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