an atlas of musculoskeletal oncology: volume 3
DESCRIPTION
TRANSCRIPT
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Volume 3
Osteosarcoma Variants
Hemorrhagic osteosarcoma------------Case 110 & 499-503Parosteal osteosarcoma-----------------Case111 & 504-510Periosteal osteosarcoma----------------Case 112 & 511-517Pagetic sarcoma-------------------------Case 113 & 518-528Low grade intramedullary OGS------Case 114 & 528.1-530Radiation induced OGS---------------Case 115 & 531-537Multicentric osteosarcoma------------Case 116 & 538-542Soft tissue osteosarcoma--------------Case 118 & 543-545Intracortical osteosarcoma------------Case 119 & 546-547
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Osteogenic Osteogenic SarcomaSarcomaVariantsVariants
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HemorrhagicHemorrhagicOsteogenic Osteogenic SarcomaSarcoma
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Hemorrhagic (Telangiectatic) Osteosarcoma The hemorrhagic (OGS), an extremely lytic and hemorrhagicvariant of the osteosarcoma, presents in the same age group andlocation as a classic osteosarcoma but has a radiographic appearance almost identical to that of an aggressive aneurysmalbone cyst, making for a very difficult differential consideration for the radiologist. At the time of biopsy the tumor is very hemorrhagic and has the gross appearance of an aneurysmalbone cyst. Even microscopically, many areas of the hemorrhagicOGS will have the appearance of an aneurysmal bone cyst withonly an occasional mitotic figure. For this reason, it is very important for the surgeon who performs the biopsy to obtainan adequate specimen with good sampling by means of an open biopsy as apposed to a simple needle biopsy. The microscopicfeatures of the hemorrhagic OGS is a large number of benign-appearing giant cells and thus the terminology “giant cell rich”
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osteosarcoma that is used by many pathologists. There is verylittle evidence of osteoblastic acitivity in the hemorrhagic OGS and, because it is so lytic in character, it frequently presents with a pathologic fracture early in the course of the disease and with thatcome potential problems for the treating orthopedic surgeon whomust deal with the major contamination that occurs during the fracture. Because of the possible complications, one might consider an early limb salvage procedure before the fracture occurs. It was once felt that the prognosis for the hemorrhagic OGSwas worse than that of the classic OGS because of its lytic dest-uctive nature. However, since the advent of systemic chemotherapy,the prognosis for survival is no different than for a classic OGS.
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CLASSICCase #110
23 year malehemorrhagic OGSproximal humerus
Aneurysmal lesion
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Coronal T-1 MRI
hemorrhagictumor
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Coronal T-1 MRIthru path fracture
tumor
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Resected tumor cut in path lab
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Photomic showing giant cells and malignant cells
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Photomic showing hemorrhagic response
blood
osteoid
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Post op x-ray withalloprostheticreconstruction
Neer
allograft
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18 year followup x-rays
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Case #499
15 year male hemorrhagic OGSdistal femur
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Lateral view
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Bone scan
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Sagittal T-2 MRI
tumor
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hemorrhagictumor
Coronal T-2 MRI
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Axial T- 2 MRI
tumor
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Photomic
blood
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3 yrs post op Compresstotal knee reconstruction
CPS
osseo-integration
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Case #500
19 year malehemorrhage OGSproximal femur
Looks like ABC
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Lateral view
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Initial biopsy reveals aneurysmal bone cyst
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6 weeks latershows lysis ofouter shell
Repeat biopsyreveals hemorrhagic OGS
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Hip disarticulation specimen
tumor
femoral head
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2nd biopsy Photomic
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Case #501
6 year femalepath fracture thruunicameral bone cyst
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Lateral view
cysticlesion
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7 weeks aftersteroid injection
cyst
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1 month later andprogressive lyticdestruction
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Biopsy here shows hemorrhagic OGS
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Case #501.1
19 year old male with acute onset of pain 2 wksago in right hip
Telangiectatic OGS
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PO 1 mo
2 mo 3 mo
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Cor T-1 T-2
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Axial T-1 T-2
Gad
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Sag T-2 Gad
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Case #502
4 year malelooks likeunicameral bone cyst
cysticlesion
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Progressive lysisafter steroid injection
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2 months laterwith progressivelysis and lookingmalignant
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Biopsy reveals hemorrhagic OGS
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Clinical appearance before shoulder disarticulation
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Case #503
17 year femalehemorrhagic OGSC-3
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AP view
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CT scan
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Photomic
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6 years later withspontaneous fusionand no tumor
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ParostealParostealOstogenic Ostogenic SarcomaSarcoma
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Parosteal Osteosarcoma The parosteal (OGS) is a low grade variant arising from the surfaceof a long bone that presents as an exophytic mass with dense fibro-osseous tissue. It carries an excellent five year survival prognosis of 85% and accounts for about 4% of all osteosarcomas. This tumor has very little, if any, medullary involvement which clearlyseparates it from the classic OGS. It is seen more commonly in females than males and is found in a slightly older age groupthan the classic OGS. By far the most common location for this tumor is in the posterior aspect of the distal femur where it is frequently presents with minimal symptoms of pain but with apalpable tumor mass that might have been present many years before medical advise was sought. Histologically, this tumor has avery low mitotic index and in many cases can be confused witha normal healing fracture callous with occasional areas of cartilagebeing seen. Because this tumor is extremely low grade, it is not
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responsive to adjuvant therapy such as chemotherapy orradiation therapy. The treatment consists of a wide surgical resection that must have safe margins, otherwise the recurrence rate will be quite high. Recurrence can occur 10 to 15 years afterthe surgery. In many cases the lesion can be resected without sacrificing the adjacent joint, but in larger lesions the best approach is a total joint replacement similar to that used for the classic OGS.
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CLASSICCase #111
32 year maleparosteal OGSdistal femur
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AP view
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Bone scan
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Sagittal T-1 MRI
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Sagittal STIR MRI
tumor
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Axial T-1 MRI
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Axial STIR MRI
tumor
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Photomic
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Higher power
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Case #504
18 year maleparosteal OGSdistal femur
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AP view
tumor
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Bone scan
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Axial T-2 MRI
tumor
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Sagittal T-2 MRI
tumor
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Macro section
tumor
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Photomic
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Compress total knee reconstruction 2 years later
osseointegration
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10 years later withrecurrence as a highgrade dedifferentiatedparosteal OGS
tumor
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Another view
tumor
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Photomic of recurrence
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Close up of osseointegration ofCompress implant
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Case #505
32 year maleparosteal OGSproximal humerus
tumor
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Axillary view
tumor
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CT scan
tumor
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Amputation specimen cut in path lab
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Photomic
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Case #506
25 year male parosteal OGSdistal femur
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Distal femoralresection specimen
tumor
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Cut specimen in path lab
tumor
fattymarrow
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Case #507
13 year maleparosteal OGSmid femur
AP view
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Lateral view
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CT scan
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Segmental resection specimen
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Autoclaved bone replaced with IM nail fixation
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Post op x-ray2 years later
autoclavedbone
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Case #508
17 year male with parosteal OGS mid tibia
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Lateral x-ray
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CT scan
tumor
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Bone scan
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Segmental resectionmid tibial lesion
biopsysite
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Surgical specimen cut in path lab
tumor
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Allograft reconstruction over IM nail
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X-ray 1 year later
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Case #509
41 year femaleparosteal OGShumerus
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CT scan
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Resected cut specimen in path lab
tumor
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Case #509.1
10/06 3/07
17 year male with football injury 9/06
Parosteal OGS pseudotumor M.O.
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Sag T-1 Sag Gad
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Axial T-1
Axial Gad
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Case #510
32 year female with high grade parosteal OGS femur
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Macro section
tumor
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Photomic
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PeriostealPeriostealOsteogenic Osteogenic SarcomaSarcoma
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Periosteal Osteosarcoma
The periosteal osteosarcoma is another surface type OGS thattends to be low grade to intermediate with potential for pulmonarymetastasis in about 25% of cases. It accounts for 2% of all OGS’sand, compared to the parosteal OGS, has a much higher percentage of cartilagenous tissue in the tumor to the point where it can looklike a periosteal chondroma but with a much higher mitotic index. One must find a few areas of osteoid formation to classify this as a periosteal OGS. It is seen typically in the second decade of lifeand is slightly more common in females than males. It arises from long bones, typically the tibia or femur, and has a higher incidence in diaphyseal bone than does OGS. Like the parosteal OGS, this lesion is treated by aggressive wide local resection that often can spare the adjacent joint. In most cases chemotherapy is not utilizedunless the clinical picture is more aggressive than usual.
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CLASSIC Case #112
15 year female with periosteal OGS tibia
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CT scan
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Sagittal CT scan
tumor
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Axial T-2 MRI
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Photomic
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Post op x-ray followingwide resection and allograft reconstruction
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Case #511
30 year male with periosteal OGS prox tibia
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CT scan
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Sagittal T-2 MRI
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Axial T-1 MRI
tumor
edema
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Wide resectionproximal tibia tumor
bulge
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Cut specimenin path lab
tumor
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Photomic
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Proximal tibia resected ready for reconstruction
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Post op x-ray withalloprostheticreconstruction
TKA
allograft
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Case # 512
9 year femaleperiosteal OGStibia
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AP x-ray
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Lateral view
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Cut specimen in path lab followingAK amputation
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Photomic
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Higher power
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Case #513
14 year maleperiosteal OGS
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Sagittal T-2 MRI
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Axial T-1 MRI
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Axial T-2 MRI
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Case #514
26 year femaleperiosteal OGSdistal femur
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Lateral view
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X-ray 10 yearsfollowing wideresection and cementedprosthetic reconstruction
stressshielding
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Case #515
12 year femaleperiosteal OGStibia
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Bone scan
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Axial T-1 MRI
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Photomic
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Case #516
15 year maleperiosteal OGSdistal tibia
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CT scan
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Bone scan
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Photomic
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Case #517
39 year femaleperiosteal OGSpseudotumor
In fact is a Nora’slesion orbizarre parostealosteochondromatousproliferation (BPOP)
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Bone scan
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CT scan
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Axial Gad contrast MRI
edema
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Sagittal PD
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Sagittal T-2 MRI
edema
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Axial gad contrast MRI
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Pagetic Pagetic SarcomaSarcoma
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Pagetic Sarcoma
There are multiple diseases of the skeletal system that can resultin a secondary form of OGS most likely brought about by a secondmutation at a later age in a patient with chronic benign disease.These diseases include Paget’s disease, osteoblastoma, fibrous dysplasia, benign giant cell tumor of bone, bone infarcts, and chronic osteomyelitis. The most common of this group is Paget’sdisease, a non-specific inflammatory osteomyelitis of bone seen in older patients that may be induced by a virus infection. Approx-imately 1% of patients with Paget’s disease can go on to PageticOGS which accounts for 3% of all OGS. The most common location for this secondary form of OGS is in the humerus, followed next by the pelvis and femur. The patients typically have a long history of dull, aching pain from their inflammatory Paget’sdisease but then suddenly develop an acute new pain in the area ofthe older pain with x-ray evidence of recent lysis and destruction
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of old Pagetic reactive bone. The prognosis for survival in thissecondary form of OGS is extremely poor with only about 8%surviving, mainly because the older age group in which the diseaseoccurs make it impractical to implement the aggressive protocols used in younger age groups.
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CLASSIC Case #113
80 year female with Pagetic sarcoma pelvis
tumor
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Bone scan
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Axial T-2 MRI
tumor
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Photomic
osteoid
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Post op internal hemipelvectomy
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Case#518
83 year femalePagetic sarcomapelvis
tumor
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CT scan
tumor
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Another CT cut
tumor
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Photomic
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Case #519
85 year female with Paget’s disease pelvis
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Same disease in lumbar spine
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Same disease in skull
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Same disease in tibia
Advancing osteolytic wedge
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Same patient withPagetic sarcomahumerus
tumor
old Paget’s
new
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Macro sectionfrom amputationspecimen tumor
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Photomic
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Post op x-ray following forequarter amputation
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Case # 520
73 year femalePagetic sarcoma skullready for resection
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Lateral view of skull
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Occipital view
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Tangential view
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tumor
Resected specimen cut in path lab
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Photomic
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Case #521
82 year male Pagetic sarcomadistal humerus
old Paget’swith priorfracture
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Close up of new tumor
tumor
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Photomic
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Case #522
80 year femalePagetic sarcoma distal humerus
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Case #523
84 male with multi focal Pagetic sarcoma
femur
humerus
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Case #524
83 year malePagetic sarcomafemur
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Case #525
60 year malePagetic sarcomafemur
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Lateral view
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Photomic
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Photomic
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Case #526
78 female Pagetic sarcomaproximal tibia
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Lateral view
tumor
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Case #527
92 year malePagetic sarcoma tibia
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Case #528
78 year femalePagetic sarcomalumbar spine
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Low Grade Low Grade IntramedullaryIntramedullary
Osteogenic Osteogenic SarcomaSarcoma
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Low Grade Intramedullary OGS
Low grade intramedullary OGS is another rare low grade fibro-osseous variant of OGS that is unique because it is totally confinedwithin the cortical anatomy of a long bone, most typically around the knee joint. It is found in an older age group than the classic OGS and is typically seen between the ages of 15 and 55 years;it affects males and females equally. The radiologic picture is that of a diffuse sclerotic change within the metaphysis of the long bone with no periosteal response or lytic destruction of the corticalanatomy. The smoky appearance of metaphyseal bone suggests the diagnosis of chronic osteomyelitis or perhaps fibrous dysplasia.Microscopically, the tumor has a histological appearance similarto parosteal OGS and because of this carries the same excellent prognosis for survival as we see in parosteal sarcoma. Likewise, treatment is similar without the use of chemotherapy or radiation.These lesions must be treated with complete wide resection that frequently involves a TKA, similar as in the classic OGS.
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CLASSICCase #114
63 year femaleintramedullary OGSdistal femur
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Lateral view
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Bone scan
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CT scan
tumor
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Macro section fromresected specimen
tumor
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Photomic
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Photomic
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Case #528.1
51 year femalelow grade intramedullary OGSdistal femur
tumor
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Bone scan
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Coronal T-1 MRI
tumor
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Axial T-1 MRI
tumor
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Resected distal femur cut in path lab
tumor
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Photomic
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Case #529
32 year femalelow grade intramedullary OGSdistal femur
tumor
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Lateral view
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CT scan
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Photomic
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Case #530
56 year malelow gradeintramedullary OGSdistal tibia
tumor
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Lateral view tumor
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Bone scan
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Photomic
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Radiation-Radiation-inducedinduced
Osteogenic Osteogenic SarcomaSarcoma
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Radiation-induced Osteosarcoma
One of the most malignant forms of OGS is the secondary typeinduced by radiation therapy, usually over 3000 rads, for some type of either benign or malignant disease process in the past. One of the most common types of radiation-induced OGS is seen in patients with breast cancer who receive local radiation following radical mastectomy and than develop OGS in the shoulder girdle area. Other malignant diseases that can result in OGS after radiation therapy include Ewing’s sarcoma and lymphomas. Benign diseases that can result in OGS from radiation therapy include GCT,ABC, and fibrous dysplasia. Theaverage delay for the occurrence of secondary OGS is 15 years,with a range from 3 to 55 years. The prognosis for this variant isextremely poor, similar to Pagetic OGS. It has a very high rate of metastasis to the lung for which chemotherapy is not very effective.
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CLASSICCase #115
33 year femaleradiation-inducedsarcoma scapula
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Widely resected specimen cut in path lab
tumor
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Close up
tumor
scapula
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Photomic
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Higher power
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Post op x-ray following scapular wing resection
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Case #531
35 year female with radiation sarcoma prox femur
tumor
prior radiation treatment for Hodgkin’s 20 yrs ago
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Frog leg lateral
tumor
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Shortly after withpathologic fracture
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Biopsy photomic
tumor
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Higher power
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Case #532
72 year maleradiation sarcoma pelvis
Prior radiation therapyfor prostate cancer3 years before
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Another view at adifferent date withhip dislocation
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Photomic
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Case #532.1
79 yr male with prior prostate CA radiation therapy and now presents with radiation OGS
Radiation induced OGS
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Coronal Anterior CT Posterior CT
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L Sagittal CT scan R Sagittal CT scan
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Low axial CTcut thru L hipshowing large tumor
Upper CT cut thruSI area showingtumor R post ilium
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Metastatic disease seen on chest x-ray
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Case #533
56 year female with radiation sarcoma scapula Prior history of radiation for breast cancer
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Oblique view
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Bone scan
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Photomic
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Case #534
63 year female with radiation sarcoma scapulawith prior radiation treatment for breast CA 12 yrs ago
tumor
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Case #535
44 year female withradiation sarcomaproximal humerus 2ndto prior radiation forbreast cancer
tumor
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Photomic with radiation OGS
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Case #536
76 year maleradiation sarcomafemur
Prior history ofradiation therapyfor soft tissue tumor10 years ago
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Bone scan
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Photomic radiation OGS
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Case #537
Elderly M.D. with longhistory working underX-ray fluoroscope
Now skin cancer andradiation sarcomaindex finger
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X-ray of index finger sarcoma
tumor
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Photomic radiation sarcoma
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MulticentricMulticentricOsteogenic Osteogenic SarcomaSarcoma
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Multicentric Osteosarcoma The multicentric variant of OGS is an extremely rare variantoccurring in approximately 1% of all OGS. It has two distinctcategories: (1) Synchronous multicentric OGS occurring in child-hood and adolescence. This is the more severe variant, consideredto be extremely high grade with a very poor prognosis associated with it. This form presents with multiple sclerotic lesions seen in a fairly symmetrical fashion in long bones, mostly in the lower extremities and because of the heavy tumor burden associatedwith multiple lesions throughout the skeleton, the alkaline phos-phatase is frequently elevated. (2) Metachronous multicentric OGS occurring mainly in adults is less aggressive than the synchronousform seen in children, presenting usually with a solitary lesion.Then, later on, more lesions develop that are considered multi-focal in nature. The possibility of metastasis can not be ruled out. These forms of OGS are quite resistant to chemotherapy and surgical treatment is frustrating because of the multi focal disease.
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CLASSICCase #116
8 year femalemulticentric OGS
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Close up distal femur
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Lateral view
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Bone scan
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Close up bone scan
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Upper bodybone scan
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Coronal T-1 MRI
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Another coronal cutT-1 MRI
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Sagittal T-1 MRI
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Photomic
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Another photomic
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Case #538
18 year femalemulticentric OGSpelvis and femur
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Gad contrast coronal MRI
tumor
tumor
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Another Gad contrast cut
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Axial T-2 MRI
pelvictumor
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Recon plate placed across pelvic ring surgical defect
Internal Hemipelvectomy
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Placement of air screws just prior to cementation
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Placement of cement around screws and plate
cement
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Constrained total hip in and securing musclesto custom proximal femoral replacement implant
totalhip
femoral implant
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Outer face of resected specimen
acetabulum
iliumtumor bulge
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Inner face
tumor bulge
ilium
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Closure
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Post op x-ray
recon plateand screws
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Case #539
16 year femalemulticentric OGS tumor
Proximal tibial lesion
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Lateral view withskip lesion indistal tibia
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Bone scan showing two lesions in tibia
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Bone scan showingiliac lesion
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Bone scan showingsternal lesion
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Photomic from tibial biopsy
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Proximal tibialresection andtotal knee reconstruction
tumorbulge
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Proximal tibialprosthesis in positionready for relocationand closure
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Reconstructioncompleted and ready for closure
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Case #540
Multicentric OGSfemur and sacrum20 year male
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Lateral view
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Coronal T-1 MRIshowing tumor atboth ends of femur
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Sagittal T-1 MRIdistal femur
tumor
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Axial T-2 MRI distal femur
tumor
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Another axial T-2 MRI
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Bone scan
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Coronal T-1 MRI
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Axial T-1 MRI
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Coronal gad contrast MRI showing sacral lesion
tumor
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Photomic from femoral biopsy
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Case #541
10 year female with multicentric OGS femur and tibia
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Lateral view
tumor
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tumor
skip lesion
AP view femur
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Coronal T-2 MRIdistal femur
tumor
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Sagittal T-2 MRIdistal femur
tumor
tibiallesions
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Coronal T-1 MRIknee joint
tumor
tumor
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Coronal T-1 MRI showing multicentric involvement
tumor
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Case #542
15 year male with multicentric OGS tibia and femur
tumor
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Coronal T-1 MRI
tumor
tumor
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Coronal T-2 MRI
tumor
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Sagittal T-1 MRI
tumor
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Axial T-2 MRI view of distal femur
tumor
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Soft TissueSoft TissueOsteogenic Osteogenic SarcomaSarcoma
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Soft Tissue Osteosarcoma
OGS can be seen in soft tissue outside the skeletal system. It accounts for 4% of all OGS and is typically in large muscle groupsaround the pelvis and thigh area. It occurs most often in patients over 40 years of age and hits males and females equally. Soft tissue OGS, with its mature appearing bone in the central area ofthe lesion and aggressive, poorly mineralized tissue at the periphery, must be differentiated from myositis ossificans, whichhas a typical zonal pattern with peripheral maturation of bone formation. As with any soft tissue sarcoma, the treatment consistsof wide local resection. Because of the poor prognosis, worse than that of bone osteosarcoma, systemic chemotherapy is utilized extensively as one would use for a typical medullary OGS.
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CLASSICCase #118
67 year malesoft tissue OGScalf
tumor
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AP view
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Sagittal T-1 MRI
tumor
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Axial T-1 MRI
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Cut surgical specimen in path lab
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Photomic
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Case #543
76 year femalesoft tissue OGScalf
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Lateral view
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CT scan
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Bone scan
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Axial T-1 MRI
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Sagittal T-1 MRItumor
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Photomic
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Case #544
60 year female withsoft tissue OGS leg
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Oblique view
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Bone scan
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Case #545
63 year male soft tissue OGShand tumor
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Lateral view
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Axial T-1 MRI
tumor
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Axial T-2 MRI
tumor
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Coronal T-2 MRI
tumor
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Multiple pulmonary mets
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IntracorticalIntracorticalOsteogenic Osteogenic SarcomaSarcoma
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Intracortical Osteosarcoma
The intracortical OGS is perhaps the rarest variant of OGS withonly 14 cases described in the world literature since 1960. It occurs between the ages of 10 and 47 years, equally betweenmales and females, and is seen most typically in the femur or tibia as a metadiaphyseal lesion with a radiographic appearancevery similar to that of osteoid oasteoma. The prognosis is usually quite good with a total of three deaths in the world literature. Itis usually treated by wide resection without chemotherapy. Afew cases are higher grade and carry a poor prognosis similarto the classic OGS.
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CLASSIC Case #119
42 year female with intracortical OGS femur
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Bone scan
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Axial PD MRI
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Sagittal T-2 MRI
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Early biopsy photomic
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X-ray 18 monthsafter curettementwith recurrence
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Bone scan at time of recurrence
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Axial Gad contrast MRI same time
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Sagittal PD MRIsame time
tumor
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Unicortical segmental wide resection
tumor
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Photomic of resected specimen
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Post op x-ray followingunicortical resectionand allograft recon
allograft
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Sagittal PD & T-2 MRI 18 months later with met to C-spine
tumor
PD T-2
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Case #546
43 year femaleintracortical OGSdistal femur
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Lateral view
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Sagittal T-1 MRI
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Sagittal T-2 MRI
tumor
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Biopsy photomic
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Photomic
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Case #547
47 year femaleintracortical OGShumerus
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Lateral view
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CT scan
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Sagittal T-1 MRI
tumor
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Post op x-ray afterwide resection andallograft recon