Atlas of Musculoskeletal Ultrasound Anatomy 2nd ed. - M. Bradley, P. O'Donnell (Cambridge, 2009) WW
An Atlas of Musculoskeletal Oncology: Volume 4
357
Volume 4 Benign Chondroid Tumors ndroma---------------------------Case 120 & ple enchondromatosis-----------Case 568-583 cci’s syndrome------------------Case 584-586 steal chondroma------------------Case 121 & chondroma-----------------------Case 122 & 6 ple hereditary exostosis---------Case 625-63 roblastoma-----------------------Case 123 & romyxoid fibroma--------------Case 124 & 647
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Transcript of An Atlas of Musculoskeletal Oncology: Volume 4
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
Chondroid Chondroid forming forming TumorsTumors
EnchondroEnchondromama
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
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.
CLASSICCase #120
38 year femaleenchondromadistal femur
Bone scan
Coronal T-1 MRI
tumor
Coronal T-2 MRI
tumor
Axial T-2 MRI
tumor
Tumor seen thru cortical window
Tumor curettement
Photomic
tumor
Closer up
High power
Curettement completed and ready for cement
Cementation completed
cement
Post op x-raytumor cavity filledwith radiolucentcement
Case #548
57 year femaleenchondroma entirefemur
Smaller periostealchondroma proximaland posterior femur
periostealchondroma
Biopsy photomic
Post op x-rayfollowing removal ofperiosteal portion
8 year followup withplacement of a longstem THA for stresspain symptoms
Case #549
48 year femaleenchondroma femur
Bone scan
Case #550
59 year femaleenchondromadistal femur
Case #550.1
Enchondroma distal femur in a 60 year male
Bone scan
Cor T-1 Cor T-2
Cor Gad
Sag T-1 Sag T-2
Axial T-2
Axial Gad
Case #551
62 year maleenchondromaproximal humerus
Coronal T-1 MRI
CT scan
Curettement specimen
Low power photomic
70 year male with enchondroma proximal humerus
degenerativearthritis
Case #552
Coronal T-1 MRI
Sagittal T-2 MRI
Case #553
58 year femaleenchondromaproximal humerus
Case #554
42 year femaleenchondromaproximal humerus
tumor
Case #554.1 Enchondroma and ganglion cyst
47 year male with shoulder pain for 6 months
ganglion cystenchondroma
Coronal T-2 MRI
Sagittal T-2 enchondroma Sagittal T-2 ganglion
Two different T-2 axial cuts showing the enchondroma and the right cut shows the ganglion
ganglion
Case #555
55 year maleenchondromatibia
Bone scan
Coronal T-1 MRI
Coronal T-2 MRI
tumor
Biopsy photomic
Case #556
55 year male with enchondroma tibia
Lateral view
Axial T-1 MRI
Axial T-2 MRI
tumor
Biopsy photomic
Case #557
40 year female with enchondroma fibula
Bone scan
Sagittal T-1 MRI
Axial T-1 MRI
Case #558
22 year male with enchondroma proximal phalanx
Biopsy photomic
Case #559
33 year female with periosteal chondroma and enchondroma in same ray
enchondroma
Periosteal chondroma
Oblique view
Case #560
Pathologic fracture enchondroma proximal phalanx thumb29 year male
Case #561
23 yr male with path fracture thru enchondroma thumb
Case #562
31 year female with enchondroma great toe
Amputationspecimen
Photomic
Case #564
11 yr boy with enchondroma lst metatarsal
Case #565
26 year male with enchondroma 5th metatarsal
Case #566
17 year male with enchondroma os calcis
Os calcis view
Case #567
28 year femaleenchondroma scapula
Multiple Multiple EnchondromatoEnchondromato
sissisOllier’s DiseaseOllier’s Disease
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%.
CLASSICCase #568
3 year male with multiple enchondromatosis
Extremities
Macro section
Photomic
Case #569
6 year maleOllier’s disease
Same patient
Case #570
4.5 year maleOllier’s tibia
Case #571
71 year female with Ollier’s left lower extremity
Lateral view of knee
Photomic
Failure followingresurfacing TKAbecause of loosening
Post op x-ray with constrained TKA
X-ray of cemented stem distal femoralresection prosthesis
Case #572
15 year femaleOllier’s disease
AP close up knee
Forearm disease
Case #573
18 year maleOllier’s pelvisand ribs
Case #574
2 year maleOllier’s lowerextremity
Case #575
28 year maleOllier’s upperextremity
Multiple sites in radial half of hand
AP x-ray
Case #576
19 year maleOllier’s femur
Case #577
22 year male with Ollier’s of the hand
Macro section of amputation specimen
tumor
Close up view of macro section
tumor
Case #578
40 year female with Ollier’s of the hand
Case #579
10 year male Ollier’s hand
Case #580
30 year femaleOllier’s hand
Case #581
3 year maleOllier’s hand
Case #582
19 year maleOllier’s feet
Case #583
5 year female with Ollier’s of hand ready for surgery
X-ray of both hands
Photo of deformed feet after hand surgery
Close up of deformed foot
Multiple Multiple EnchondromatosisEnchondromatosis
PlusPlusSoft tissue Soft tissue
HemangiomatosisHemangiomatosis
Maffucci’ DiseaseMaffucci’ Disease
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.
CLASSICCase #584
33 year maleMaffucci’s disease
X-ray tibias
X-ray forearm
phleboliths
Hand x-ray
Hand amputation specimen
Macrosection thru large enchondroma
Photomic of enchondroma
Case #585
36 year female with Muffucci’s syndrome
Lateral view showingphleboliths
Opposite femur
Lateral view withphleboliths
Gross specimen
hemangioma
enchondroma
Case #586
25 year female with Muffucci’s syndrome hands
PeriostealPeriosteal
ChondromaChondroma
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
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.
Case #121
17 year femaleperiosteal chondromadistal femur
CLASSIC
Bone scan
CT scan
Axial proton density MRI
Sagittal proton density MRI
Sagittal T-2 MRI
Photomic
Case #587
42 year male withcombined periosteal chondroma andenchondroma next to each other in femur
enchondroma
periosteal chondroma
Bone scan
Sagittal CT scan
enchondroma
periosteal chondroma
Axial CT scan at level of enchondroma
CT scan
enchondroma
periostealchondroma
Coronal T-1 MRI
enchondroma
Axial T-1 MRI
Case #588
22 year maleperiosteal chondromafemur
Case #589
16 year maleperiosteal chondromadistal femur
AP view
Case #590
12 year femaleperiosteal chondromaproximal femur
Frog leg lateral
Case #592
11 year femaleperiosteal chondromaproximal tibia
Another view
CTscan
Axial proton density MRI
Photomic
Case #593
19 year female with periosteal chondroma tibia
Case #594
26 year maleperiosteal chondromaproximal humerus
tumor
CT scan
Coronal T-1 MRI
tumor
small enchondroma
Photomic
Case #595
46 year female with periosteal chondroma humerus
CT scan
Another CT cut
Coronal T-1 MRI
Coronal T-2 MRI
Photomic
Case #595.1
17 year old female withslight pain right shoulderfor 1 year
Periosteal chondroma
Sag T-1 T-2 Gad
Axial T-1 T-2
Gad
Case #596
14 year maleperiosteal chondromaproximal humerus
AP view
Case #598
62 year male with periosteal chondroma thumb
Axial T-1 MRI
Coronal proton density MRI
T-2 MRI
Photomic
Case #599
8 year female with periosteal chondroma 4th metacarpal
Case #600
25 year female with periosteal chondroma 3rd metatarsal
Case #601
26 year male with periosteal chondroma ring finger
Case #602
54 year male with periosteal chondroma 5th metatarsal
Case #603
18 year maleperiosteal chondroma3rd toe
Case #604
33 year male with soft tissue chondroma finger
Lateral x-ray
Oblique view
Surgical excision
Photomic
Case #605
19 year maleperiosteal chondromaC-2
Bone scan
CT scan
Case #606
38 year male with periosteal chondroma ischium
Case #607
6l year female with periosteal chondroma clavicle
OsteochondromOsteochondromaa
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-
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
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.
CLASSICCase #122
25 year maleosteochondroma tibia
Lateral view
Sagittal T-1 MRI
cartilage cap
fatty marrow
Macro section
cartilage cap
fatty marrow
Case #608
17 year male with osteochondroma humerus
Axial T-1 MRI
cap
marrow
Sagittal T-1 MRI
marrow
Coronal T-2 MRI
cap
Macro section
cap
Case #609
30 year malesessile based osteochondromahumerus
Coronal T-1 MRI
Coronal proton density MRI
cap
Axial T-1 MRI
cap
Axial T-2 MRI
cap
fatty marrow
Case #610
14 year male with sessile based osteochondroma humerus
Bone scan
Coronal T-1 MRI
Cut surgicalspecimen
cap
Photomic
cartilage cap
Case #611
10 year malesessile basedosteochondromaproximal humerus
Lateral view
Case #612
25 year maleosteochondomascapula
Scapular view
Resected specimen
cartilage cap
Case #613
32 year male with osteochondroma proximal femur
Frog leg lateral
CT scan
Case #614
12 year femaleosteochondromaproximal femur
Case #615
15 year maleosteochondromadistal femur
Cut specimen in path lab
cap
Photomic
cartilage cap
Case #616
23 year male with osteochondroma distal femur
Cut specimen in path lab
cap
Photomic
cartilage cap
Case #617
26 year male with osteochondroma proximal fibula
Lateral view
Case #618
18 year male with sessile based osteochondroma tibia
Case #619
11 year male sessile basedosteochondromadistal tibia
Case #620
38 year male osteochondromaL-5
Lateral view
Bone scan
CT scan
Coronal T-2 MRI
cap
Case #621
6 year male with osteochondroma C-6 spinous process
CT scan
Case #621
26 year male osteochondromailium
Cut specimen in path lab
cap
Case #621.1
22 year female with painless lump right iliac crest for years
Axial T-1 Gad
Cor T-2 Sag T-2
Case #623
31 year female with osteochondroma ant 3rd rib
Case #624
16 year male subungual exostosis
AP x-ray
Multiple Multiple Hereditary Hereditary ExostosisExostosis
Case #625
15 year male with multiple hereditary exostoses
CLASSIC
Tibia and fibula
Lateral view
Case #625.1
50 yr old female with short stature and R hip pain for years
Multiple hereditary exostoses
AP and lateral of right hip
AP and lat tibia
Proximal humeral x-rays
AP and lateral of femur
Lateral ankle
Oblique foot
Case #626
13 year femalemultiple exostosesforearm
Distal femur
Case #627
20 year malemultiple exostosesknee
Lateral view
Case #628
15 year male with multiple exostoses hips
Case #629
26 year female with multiple exostoses thorax & arms
Case #630
31 year malemultiple exostosesscapula & humerus
Scapular view
CT scan
Case #631
26 year malemultiple exostosesleg
ChondroblastoChondroblastomama
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
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.
CLASSIC Case #123
12 year female with chondroblastoma proximal tibia
Coronal post gad MRI
Sagittal T-2 MRI
Photomic
Case #632
26 year male with chondroblastoma prox tibia
Lateral view
Sagittal T-1 MRI
Sagittal T-2 MRI
Photomic
52 yr female with pain in knee for 1 yr
Case #632.1 Chondroblastoma pseudotumor (geode)
Cor T-1 T-2
Sag T-1
Axial T-2
Case #633
21 year female with chondroblastoma prox tibia
Case #634
16 year male with chondroblastoma distal femur
Lateral view
Coronal proton density MRI
Sagittal proton density MRI
Axial proton density MRI
Curettement of tumorfrom window inintercondylar notch
tumor
Photomic
Higher power
Case #635
18 year male chondroblastomadistal femur
CT scan
Photomic
Case #636
20 year male with chondroblastoma & ABC prox femur
Axial T-2 MRI
tumor
Coronal T-2 MRItumor
edema
Photomic
Post op x-ray with cement & DHS
Case #637
12 year female with chondroblastoma prox femoral epiphysis
CT scan
Another CT cut
Photomic
giant cells
cartilage
Case #638
13 year female with chondroblastoma prox fem epiphysis
Case #639
15 year male with chondroblastoma prox humerus
CT scan
Axial T-1 MRI
Coronal T-2 MRI
Macro section
tumor
Photomic
Photomic with giant cells
Case #640
15 year male with chondroblastoma proximal humerus
Coronal T-1 MRI
Coronal T-2 MRI
tumor
Photomic with giant cells
Another photomic
Case #641
24 year female chondroblastoma pelvis
CT scan
Another CT cut
Photomic
Case #642
21 year male with chondroblastoma ilium
CT scan
Another CT cut
T-2 MRI
tumor
Case #643
43 year female with chondroblastoma distal humerus
CT scan
Case #644
11 year male with chondroblastoma lateral malleolus
Sagittal T-1 MRI
Axial T-2 MRI
Photomic
Case #645
12 year femalechondroblastomadistal fibula
Case #646
18 year male with chondroblastoma talus
Lateral view
Chondromyxoid Chondromyxoid FibromaFibroma
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.
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.
CLASSICCase #124
11 year malechondromyxoid fibromaproximal tibia
Tomogram cut
Surgical exposure of tumor
Low power photomic
Higher power
giant cells
myxoid
Surgical curettement completed ready for bone graft
Placement of fibular strut and cancellous graft
Post op x-ray
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
Coronal T-1 MRI
Coronal PD FS MRI
Axial PD FS MRI
Axial Gad MRI
Case #647
12 year female with chondromyxoid fibroma tibia
Case #648
13 year female with chondromyxoid fibroma tibia
Case #649
25 year female chondromyxoid fibroma 1st metatarsal
Oblique view
Photomic
Photomic
Case #650
7 year femalechondromyxoid fibroma ulna
Photomic
Case #651
25 year female with chondromyxoid fibroma rib
Photomic
Case #651.1
60 year female with chondromyxoid fibroma distal sacrum
Sag T-1 Sag T-2
Axial T-1
Axial T-2
Coronal T-1
