant multinucleated cells are encountered particularly at the periphery. Chondromyxoid fibroma_sentence_12

Most cells are morphologically bland, and mitotic figures are rare or absent 13. Chondromyxoid fibroma_sentence_13

Diagnosis Chondromyxoid fibroma_section_2

Radiographic features Chondromyxoid fibroma_section_3

Plain film often seen as a lobulated, eccentric radiolucent lesion long axis parallel to long axis of long bone no periosteal reaction (unless a complicating fracture present) geographic bone destruction: almost 100% well defined sclerotic margin: 86% there can be presence of septations (pseudotrabeculation): 57% 2 there can be presence of matrix calcification in a small proportion of cases: 12.5%1 MRI MR features are often not particularly specific. Chondromyxoid fibroma_sentence_14

Signal characteristics include Chondromyxoid fibroma_sentence_15

T1 - low signal T1 C+ (Gd) - the majority (~70%) tend to show peripheral nodular enhancement ~ 30% diffuse contrast enhancement and this can be either homogeneous or heterogeneous 19 T2 - high signal Bone scan A scintigraphic "doughnut sign" has been described in this tumour type 11. Chondromyxoid fibroma_sentence_16

However, this is very non-specific and can be found in a plethora of other bone lesions. Chondromyxoid fibroma_sentence_17

Treatment Chondromyxoid fibroma_section_4

They are benign lesions and malignant degeneration is rare. Chondromyxoid fibroma_sentence_18

They are usually treated with curettage which however have a high recurrence rate of 25%. Chondromyxoid fibroma_sentence_19

As such if an en-bloc resection is possible this is advisable Chondromyxoid fibroma_sentence_20

Epidemiology Chondromyxoid fibroma_section_5

The majority of cases occur in the second and third decades, with approximately 75% of cases occurring before the age of 30 years 1,12-15. Chondromyxoid fibroma_sentence_21

There is no recognised gender predilection. Chondromyxoid fibroma_sentence_22

Examples have however been seen in patients up to the age of 75 years. Chondromyxoid fibroma_sentence_23

In some series there is a male predilection 12 whilst in others no such distribution is found 2 Chondromyxoid fibroma_sentence_24

Credits to the contents of this page go to the authors of the corresponding Wikipedia page: fibroma.