an atlas of musculoskeletal oncology: volume 1

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Contents for Volume 1

Osteoid osteoma--------------------------------Case 1-50 Osteoblastoma----------------------------------Case 51-94Osteofibrous dysplasia-------------------------Case 95-101Ossifying fibroma of jawbone----------------Case 102-107

Osteoid Osteoid OsteomaOsteoma

Osteoid Osteoma

The osteoid osteoma is the most common osteoid-forming tumorseen in the skeletal system, accounting for 10% of all benignbone tumors. It is more common in males than females with the peak incidence in the second decade of life. The lesions producesymptoms of dull, aching pain that is more severe at night. Thepain is relieved with anti-inflammatory medication such as aspirin or non-steroidal anti-inflammatory drugs (NSAID)that inhibit the high concentration of prostaglandins found in the nidus of the osteoid osteoma. The lesions occur typically inthe cortical structures of long bones such as the femur or tibia but can also be found in the small bones of the ankle, wrist or vertebral column. The lesion is characteristic for its dense,sclerotic response in the cortical bone resulting in a fusiform enlargement on the surface of the cortex as a response to thesmall inflammatory nidus or granuloma (measuring up to one

centimeter in diameter) in the central area of the scleroticresponse. If the lesion is located in a central medullary area,such as the metaphysis or on the very superficial surface of abone, the sclerotic response is less severe. If the nidus is located close to or within a joint structure, as in the femoralneck area, there will be a significant inflammatory synovitisin the adjacent joint that gives the clinical appearance of apyarthrosis. In the spine, the lesions are usually located in theposterior elements of the spine such as the laminae or pedicles.The lumbar spine is the most common location followed secondby the dorsal spine. Atypical secondary painful scoliosis willdevelop with the convexity seen on the side opposite the inflammatory lesion. The two most significant diagnostic imaging studies are a CTscan through the nidus area to identify its anatomic location and a bone isotope scan which is invariably positive, especially in asymptomatic lesion. It is the current feeling that the osteoid.

osteoma is a true benign neoplasm of bone in which a com-bination of osteoclastic and osteoblastic activity are seen. There are no inflammatory cells such as lymphocytes orplasma cells as would be seen in a Brodie’s abscess. Non-steroidal anti-inflammatory medications such as prostaglandininhibitors can be very effective in controlling the disablingsymptoms and helping the patient get thru a period of one ortwo years during which time the lesion will typically involuteinto a painless lesion that does not require surgery. However,if the patient is unable to live with the pain during this time,a surgical approach is indicated. Following exposure of the lesion, a high speed burr is used to burr into the inflammatorybone that is characterized by a pinkish hyperemia. When the nidus is found, a small curette is used to core the inflammatorynidus for pathological diagnosis and the cavity of the nidusis burred an additional 2 or 3 mm which usually results in a verydramatic relief of symptoms and a permanent cure from the

disease process. It is not necessary to remove the large dense sclerotic bone surrounding the nidus; doing so couldresult in a pathologic fracture following surgery. In the spine, a simple laminectomy is frequently carried out. If the lesion islocated in the pedicle a simple burring into the pedicle will relieve the patient of the inflammatory pain. More recently, relatively non-invasive techniques have beentried to remove the nidus of the osteoid osteoma by means of a CT guided burr placed through a protective trocar or by place-ment of a heat-producing radio frequency probe that, when properly positioned, can destroy the nidus through thermal coagulation. These techniques are particularly valuable for deep-seated lesions located in the acetabulum. For lesions located inthe pedicle of a vertebra one must be careful not to damage anadjacent spinal root or spinal cord.

19 yr old femaleOsteoid osteomafemur

painfulnidus

CLASSICCase #1

Bone scan

CT scan

nidus

Macro section of nidus

Photomic of nidus

29 year maleosteoid osteomafemur

nidus

Case #2

nidus

CT scan

11 yr maleosteoid osteomafemur

Case #3

Bonescan

CT scan

nidus

Photomic

5 yr male osteoid osteoma femur

nidus

Case #4

12 yr maleosteoid osteoma femur

Case #5

nidus

10 yr maleosteoid osteomafemur

nidus

Case #6

nidus

CT scan

nidus

30 yr femaleosteoid osteomafemur

nidus

Case #7

Coronal T-1 MRI

nidus

Axial PD MRI

edema

nidus

Macrosection

nidus

Case #8

23 yr femaleosteoid osteomafemur

nidus

Bone Scan

CT Scan

Nidus

Case #9

12 yr maleosteoid osteomafemur nidus

Macro section nidus

Photomic

Case #10

10 yr female with osteoid osteoma femoral neck

nidus

Photomic

One yr after curettement

Case #11

25 yr male with osteoid osteoma femoral neck

nidus

CT scan

nidus

Case #12

6 yr male osteoid osteoma acetabulum

nidus

nidus

Bone scan

Case #13

11 yr maleosteoid osteomadistal femur

nidus

Bone scan

Case #14

16 yr maleosteoid osteomadistal femur

nidus

Case #15

6 yr maleosteoid osteomatibia

nidus

Bone scan

Excessivebone blockresection

nidus

Macro section of nidus

nidus

Photomic

Post op x-ray

Resultant fractureone year later

Case # 16

10 yr maleosteoid osteomatibia

Shaving down with osteotome

Further shaving to find nidus

Burred out nidus defect

Case # 17

15 yr maleosteoid osteomatibia

Lateral view

nidus

Bone scan

Coronal T-2 MRI

nidus

Coronal Gad contrast MRI

nidus

edema

T-1 axial MRI

nidus

Photomic

Photomic

Case #17.1

52 year old male with ankle pain for 3 mos.

Osteoid osteoma

Sag T-1 T-2 Gad

Axial T-1 T-2

Gad

Case #18

17 yr maleosteoid osteomafemur

nidus

Grossspecimen

nidus

Photomic

Case #19

18 yr maleOsteoid osteoma tibia

Case #20

18 yr femaleosteoid osteomatibia

nidus

Case #21

12 yr male with parosteal osteoid osteoma tibia

nidus

Case #22

17 yr maleosteoid osteomafibula

nidus

Lateral view

Case #23

26 year female with epiphyseal osteoidosteoma prox radius

nidus

Case #24

16 yr femaleosteoid osteomaulna

nidus

Bone scan

Case #25

16 yr maleosteoid osteomahumerus

nidus

Case # 26

14 yr maleosteoid osteomaL-4

nidus

Bone scan

Photomic

Case # 27

15 yr maleosteoid osteomaL-3

nidus

Case #28

46 yr maleosteoid osteomadorsal spine

nidus

Bone scan

Case #29

18 yr male with osteoid osteoma C-3

nidus

Laminogram

nidus

Bone scan

Case #30

12 yr male with osteoid osteoma C-3 spine

nidus

Case #31

25 yr maleosteoid osteomaC-5

nidus

Case # 32

27 yr female with osteoid osteoma S-1

nidus

Case #33

22 yr male with osteoid osteoma sacrum

nidus

Case #34

18 yr maleosteoid osteomafinger

nidus

X-ray

nidus

AP x-ray

nidus

Case #35

24 yr male with osteoid osteoma thumb

nidus

Bone scan

Case #36

19 yr female with osteoid osteoma finger

nidus

Case #37

20 yr male with osteoid osteoma thumb

nidus

Case #37.1

17 year female with dull aching pain index finger 1 yr

nidus

Case #38

23 yr male with osteoid osteoma capitate

nidus

Case #39

25 yr male with osteoid osteoma narvicular

nidus

Case # 40

34 yr female with osteoid osteoma 2nd metatarsal

nidus

CT scan

nidusdouble

Axial T-2 MRI

edema

Case #41

24 yr female with osteoid osteoma big toe

nidus

Gross Specimen

nidus

Photomic

Photomic

Case #42

Osteoid osteoma talus

nidus

Photomic

Case #43

Osteoid osteomapseudotumor

Brodie’s abscess13 yr femaletibia

nidus

Gopher’s sign

tunnel

Case # 44

Osteoid osteomapseudotumor

Brodie’s abscesstibia38 yr male

nidus

Axial T-2 MRI

nidus

Case #45

Osteoid osteoma pseudotumorstress fracture femoral neck 38 yr male

fractureline

pseudo-nidus

Bone scan

Case #46

Osteoid osteoma pseudotumor 33 year female

e

Early stressfracture femoralneck with pain for 3 mos

Bone scan

One month later

Case #47

Osteoid osteoma pseudotumorstress fracture femoral neck 50 yr female

pseudonidus

Axial T-2 MRI

pseudonidus

Coronal T-2 MRI

fracture line

Case #48

20 year old male with pre-tibial pain for 3 months

Stress fractureOO pseudotumor

Sag T-1 PD Gad

Case #49

Pseudo osteoid osteoma femur

Chronic stressperiostitis from adductor pull14 yr male

CT scan

pseudonidus

Bone scan

Case #50

Pseudo osteoid osteoma femur

Bone island

38 yr male

Bone scan

CoronalT-1 MRI

pseudonidus

OsteoblastoOsteoblastomama

Osteoblastoma The so-called osteoblastoma is considered by most to be a largeor giant form of the similar clinical entity known as the osteoidosteoma. As with the osteoid osteoma, it is found in children andyoung adults, more often in males than females. It is less commonthan the osteoid osteoma, representing approximately 1% of allbone tumors. It is found mostly in metaphyseal areas of long boneand in the posterolateral elements of the axial skeleton wherenearly 50% of the lesions are identified. Radiographically these lesions are more osteolytic than the osteoid osteoma and have acentral nidus that measures more than 1.5 cm in diameter with less reactive sclerosis seen at the periphery than an osteoid osteoma. It is not unusual for an aneurysmal component to beassociated with the osteoblastoma, similar to the reaction seenadjacent to chondroblastomas and giant cell tumors. The micro-scopic appearance of the nidus material is almost identical to

that of the osteoid osteoma and under higher power cannot bedifferentiated by even the most competent pathologists. The major concern is a microscopic appearance similar to an osteo-sarcoma and, for this reason, multiple samples must be evaluatedin order to establish the correct diagnosis in cases where these lesions arise from the metaphyseal areas of long bone. In the spinal area, the osteoblastoma is typically located in theposterolateral elements where it can cause problems with spinal cord or nerve root compression that may require aggressive surgical decompression and even spinal stabilization. On occasionan osteoblastoma will spontaneously convert into an osteo-sacoma, especially if it has been treated with radiation therapy. The primary treatment for the osteoblastoma is a surgical one witha fairly aggressive curettement of the lesion. There is no particularreason to take wide margins because the recurrence rate is quite low and in some cases the lesions resolve spontaneously with out any surgery at all, similar to what occurs with osteoid osteomas.

A variant of the osteoblastoma, the so-called aggressive or“malignant” osteoblastoma, is a clinical entity halfway between theclassic benign osteoblastoma and a full blown malignant osteo-sarcoma. This “malignant” osteoblastoma appears and behaves clinically at the local site like an osteosarcoma but has no potential to metastasize to distant parts. In this situation, local treatment must be more aggressive and probably would require a wide localresection to avoid a local recurrence because radiation therapy or even systemic chemotherapy is not effective for this aggressive but benign entity.

CLASSICCase #51

26 yr maleosteoblastoma L-4

Lateral view

Bone scan

CT scan

Sagittal T-2 MRI

Photomic

Photomic

5 yrs post op with spontaneous fusion

AP x-ray 5 yrs post op

Sagittal gad contrast MRI 5 yrs post op

Axial gad contrast MRI 5 yrs post op

Case #52

23 yr male with osteoid osteoma L-5

nidus

CT scan

nidus

Photomic

Photomic

Recurrence 8 mos post op laminectomy

Recurrence

CT

Recurrence as an osteoblastoma

ABC

Secondary ABCbehind osteoblastoma

osteoblastoma

Sagittal T-2 MRI

Axial T-2 MRI

fluid-fluidlevel

ABC histology

ABC histology

Osteoblastoma histology

Osteoblastoma histology

Post op posteriorresection and fusion

AP x-raypost op fusion

CT scan later shows anterior recurrence

Post op anteriorresection and bonecement reconstruction

Lateral x-ray showinganterior reconstructionwith cement

cement

Post op CT scan showing cement reconstruction

cement

CD rods

bone graft

Post op CT

cement

graft

Case #53

26 yr male with osteoblastoma L-4 following a laminectomy curettement three years previous for an osteoid osteoma

surgical towel clip

Appearance 4 months later with a 1.5 cm nidus

CT scan

A curettement at this time revealed an osteoblastoma

Myelogram 4 yrslater

CT scan at same time and no pain

burned-outlesion

Another CT cut

Sagittal T-1 MRI at same time

Burnt outlesion

Sagittal T-2 MRI same time

T-2 MRI with residual ABC in inactive lesion

Axial T-1 MRI same time

Axial T-2 MRI with residual ABC component

Case #54

7 yr femaleosteoblastoma L-2

Bone scan

T-2 MRI showshigh signal ABCposterior to low signalosteoblastoma

osteoblastoma

ABC

SagittalT-2 MRI

osteoblastoma

ABC

Osteoblastoma Photomic

AP x-raypost op posteriorspinal fusion

Lateral X-raypost op

Case #55

14 yr femaleosteoblastoma L-5

Oblique X-ray

Bone scan

CT scan

CT scan

Case #56

24 yr male with osteoblastoma L-3

Lateral view

Oblique view

Opposite oblique

Case #57

22 year male with osteoblastoma L-1 vertebra

Oblique view

Case #58

23yr male with burned out obteoblastoma L-4

Case #59

14 yr maleosteoblastoma C-3

Oblique view

Opposite oblique

post op posteriorfusion and recurrenttumor anterior

One year

Anterior viewwith recurrence

Post op anteriordebriedment andfusion

Case #60

18 yr maleosteoblastomaC-7

Oblique view

Lateral view2 mo later

AP view 1 mo later

Resected nidus

Photomic

Photomic

Case #61

CT scan

55 yr female with osteoblastoma C-6

Bone scan

Sagittal T-1 MRI

Sagittal T-2 MRI

Axial T-2 MRI

Case #62

25 yr female with osteoblastoma C-6

AP x-ray

10 yrs later

Lateral view 10 yrs later

Photomic

Photomic

Case #63

CT scan

40 yr male with osteoblastoma C 5-6

CT scan

Sagittal T-2 MRI

T-2 MRI showing bulge in floor ofvertebral canal

Sagittal

Case #64

13 yr female with osteoblastoma C-6

Lateral view

Post op X-rayAfter anteriorinterbody fusion

Case #65

14 yr female with osteoblastoma C-7

AP X-ray

AP myelogram

Arteriogram showingpressure on vertebralartery

Post op debriedment

Case #66

CT scan

43 yr female with osteoblastoma T-8

Axial T-2 MRI

Sagittal T-1 MRI

Post op posteriorspine fusion

28 yr male with osteoblastoma thoracic spine

Case #67

CT scan

Post op resection

CT scan post op resection

Case #68

21 yr male osteoblastoma S-1

2 years later

5 years later

Lateral view 5 years later

Photomic

Photomic

Case #69

8 yr male with ostoblastoma sacrum

Case #70

26 yr female with osteoblastoma acetabulum

Close up AP

Photomic

Photomic

X-ray 3 mosfollowing THA

Case #71

19 yr male with aggressive osteoblastoma acetabulum

Bone scan

Gross specimen from curettement

Photomic

Photomic

Post op internalhemipelvectomy

Recurrence 4 months later

Ablative hemipelvectomy specimen

tumor

Hemipelvectomy specimen

tumor

Several years later with recurrent tumor on sacrum

Lateral view of recurrence

Case #72

38 yr male osteoblastomapelvis

Frog lateral

Post op x-ray following curettage and cementation

Case #73

33 yr female with osteoblastoma acetabulum

Axial T-1 MRI

Coronal T-2 MRI

Case #74

26 yr male with osteoblastoma acetabulum

Frog lateral

Case #75

24 yr male with osteoblastoma pelvis

18 mos later

Case #76

22 yr male with osteoblastoma ischium

X-ray of resected specimen

nidus

Case #77

26 yr male with osteoblastoma ilium

CT scan

Post op x-ray appearance after resection and cementation

Case #78

23 yr maleosteoblastomafemur

CT scan

T-2 MRI

Sagittal T-2 MRI

Photomic

Case #78.1

19 year male with tibial pain for 6 mos

Axial

T-1 T-2

Gad +

Coronal T-1 T-2 Gad

X-ray 3 months post op

Case #79

15 yr maleosteoblastomafemur

Bone scan

CT scan

Axial T-2 MRI

Coronal T-1MRI

X-ray at time ofsurgery with markerin tumor defect

Photomic of nidus

Case #79.1

14 yr male with dull aching thigh pain for 1 year

CT scan Sclerosing osteomyelitis of Garre

Coronal T-1 Coronal T-2

Sagittal T-2 Axial T-2

Case #80

13 yr male with osteoblastoma femoral neckfrog lateral view

AP view

Bone scan

Case #81

22 yr male with osteoblastoma talus

Sagittal T-1 MRI

Coronal T-2 MRI

Coronal T-2 MRI

Photomic

Case #82

25 yr maleosteoblastomatalus

Lateral view

Close up lateral

Photomic

Case #83

18 yr female with osteoblastoma talus

Mortise view

X-ray 1 yr after curettage

Case #84

23 yr male with osteoblastoma talus

Case #85

25 yr female with osteoblastoma os calcis

Os calcis view

X-ray several months with progression of disease

Case #86

29 yr male with osteoblastoma os calcis

Case #87

53 yr male with osteoblastoma distal humerus

X-ray 3 years later

tumor

edema

Axial T-2 MRI

Coronal T-2 MRI

nidusedema

Post op x-rayfollowing distalhumeral resectionand allograft reconstruction

Case #88

9 yr femaleosteoblastomadistal humerus

Coronal T-1 MRI

edema

Axial T-2 MRI

tumor

edema

Case #88.1

11 yr male with ABC response to osteoblastoma humerus

nidus

Sag T-1

Sag T-2

Axial T-2 MRI shows the multiloculated aneurysmal cysts

Case #89

21 yr male with osteoblastoma ring finger

AP and Lateral radiographs

Gross specimen

Photomic

Case #90

18 yr male with osteoblastoma finger

Case #91

21 yr male with osteoblastoma thumb

Case #92

10 yr female with osteoblastoma finger tip

AP X-ray

Case #93

9 yr femaleosteoblastoma tibia

AP x-ray

Case #94

24 yr male with osteoblastoma fibula

Lateral view

Osteofibrous Osteofibrous DysplasiaDysplasia

Osteofibrous Dysplasia

Osteofibrous dysplasia is a rare condition occurring in children less than 10 years of age. This benign lesion has radiographic and microscopic features similar to fibrousdysplasia of the tibia, the main difference being the presenceof more heavily ossified tissue at the periphery of the lesiongiving it a soap-bubbly appearance on x-ray. Microscopicallyit has a similar “alphabet-soup” metablastic bone appearance in a benign fibrous tissue stroma but with the additional featureof heavy osteoblastic rimming of the trabeculae not seen in fibrous dysplasia. It typically arises from the anterior cortex of the tibia at mid shaft and causes a progressive anterior bowing of the tibia over time, creating a cosmetic deformity associated with pain of a dull, aching nature. The radiographic appearanceIs very similar to and impossible to separate from that of theMalignant adamantinoma that also affects the tibia in children

and, for this reason, a biopsy must be performed to rule out themalignant possibility. Occasionally osteofibrous dysplasia of thetibia can progress gradually into a well-differentiated formof the adamantinoma, which will have the microscopic features of both osteofibrous dysplasia and adamantinomain the same lesion. As far as treatment is concerned, early surgical debriedment and bone grafting before age 15 years results in a high local recurrence rate and thus should be discouraged until the child reaches full bone maturity at which time a definitive debriedment and bone grafting procedurecan be performed without significant risk of recurrence. Thislesion occurs bilaterally in a very small percentage of cases and can also involve the fibula on the same side. This processin newborns appears to be more osteolytic and destructive in nature.

CLASSICCase #95

9 yr male osteofibrous dysplasiatibia

AP and lateral x-ray

Photomic

Case #96

6 year femaleosteofibrous dysplasiatibia

Same case

Case #97

16 year femaleosteofibrous dysplasiatibia

Case #98

8 yr maleosteofibrous dysplasiatibia

X-ray appearance3 yrs later

8 yrs later with spontaneous healing

Case #98.1

61 year old male with incidental finding in tibia for years

Burnt out osteofibrous dysplasia

Bone scan

Sag T-1 PD FS Gad

Cor STIR Gad

Axial T-1 T-2 FS

Gad

Case #99

15 yr femaleosteofibrous dysplasiatibia

AP x-ray

Lateral x-ray

Case #100

Newborn with osteofibrous dysplasia tibia

T-2 axial MRI

edema

Photomic

Photomic

X-ray 1 yr later

Case #101

Another newbornosteofibrous dysplasiatibia

Another view

Ossifying Ossifying Fibroma of Fibroma of

JawboneJawbone

Ossifying Fibroma of Jawbone

Ossifying fibroma of jawbone is a benign condition similar toosteofibrous dysplasia of the tibia in that histologically they arealmost identical. It is also a condition that is related to fibrous dysplasia, which can have a very similar radiographic appearance,but in the case of fibrous dysplasia of jawbone osteoblastic rimming of the trabecular bone is not seen under microscopicevaluation. As with fibrous dysplasia, this condition is found in children and is almost always an asymptomatic process resulting in cosmetic deformation of the maxilla or mandible, usually on one side but can be bilateral. The condition can result in significant malocclusion of the teeth because of deformationof the subadjacent alveolar ridge or maxillary structures. It alsocan create problems with normal drainage from the air sinuses in the maxilla that can lead to infection requiring surgical drainage.Most of the surgical treatment for this condition is for cosmeticdeformity.

Case #102

5 yr female ossifying fibromamaxilla

Maxillary view

tumor

Tomogram cut

tumor

CT scan

tumor

Another cut

tumor

Another cut

Photomic

Case #103

18 yr female with ossifying fibroma mandible

Oblique view

Photomic

Closeup of alphabet-soup metaplastic bone

Case #104

12 yr female with ossifying fibroma mandible

tumor

Opposite ramus

Case #105

14 yr female with ossifying fibroma mandible

Case #106

Young adultosteoma skull

tumor

Occipital view

tumor

Lateral view

Case #107

Cementoma mandible

Oblique view

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