an atlas of musculoskeletal oncology: volume 1

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Contents for Volume 1 osteoma--------------------------------Case astoma----------------------------------Case brous dysplasia-------------------------Case ng fibroma of jawbone----------------Case 10

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Page 1: An Atlas of Musculoskeletal Oncology: Volume 1

Contents for Volume 1

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

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Osteoid Osteoid OsteomaOsteoma

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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

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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.

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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

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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.

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19 yr old femaleOsteoid osteomafemur

painfulnidus

CLASSICCase #1

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Bone scan

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CT scan

nidus

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Macro section of nidus

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Photomic of nidus

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29 year maleosteoid osteomafemur

nidus

Case #2

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nidus

CT scan

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11 yr maleosteoid osteomafemur

Case #3

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Bonescan

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CT scan

nidus

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Photomic

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5 yr male osteoid osteoma femur

nidus

Case #4

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12 yr maleosteoid osteoma femur

Case #5

nidus

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10 yr maleosteoid osteomafemur

nidus

Case #6

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nidus

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CT scan

nidus

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30 yr femaleosteoid osteomafemur

nidus

Case #7

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Coronal T-1 MRI

nidus

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Axial PD MRI

edema

nidus

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Macrosection

nidus

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Case #8

23 yr femaleosteoid osteomafemur

nidus

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Bone Scan

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CT Scan

Nidus

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Case #9

12 yr maleosteoid osteomafemur nidus

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Macro section nidus

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Photomic

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Case #10

10 yr female with osteoid osteoma femoral neck

nidus

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Photomic

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One yr after curettement

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Case #11

25 yr male with osteoid osteoma femoral neck

nidus

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CT scan

nidus

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Case #12

6 yr male osteoid osteoma acetabulum

nidus

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nidus

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Bone scan

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Case #13

11 yr maleosteoid osteomadistal femur

nidus

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Bone scan

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Case #14

16 yr maleosteoid osteomadistal femur

nidus

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Case #15

6 yr maleosteoid osteomatibia

nidus

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Bone scan

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Excessivebone blockresection

nidus

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Macro section of nidus

nidus

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Photomic

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Post op x-ray

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Resultant fractureone year later

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Case # 16

10 yr maleosteoid osteomatibia

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Shaving down with osteotome

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Further shaving to find nidus

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Burred out nidus defect

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Case # 17

15 yr maleosteoid osteomatibia

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Lateral view

nidus

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Bone scan

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Coronal T-2 MRI

nidus

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Coronal Gad contrast MRI

nidus

edema

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T-1 axial MRI

nidus

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Photomic

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Photomic

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Case #17.1

52 year old male with ankle pain for 3 mos.

Osteoid osteoma

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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 #18

17 yr maleosteoid osteomafemur

nidus

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Grossspecimen

nidus

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Photomic

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Case #19

18 yr maleOsteoid osteoma tibia

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Case #20

18 yr femaleosteoid osteomatibia

nidus

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Case #21

12 yr male with parosteal osteoid osteoma tibia

nidus

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Case #22

17 yr maleosteoid osteomafibula

nidus

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Lateral view

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Case #23

26 year female with epiphyseal osteoidosteoma prox radius

nidus

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Case #24

16 yr femaleosteoid osteomaulna

nidus

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Bone scan

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Case #25

16 yr maleosteoid osteomahumerus

nidus

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Case # 26

14 yr maleosteoid osteomaL-4

nidus

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Bone scan

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Photomic

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Case # 27

15 yr maleosteoid osteomaL-3

nidus

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Case #28

46 yr maleosteoid osteomadorsal spine

nidus

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Bone scan

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Case #29

18 yr male with osteoid osteoma C-3

nidus

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Laminogram

nidus

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Bone scan

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Case #30

12 yr male with osteoid osteoma C-3 spine

nidus

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Case #31

25 yr maleosteoid osteomaC-5

nidus

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Case # 32

27 yr female with osteoid osteoma S-1

nidus

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Case #33

22 yr male with osteoid osteoma sacrum

nidus

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Case #34

18 yr maleosteoid osteomafinger

nidus

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X-ray

nidus

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AP x-ray

nidus

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Case #35

24 yr male with osteoid osteoma thumb

nidus

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Bone scan

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Case #36

19 yr female with osteoid osteoma finger

nidus

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Case #37

20 yr male with osteoid osteoma thumb

nidus

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Case #37.1

17 year female with dull aching pain index finger 1 yr

nidus

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Case #38

23 yr male with osteoid osteoma capitate

nidus

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Case #39

25 yr male with osteoid osteoma narvicular

nidus

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Case # 40

34 yr female with osteoid osteoma 2nd metatarsal

nidus

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CT scan

nidusdouble

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Axial T-2 MRI

edema

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Case #41

24 yr female with osteoid osteoma big toe

nidus

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Gross Specimen

nidus

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Photomic

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Photomic

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Case #42

Osteoid osteoma talus

nidus

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Photomic

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Case #43

Osteoid osteomapseudotumor

Brodie’s abscess13 yr femaletibia

nidus

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Gopher’s sign

tunnel

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Case # 44

Osteoid osteomapseudotumor

Brodie’s abscesstibia38 yr male

nidus

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Axial T-2 MRI

nidus

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Case #45

Osteoid osteoma pseudotumorstress fracture femoral neck 38 yr male

fractureline

pseudo-nidus

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Bone scan

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Case #46

Osteoid osteoma pseudotumor 33 year female

e

Early stressfracture femoralneck with pain for 3 mos

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Bone scan

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One month later

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Case #47

Osteoid osteoma pseudotumorstress fracture femoral neck 50 yr female

pseudonidus

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Axial T-2 MRI

pseudonidus

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Coronal T-2 MRI

fracture line

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Case #48

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

Stress fractureOO pseudotumor

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Sag T-1 PD Gad

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Case #49

Pseudo osteoid osteoma femur

Chronic stressperiostitis from adductor pull14 yr male

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CT scan

pseudonidus

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Bone scan

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Case #50

Pseudo osteoid osteoma femur

Bone island

38 yr male

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Bone scan

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CoronalT-1 MRI

pseudonidus

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OsteoblastoOsteoblastomama

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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

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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.

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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.

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CLASSICCase #51

26 yr maleosteoblastoma L-4

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Lateral view

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Bone scan

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CT scan

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Sagittal T-2 MRI

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Photomic

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Photomic

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5 yrs post op with spontaneous fusion

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AP x-ray 5 yrs post op

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Sagittal gad contrast MRI 5 yrs post op

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Axial gad contrast MRI 5 yrs post op

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Case #52

23 yr male with osteoid osteoma L-5

nidus

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CT scan

nidus

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Photomic

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Photomic

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Recurrence 8 mos post op laminectomy

Recurrence

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CT

Recurrence as an osteoblastoma

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ABC

Secondary ABCbehind osteoblastoma

osteoblastoma

Sagittal T-2 MRI

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Axial T-2 MRI

fluid-fluidlevel

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ABC histology

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ABC histology

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Osteoblastoma histology

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Osteoblastoma histology

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Post op posteriorresection and fusion

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AP x-raypost op fusion

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CT scan later shows anterior recurrence

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Post op anteriorresection and bonecement reconstruction

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Lateral x-ray showinganterior reconstructionwith cement

cement

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Post op CT scan showing cement reconstruction

cement

CD rods

bone graft

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Post op CT

cement

graft

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Case #53

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

surgical towel clip

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Appearance 4 months later with a 1.5 cm nidus

CT scan

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A curettement at this time revealed an osteoblastoma

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Myelogram 4 yrslater

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CT scan at same time and no pain

burned-outlesion

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Another CT cut

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Sagittal T-1 MRI at same time

Burnt outlesion

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Sagittal T-2 MRI same time

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T-2 MRI with residual ABC in inactive lesion

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Axial T-1 MRI same time

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Axial T-2 MRI with residual ABC component

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Case #54

7 yr femaleosteoblastoma L-2

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Bone scan

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T-2 MRI showshigh signal ABCposterior to low signalosteoblastoma

osteoblastoma

ABC

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SagittalT-2 MRI

osteoblastoma

ABC

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Osteoblastoma Photomic

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AP x-raypost op posteriorspinal fusion

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Lateral X-raypost op

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Case #55

14 yr femaleosteoblastoma L-5

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Oblique X-ray

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Bone scan

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CT scan

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CT scan

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Case #56

24 yr male with osteoblastoma L-3

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Lateral view

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Oblique view

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Opposite oblique

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Case #57

22 year male with osteoblastoma L-1 vertebra

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Oblique view

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Case #58

23yr male with burned out obteoblastoma L-4

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Case #59

14 yr maleosteoblastoma C-3

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Oblique view

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Opposite oblique

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post op posteriorfusion and recurrenttumor anterior

One year

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Anterior viewwith recurrence

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Post op anteriordebriedment andfusion

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Case #60

18 yr maleosteoblastomaC-7

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Oblique view

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Lateral view2 mo later

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AP view 1 mo later

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Resected nidus

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Photomic

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Photomic

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Case #61

CT scan

55 yr female with osteoblastoma C-6

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Bone scan

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Sagittal T-1 MRI

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Sagittal T-2 MRI

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Axial T-2 MRI

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Case #62

25 yr female with osteoblastoma C-6

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AP x-ray

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10 yrs later

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Lateral view 10 yrs later

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Photomic

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Photomic

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Case #63

CT scan

40 yr male with osteoblastoma C 5-6

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CT scan

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Sagittal T-2 MRI

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T-2 MRI showing bulge in floor ofvertebral canal

Sagittal

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Case #64

13 yr female with osteoblastoma C-6

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Lateral view

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Post op X-rayAfter anteriorinterbody fusion

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Case #65

14 yr female with osteoblastoma C-7

AP X-ray

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AP myelogram

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Arteriogram showingpressure on vertebralartery

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Post op debriedment

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Case #66

CT scan

43 yr female with osteoblastoma T-8

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Axial T-2 MRI

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Sagittal T-1 MRI

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Post op posteriorspine fusion

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28 yr male with osteoblastoma thoracic spine

Case #67

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CT scan

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Post op resection

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CT scan post op resection

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Case #68

21 yr male osteoblastoma S-1

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2 years later

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5 years later

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Lateral view 5 years later

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Photomic

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Photomic

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Case #69

8 yr male with ostoblastoma sacrum

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Case #70

26 yr female with osteoblastoma acetabulum

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Close up AP

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Photomic

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Photomic

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X-ray 3 mosfollowing THA

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Case #71

19 yr male with aggressive osteoblastoma acetabulum

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Bone scan

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Gross specimen from curettement

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Photomic

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Photomic

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Post op internalhemipelvectomy

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Recurrence 4 months later

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Ablative hemipelvectomy specimen

tumor

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Hemipelvectomy specimen

tumor

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Several years later with recurrent tumor on sacrum

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Lateral view of recurrence

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Case #72

38 yr male osteoblastomapelvis

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Frog lateral

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Post op x-ray following curettage and cementation

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Case #73

33 yr female with osteoblastoma acetabulum

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Axial T-1 MRI

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Coronal T-2 MRI

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Case #74

26 yr male with osteoblastoma acetabulum

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Frog lateral

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Case #75

24 yr male with osteoblastoma pelvis

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18 mos later

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Case #76

22 yr male with osteoblastoma ischium

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X-ray of resected specimen

nidus

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Case #77

26 yr male with osteoblastoma ilium

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CT scan

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Post op x-ray appearance after resection and cementation

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Case #78

23 yr maleosteoblastomafemur

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CT scan

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T-2 MRI

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Sagittal T-2 MRI

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Photomic

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Case #78.1

19 year male with tibial pain for 6 mos

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Axial

T-1 T-2

Gad +

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Coronal T-1 T-2 Gad

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X-ray 3 months post op

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Case #79

15 yr maleosteoblastomafemur

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Bone scan

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CT scan

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Axial T-2 MRI

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Coronal T-1MRI

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X-ray at time ofsurgery with markerin tumor defect

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Photomic of nidus

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Case #79.1

14 yr male with dull aching thigh pain for 1 year

CT scan Sclerosing osteomyelitis of Garre

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Coronal T-1 Coronal T-2

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Sagittal T-2 Axial T-2

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Case #80

13 yr male with osteoblastoma femoral neckfrog lateral view

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AP view

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Bone scan

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Case #81

22 yr male with osteoblastoma talus

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Sagittal T-1 MRI

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Coronal T-2 MRI

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Coronal T-2 MRI

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Photomic

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Case #82

25 yr maleosteoblastomatalus

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Lateral view

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Close up lateral

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Photomic

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Case #83

18 yr female with osteoblastoma talus

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Mortise view

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X-ray 1 yr after curettage

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Case #84

23 yr male with osteoblastoma talus

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Case #85

25 yr female with osteoblastoma os calcis

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Os calcis view

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X-ray several months with progression of disease

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Case #86

29 yr male with osteoblastoma os calcis

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Case #87

53 yr male with osteoblastoma distal humerus

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X-ray 3 years later

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tumor

edema

Axial T-2 MRI

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Coronal T-2 MRI

nidusedema

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Post op x-rayfollowing distalhumeral resectionand allograft reconstruction

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Case #88

9 yr femaleosteoblastomadistal humerus

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Coronal T-1 MRI

edema

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Axial T-2 MRI

tumor

edema

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Case #88.1

11 yr male with ABC response to osteoblastoma humerus

nidus

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Sag T-1

Sag T-2

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Axial T-2 MRI shows the multiloculated aneurysmal cysts

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Case #89

21 yr male with osteoblastoma ring finger

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AP and Lateral radiographs

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Gross specimen

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Photomic

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Case #90

18 yr male with osteoblastoma finger

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Case #91

21 yr male with osteoblastoma thumb

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Case #92

10 yr female with osteoblastoma finger tip

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AP X-ray

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Case #93

9 yr femaleosteoblastoma tibia

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AP x-ray

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Case #94

24 yr male with osteoblastoma fibula

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Lateral view

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Osteofibrous Osteofibrous DysplasiaDysplasia

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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

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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.

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CLASSICCase #95

9 yr male osteofibrous dysplasiatibia

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AP and lateral x-ray

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Photomic

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Case #96

6 year femaleosteofibrous dysplasiatibia

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Same case

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Case #97

16 year femaleosteofibrous dysplasiatibia

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Case #98

8 yr maleosteofibrous dysplasiatibia

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X-ray appearance3 yrs later

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8 yrs later with spontaneous healing

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Case #98.1

61 year old male with incidental finding in tibia for years

Burnt out osteofibrous dysplasia

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Bone scan

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Sag T-1 PD FS Gad

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Cor STIR Gad

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Axial T-1 T-2 FS

Gad

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Case #99

15 yr femaleosteofibrous dysplasiatibia

AP x-ray

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Lateral x-ray

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Case #100

Newborn with osteofibrous dysplasia tibia

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T-2 axial MRI

edema

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Photomic

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Photomic

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X-ray 1 yr later

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Case #101

Another newbornosteofibrous dysplasiatibia

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Another view

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Ossifying Ossifying Fibroma of Fibroma of

JawboneJawbone

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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.

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Case #102

5 yr female ossifying fibromamaxilla

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Maxillary view

tumor

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Tomogram cut

tumor

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CT scan

tumor

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Another cut

tumor

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Another cut

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Photomic

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Case #103

18 yr female with ossifying fibroma mandible

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Oblique view

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Photomic

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Closeup of alphabet-soup metaplastic bone

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Case #104

12 yr female with ossifying fibroma mandible

tumor

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Opposite ramus

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Case #105

14 yr female with ossifying fibroma mandible

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Case #106

Young adultosteoma skull

tumor

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Occipital view

tumor

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Lateral view

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Case #107

Cementoma mandible

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Oblique view