An Atlas of Musculoskeletal Oncology: Volume 4
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Transcript of An Atlas of Musculoskeletal Oncology: Volume 4
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Volume 4
Benign Chondroid Tumors
Enchondroma---------------------------Case 120 & 548-567Multiple enchondromatosis-----------Case 568-583Maffucci’s syndrome------------------Case 584-586Periosteal chondroma------------------Case 121 & 587-607Osteochondroma-----------------------Case 122 & 608-624Multiple hereditary exostosis---------Case 625-631Chondroblastoma-----------------------Case 123 & 632-646Chondromyxoid fibroma--------------Case 124 & 647-651
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Chondroid Chondroid forming forming TumorsTumors
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EnchondroEnchondromama
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Enchondroma The enchondroma is one of the most common benign tumors seenin the skeletal system. It is centrally located and in 50% of the caseswill be found in small tubular bones of hands and feet where it arises as a hamartomatous process and frequently remains un-diagnosed throughout the life of the patient until which time asthey develop a pathologic fracture thru the lesion. The lesions have a typical geographic pattern with sharp margination andfrequently demonstrate matrix calcification, especially as the patient ages. In the hand or foot, the lesions frequently cause marked thinning of the surrounding cortex and dilatation of the bone, whereas with enchondromas found in larger bones, thereis minimal evidence of cortical invasion and little if any evidenceof cortical thinning or dilatation. It is rare for an enchondroma to convert into a chondosarcoma in the hand or foot, but in larger bones such as the femur or pelvis a primary enchondroma can
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convert very gradually into a low grade chondrosarcoma. Theincidence of conversion is less than 5% of cases and almost alwaysin adults. In most cases, the enchondroma will be discovered as an incidental finding, because it is asymptomatic and non-disabling. There is no particular need to biopsy or bone graft the lesion unless the patient develops an interest in a specific sport or occupationalactivity that would put him at risk for fracture. If an acute fracture occurs, it is best to wait until the fracture heals and thenconsider the patient for a bone grafting procedure at a later date.
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CLASSICCase #120
38 year femaleenchondromadistal femur
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Bone scan
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Coronal T-1 MRI
tumor
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Coronal T-2 MRI
tumor
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Axial T-2 MRI
tumor
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Tumor seen thru cortical window
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Tumor curettement
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Photomic
tumor
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Closer up
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High power
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Curettement completed and ready for cement
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Cementation completed
cement
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Post op x-raytumor cavity filledwith radiolucentcement
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Case #548
57 year femaleenchondroma entirefemur
Smaller periostealchondroma proximaland posterior femur
periostealchondroma
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Biopsy photomic
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Post op x-rayfollowing removal ofperiosteal portion
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8 year followup withplacement of a longstem THA for stresspain symptoms
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Case #549
48 year femaleenchondroma femur
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Bone scan
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Case #550
59 year femaleenchondromadistal femur
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Case #550.1
Enchondroma distal femur in a 60 year male
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Bone scan
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Cor T-1 Cor T-2
Cor Gad
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Sag T-1 Sag T-2
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Axial T-2
Axial Gad
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Case #551
62 year maleenchondromaproximal humerus
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Coronal T-1 MRI
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CT scan
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Curettement specimen
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Low power photomic
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70 year male with enchondroma proximal humerus
degenerativearthritis
Case #552
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Coronal T-1 MRI
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Sagittal T-2 MRI
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Case #553
58 year femaleenchondromaproximal humerus
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Case #554
42 year femaleenchondromaproximal humerus
tumor
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Case #554.1 Enchondroma and ganglion cyst
47 year male with shoulder pain for 6 months
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ganglion cystenchondroma
Coronal T-2 MRI
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Sagittal T-2 enchondroma Sagittal T-2 ganglion
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Two different T-2 axial cuts showing the enchondroma and the right cut shows the ganglion
ganglion
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Case #555
55 year maleenchondromatibia
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Bone scan
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Coronal T-1 MRI
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Coronal T-2 MRI
tumor
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Biopsy photomic
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Case #556
55 year male with enchondroma tibia
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Lateral view
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Axial T-1 MRI
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Axial T-2 MRI
tumor
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Biopsy photomic
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Case #557
40 year female with enchondroma fibula
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Bone scan
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Sagittal T-1 MRI
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Axial T-1 MRI
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Case #558
22 year male with enchondroma proximal phalanx
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Biopsy photomic
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Case #559
33 year female with periosteal chondroma and enchondroma in same ray
enchondroma
Periosteal chondroma
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Oblique view
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Case #560
Pathologic fracture enchondroma proximal phalanx thumb29 year male
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Case #561
23 yr male with path fracture thru enchondroma thumb
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Case #562
31 year female with enchondroma great toe
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Amputationspecimen
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Photomic
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Case #564
11 yr boy with enchondroma lst metatarsal
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Case #565
26 year male with enchondroma 5th metatarsal
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Case #566
17 year male with enchondroma os calcis
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Os calcis view
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Case #567
28 year femaleenchondroma scapula
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Multiple Multiple EnchondromatoEnchondromato
sissisOllier’s DiseaseOllier’s Disease
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Multiple Enchondromatosis (Ollier’s)
Multiple enchondromatosis or Ollier’s disease, is a rare non-familial cartilagenous dysplasia that is typically seen on one halfof the body and appears similar to fibrous dysplasia. Extensive disease of the metaphyseal areas of long bone can result in bowingand shortening. In the case of the femur a major bowing is seendistally compared to the proximal bowing seen in fibrous dysplasia.The cortical thinning and epiphyseal involvement seen Ollier’sdisease is rarely seen in solitary enchondromas. The chance formalignant conversion to a low grade chondrosarcoma in adult lifeis only about 25%.
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CLASSICCase #568
3 year male with multiple enchondromatosis
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Extremities
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Macro section
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Photomic
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Case #569
6 year maleOllier’s disease
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Same patient
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Case #570
4.5 year maleOllier’s tibia
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Case #571
71 year female with Ollier’s left lower extremity
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Lateral view of knee
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Photomic
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Failure followingresurfacing TKAbecause of loosening
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Post op x-ray with constrained TKA
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X-ray of cemented stem distal femoralresection prosthesis
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Case #572
15 year femaleOllier’s disease
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AP close up knee
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Forearm disease
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Case #573
18 year maleOllier’s pelvisand ribs
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Case #574
2 year maleOllier’s lowerextremity
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Case #575
28 year maleOllier’s upperextremity
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Multiple sites in radial half of hand
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AP x-ray
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Case #576
19 year maleOllier’s femur
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Case #577
22 year male with Ollier’s of the hand
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Macro section of amputation specimen
tumor
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Close up view of macro section
tumor
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Case #578
40 year female with Ollier’s of the hand
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Case #579
10 year male Ollier’s hand
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Case #580
30 year femaleOllier’s hand
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Case #581
3 year maleOllier’s hand
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Case #582
19 year maleOllier’s feet
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Case #583
5 year female with Ollier’s of hand ready for surgery
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X-ray of both hands
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Photo of deformed feet after hand surgery
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Close up of deformed foot
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Multiple Multiple EnchondromatosisEnchondromatosis
PlusPlusSoft tissue Soft tissue
HemangiomatosisHemangiomatosis
Maffucci’ DiseaseMaffucci’ Disease
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Maffucci’s Syndrome
Patients with multiple enchondromatosis or Ollier’s disease will onoccasion develop soft tissue hemangiomatosis in the same areas as the enchondromatosis is seen. This combination of both a bonycartilaginous and a soft tissue angiomatosis is known as Maffucci’ssyndrome. The clinical appearance of the multiple enchondromatosisis the same as Ollier’s disease but with a higher potential for a malignant conversion to a low grade chondrosarcoma in adult life.
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CLASSICCase #584
33 year maleMaffucci’s disease
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X-ray tibias
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X-ray forearm
phleboliths
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Hand x-ray
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Hand amputation specimen
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Macrosection thru large enchondroma
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Photomic of enchondroma
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Case #585
36 year female with Muffucci’s syndrome
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Lateral view showingphleboliths
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Opposite femur
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Lateral view withphleboliths
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Gross specimen
hemangioma
enchondroma
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Case #586
25 year female with Muffucci’s syndrome hands
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PeriostealPeriosteal
ChondromaChondroma
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Periosteal Chondroma
The periosteal chondroma has the same basic pathology as theenchondroma except that it occurs on the surface of a tubularbone. It can occur as a hamartomatous process in growing patientsbut as with enchondroma it is usually asymptomatic and growthstops at bone maturity. The lesions are found in large bones, suchas the humerus and femur, and in the small bones of the hand andfoot. Radiographically one sees slight erosion into the adjacent cortex but the lesion will not penetrate into the medullary canal. There will be a sclerotic response at the base that extends around the periphery of the lesion taking on the appearance of a blisteron the surface of the bone with matrix calcification seen mainlyat the periphery but found also in the central area. The lesions usually stop growing before they reach the upper limit of 3-4 cmin diameter. If growth continues beyond this, one must stronglyconsider the possibility of a peripheral surface type chondrosarcoma
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that would continue to grow after bone maturity. Periosteal sarcoma is a similar chondroid lesion found typicallyon large bones. It demonstrates a more aggressive pattern, permeation into surrounding cortical structures and a more aggressive histology with some osteoid formation. Osteo-chondroma also has a similar appearance but would demonstrate a more mature bony base on which a cap of cartilage is typically seen. In the case of the periosteal chondroma, the cartilage extends down into the depths of the tumor. Treatment consists of a simple resection without disturbingthe subadjacent cortex so that bone grafting or internal fixation devices are not necessary. The recurrence rate is very low aftermaturity.
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Case #121
17 year femaleperiosteal chondromadistal femur
CLASSIC
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Bone scan
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CT scan
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Axial proton density MRI
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Sagittal proton density MRI
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Sagittal T-2 MRI
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Photomic
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Case #587
42 year male withcombined periosteal chondroma andenchondroma next to each other in femur
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enchondroma
periosteal chondroma
Bone scan
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Sagittal CT scan
enchondroma
periosteal chondroma
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Axial CT scan at level of enchondroma
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CT scan
enchondroma
periostealchondroma
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Coronal T-1 MRI
enchondroma
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Axial T-1 MRI
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Case #588
22 year maleperiosteal chondromafemur
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Case #589
16 year maleperiosteal chondromadistal femur
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AP view
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Case #590
12 year femaleperiosteal chondromaproximal femur
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Frog leg lateral
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Case #592
11 year femaleperiosteal chondromaproximal tibia
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Another view
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CTscan
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Axial proton density MRI
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Photomic
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Case #593
19 year female with periosteal chondroma tibia
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Case #594
26 year maleperiosteal chondromaproximal humerus
tumor
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CT scan
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Coronal T-1 MRI
tumor
small enchondroma
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Photomic
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Case #595
46 year female with periosteal chondroma humerus
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CT scan
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Another CT cut
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Coronal T-1 MRI
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Coronal T-2 MRI
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Photomic
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Case #595.1
17 year old female withslight pain right shoulderfor 1 year
Periosteal chondroma
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Sag T-1 T-2 Gad
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Axial T-1 T-2
Gad
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Case #596
14 year maleperiosteal chondromaproximal humerus
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AP view
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Case #598
62 year male with periosteal chondroma thumb
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Axial T-1 MRI
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Coronal proton density MRI
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T-2 MRI
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Photomic
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Case #599
8 year female with periosteal chondroma 4th metacarpal
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Case #600
25 year female with periosteal chondroma 3rd metatarsal
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Case #601
26 year male with periosteal chondroma ring finger
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Case #602
54 year male with periosteal chondroma 5th metatarsal
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Case #603
18 year maleperiosteal chondroma3rd toe
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Case #604
33 year male with soft tissue chondroma finger
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Lateral x-ray
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Oblique view
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Surgical excision
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Photomic
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Case #605
19 year maleperiosteal chondromaC-2
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Bone scan
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CT scan
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Case #606
38 year male with periosteal chondroma ischium
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Case #607
6l year female with periosteal chondroma clavicle
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OsteochondromOsteochondromaa
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Osteochondroma
The solitaty osteochondroma or exostosis is one of the mostcommon benign bone tumors seen in the skeleton. As in the enchondroma, this condition is developmental, or hamartomatousin nature arising from the outer edge of the growth plate and growing down the metaphyseal side where it tends to point awayfrom the adjacent joint. Because it originates from the growth plate,it continues to grow during the growing years of the patient and then stops at maturity. It is made up of a bony base with a pedunc-ulated stock with fatty marrow extending up inside the stock that has a cartilagenous cap giving it the appearance of a cauliflower.Histologically, the cap has features of a normal growth plate if abiopsy is performed during the growing years. Most of these lesions arise from large bones, especially about the knee joint, proximal femur, and proximal humerus. In the proximal humerus, the osteochondroma is usually sessile-based without a typical pedunc-
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ulated stock and is frequently misdiagnosed for that reason.Multiple hereditary exostosis presents with multiple lesions through-out the skeletal system and is considered to be an autosomaldominant disorder, being one-tenth as common as the solitaryosteochondroma which is not inherited. The conversion of a solitary osteochondoma to a chondro-sarcoma can only occur in the adult. It is an extremely rare event compared to the 1% chance of a malignant conversion in multiplehereditary exostosis. It is the cartilaginous cap that converts into alow grade secondary type of peripheral chondrosarcoma with an excellent prognosis for survival compared to a central chondro-sarcoma. These malignant conversions usually arise from largeosteochondromas seen in more proximal locations, such as aroundthe hip or pelvis. Because osteochondromata are usually withoutsymptoms, surgical treatment is frequently unnecessary unless the lesions create mechanical problems such as around the knee joint
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where larger lesions can interfere with normal ambulation.Sometimes, a large proximal lesion with a cartilage cap exceeding2 cm should be prophylactically resected in order to avoid apossible chondrosarcoma. When removing an osteochondroma, it is necessary only to remove the complete cartilaginous cap, leaving most of the bony base intact to avoid a facture.
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CLASSICCase #122
25 year maleosteochondroma tibia
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Lateral view
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Sagittal T-1 MRI
cartilage cap
fatty marrow
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Macro section
cartilage cap
fatty marrow
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Case #608
17 year male with osteochondroma humerus
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Axial T-1 MRI
cap
marrow
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Sagittal T-1 MRI
marrow
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Coronal T-2 MRI
cap
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Macro section
cap
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Case #609
30 year malesessile based osteochondromahumerus
Coronal T-1 MRI
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Coronal proton density MRI
cap
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Axial T-1 MRI
cap
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Axial T-2 MRI
cap
fatty marrow
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Case #610
14 year male with sessile based osteochondroma humerus
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Bone scan
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Coronal T-1 MRI
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Cut surgicalspecimen
cap
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Photomic
cartilage cap
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Case #611
10 year malesessile basedosteochondromaproximal humerus
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Lateral view
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Case #612
25 year maleosteochondomascapula
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Scapular view
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Resected specimen
cartilage cap
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Case #613
32 year male with osteochondroma proximal femur
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Frog leg lateral
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CT scan
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Case #614
12 year femaleosteochondromaproximal femur
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Case #615
15 year maleosteochondromadistal femur
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Cut specimen in path lab
cap
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Photomic
cartilage cap
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Case #616
23 year male with osteochondroma distal femur
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Cut specimen in path lab
cap
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Photomic
cartilage cap
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Case #617
26 year male with osteochondroma proximal fibula
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Lateral view
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Case #618
18 year male with sessile based osteochondroma tibia
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Case #619
11 year male sessile basedosteochondromadistal tibia
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Case #620
38 year male osteochondromaL-5
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Lateral view
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Bone scan
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CT scan
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Coronal T-2 MRI
cap
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Case #621
6 year male with osteochondroma C-6 spinous process
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CT scan
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Case #621
26 year male osteochondromailium
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Cut specimen in path lab
cap
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Case #621.1
22 year female with painless lump right iliac crest for years
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Axial T-1 Gad
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Cor T-2 Sag T-2
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Case #623
31 year female with osteochondroma ant 3rd rib
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Case #624
16 year male subungual exostosis
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AP x-ray
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Multiple Multiple Hereditary Hereditary ExostosisExostosis
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Case #625
15 year male with multiple hereditary exostoses
CLASSIC
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Tibia and fibula
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Lateral view
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Case #625.1
50 yr old female with short stature and R hip pain for years
Multiple hereditary exostoses
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AP and lateral of right hip
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AP and lat tibia
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Proximal humeral x-rays
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AP and lateral of femur
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Lateral ankle
Oblique foot
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Case #626
13 year femalemultiple exostosesforearm
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Distal femur
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Case #627
20 year malemultiple exostosesknee
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Lateral view
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Case #628
15 year male with multiple exostoses hips
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Case #629
26 year female with multiple exostoses thorax & arms
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Case #630
31 year malemultiple exostosesscapula & humerus
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Scapular view
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CT scan
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Case #631
26 year malemultiple exostosesleg
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ChondroblastoChondroblastomama
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Chondroblastoma
The chondroblastoma is a benign, cartilage-forming tumor seenin the metaepiphyseal area of bone in children and young adults. The histologic appearance is typical of a giant cell tumor in youngadults. The chondroblastoma is one fifth as common as the giant cell tumor and the majority of cases will occur during the seconddecade of life. It is rare to find the giant cell tumor in patients under the age of 13 years. The most common location for the chondroblastoma is in the epiphysis of the distal femur, proximal tibia, and proximal humerus. These lesions are usually located near a joint and are quite painful because of a secondary inflamm-atory synovitis of the adjacent joint that can masquerade as a pyarthrosis. It is not unusual to find an aneurysmal bone cystassociated with the chondroblastoma, similar to the situation seen with giant cell tumors. Histologically, one sees round polyhedral-shaped stromal cells located in clear cytoplasmic halos that gives
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the appearance of chicken wire under the microscope. Giant cellsare frequently associated with the chondroid tissue that gives theappearance of a giant cell tumor. It is very rare for a chondro-blastoma to convert to a malignant tumor, however, as in the caseof giant cell tumor, they can metastasize to the lung and stillcarry an excellent prognosis for survival. Treatment consists of a simple curettement of the lesion followed by packing the defect with either bone graft or bone cement. There is a relatively low (10%) recurrence rate.
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CLASSIC Case #123
12 year female with chondroblastoma proximal tibia
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Coronal post gad MRI
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Sagittal T-2 MRI
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Photomic
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Case #632
26 year male with chondroblastoma prox tibia
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Lateral view
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Sagittal T-1 MRI
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Sagittal T-2 MRI
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Photomic
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52 yr female with pain in knee for 1 yr
Case #632.1 Chondroblastoma pseudotumor (geode)
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Cor T-1 T-2
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Sag T-1
Axial T-2
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Case #633
21 year female with chondroblastoma prox tibia
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Case #634
16 year male with chondroblastoma distal femur
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Lateral view
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Coronal proton density MRI
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Sagittal proton density MRI
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Axial proton density MRI
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Curettement of tumorfrom window inintercondylar notch
tumor
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Photomic
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Higher power
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Case #635
18 year male chondroblastomadistal femur
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CT scan
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Photomic
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Case #636
20 year male with chondroblastoma & ABC prox femur
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Axial T-2 MRI
tumor
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Coronal T-2 MRItumor
edema
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Photomic
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Post op x-ray with cement & DHS
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Case #637
12 year female with chondroblastoma prox femoral epiphysis
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CT scan
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Another CT cut
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Photomic
giant cells
cartilage
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Case #638
13 year female with chondroblastoma prox fem epiphysis
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Case #639
15 year male with chondroblastoma prox humerus
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CT scan
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Axial T-1 MRI
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Coronal T-2 MRI
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Macro section
tumor
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Photomic
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Photomic with giant cells
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Case #640
15 year male with chondroblastoma proximal humerus
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Coronal T-1 MRI
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Coronal T-2 MRI
tumor
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Photomic with giant cells
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Another photomic
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Case #641
24 year female chondroblastoma pelvis
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CT scan
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Another CT cut
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Photomic
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Case #642
21 year male with chondroblastoma ilium
CT scan
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Another CT cut
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T-2 MRI
tumor
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Case #643
43 year female with chondroblastoma distal humerus
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CT scan
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Case #644
11 year male with chondroblastoma lateral malleolus
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Sagittal T-1 MRI
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Axial T-2 MRI
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Photomic
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Case #645
12 year femalechondroblastomadistal fibula
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Case #646
18 year male with chondroblastoma talus
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Lateral view
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Chondromyxoid Chondromyxoid FibromaFibroma
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Chondromyxoid Fibroma
Chondromyxoid fibroma is a very rare solitary benign tumor seenin bone. It occurs typically in the second or third decade of life andaffects men more than women. By far the more common location for this lesion is in the proximal tibial metaphysis, followed second by the distal femur and the first ray of the foot. This lesionis slow growing and is associated with mild symptoms of pain. Radiographically, there is a lytic lesion of bone with a soap-bubbly appearance secondary to the thin sclerotic peripheral margin giving it a pseudoloculated appearance similar to that of a solitary bone cyst. The adjacent cortex is frequently thinned outand slightly dilated, similar to the pattern that is seen in fibrousdysplasia. Histologically, there will be evidence of benign fibroustissue with large areas of myxomatous degeneration with reactivemacrophage activity seen at the periphery of the lesion that could suggest the diagnosis of a chondrosarcoma.
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Treatment for this lesion consists of a simple curettement and bonegrafting. The fairly high recurrence rate of 25% can be reduced by a more aggressive, marginal resection of the tumor. Occasionally, this lesion can convert into a chondrosarcoma.
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CLASSICCase #124
11 year malechondromyxoid fibromaproximal tibia
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Tomogram cut
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Surgical exposure of tumor
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Low power photomic
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Higher power
giant cells
myxoid
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Surgical curettement completed ready for bone graft
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Placement of fibular strut and cancellous graft
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Post op x-ray
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Case # 124.1
50 year male with chondromyxoid fibroma proximaltibia with 6 months of a tender tumor mass anterior tibia
AP and lat x-ray
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Coronal T-1 MRI
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Coronal PD FS MRI
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Axial PD FS MRI
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Axial Gad MRI
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Case #647
12 year female with chondromyxoid fibroma tibia
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Case #648
13 year female with chondromyxoid fibroma tibia
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Case #649
25 year female chondromyxoid fibroma 1st metatarsal
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Oblique view
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Photomic
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Photomic
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Case #650
7 year femalechondromyxoid fibroma ulna
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Photomic
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Case #651
25 year female with chondromyxoid fibroma rib
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Photomic
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Case #651.1
60 year female with chondromyxoid fibroma distal sacrum
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Sag T-1 Sag T-2
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Axial T-1
Axial T-2
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Coronal T-1