Volume 3
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This document describes several rare variants of osteosarcoma, including hemorrhagic osteosarcoma, parosteal osteosarcoma, periosteal osteosarcoma, pagetic sarcoma, and low grade intramedullary osteosarcoma. For each variant, it provides details on clinical presentation, radiographic appearance, histology, treatment approach, and prognosis. It also includes several case examples with images to illustrate the key features of each osteosarcoma variant.
Volume 3
- 1. Volume 3 Osteosarcoma Variants Hemorrhagic osteosarcoma------------Case 110 & 499-503 Parosteal osteosarcoma-----------------Case111 & 504-510 Periosteal osteosarcoma----------------Case 112 & 511-517 Pagetic sarcoma-------------------------Case 113 & 518-528 Low grade intramedullary OGS------Case 114 & 528.1-530 Radiation induced OGS---------------Case 115 & 531-537 Multicentric osteosarcoma------------Case 116 & 538-542 Soft tissue osteosarcoma--------------Case 118 & 543-545 Intracortical osteosarcoma------------Case 119 & 546-547
- 3. Hemorrhagic Osteogenic Sarcoma
- 4. Hemorrhagic (Telangiectatic) Osteosarcoma The hemorrhagic (OGS), an extremely lytic and hemorrhagic variant of the osteosarcoma, presents in the same age group and location as a classic osteosarcoma but has a radiographic appearance almost identical to that of an aggressive aneurysmal bone 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 aneurysmal bone cyst. Even microscopically, many areas of the hemorrhagic OGS will have the appearance of an aneurysmal bone cyst with only an occasional mitotic figure. For this reason, it is very important for the surgeon who performs the biopsy to obtain an adequate specimen with good sampling by means of an open biopsy as apposed to a simple needle biopsy. The microscopic features of the hemorrhagic OGS is a large number of benign- appearing giant cells and thus the terminology “giant cell rich”
- 5. osteosarcoma that is used by many pathologists. There is very little 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 that come potential problems for the treating orthopedic surgeon who must 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 OGS was 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.
- 6. CLASSIC Case #110 23 year male hemorrhagic OGS proximal humerus Aneurysmal lesion
- 7. hemorrhagic tumor Coronal T-1 MRI
- 8. Coronal T-1 MRI tumor thru path fracture
- 9. Resected tumor cut in path lab
- 10. Photomic showing giant cells and malignant cells
- 11. osteoid blood Photomic showing hemorrhagic response
- 12. Neer allograft Post op x-ray with alloprosthetic reconstruction
- 13. 18 year followup x-rays
- 14. Case #499 15 year male hemorrhagic OGS distal femur
- 15. Lateral view
- 16. Bone scan
- 18. hemorrhagic tumor Coronal T-2 MRI
- 19. tumor Axial T- 2 MRI
- 20. blood Photomic
- 21. osseo- integration 3 yrs post op Compress total knee reconstruction CPS
- 22. Case #500 19 year male hemorrhage OGS proximal femur Looks like ABC
- 23. Lateral view
- 24. Initial biopsy reveals aneurysmal bone cyst
- 25. 6 weeks later shows lysis of outer shell Repeat biopsy reveals hemorrhagic OGS
- 26. femoral head tumor Hip disarticulation specimen
- 28. Case #501 6 year female path fracture thru unicameral bone cyst
- 29. cystic lesion Lateral view
- 30. 7 weeks after cyst steroid injection
- 31. 1 month later and progressive lytic destruction
- 32. Biopsy here shows hemorrhagic OGS
- 33. Case #501.1 Telangiectatic OGS 19 year old male with acute onset of pain 2 wks ago in right hip
- 34. PO 1 mo 2 mo 3 mo
- 35. Cor T-1 T-2
- 36. Axial T-1 T-2 Gad
- 37. Sag T-2 Gad
- 38. Case #502 4 year male cystic looks like lesion unicameral bone cyst
- 39. Progressive lysis after steroid injection
- 40. 2 months later with progressive lysis and looking malignant
- 41. Biopsy reveals hemorrhagic OGS
- 42. Clinical appearance before shoulder disarticulation
- 43. Case #503 17 year female hemorrhagic OGS C-3
- 44. AP view
- 45. CT scan
- 46. Photomic
- 47. 6 years later with spontaneous fusion and no tumor
- 49. Parosteal Osteosarcoma The parosteal (OGS) is a low grade variant arising from the surface of 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 clearly separates it from the classic OGS. It is seen more commonly in females than males and is found in a slightly older age group than 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 a palpable tumor mass that might have been present many years before medical advise was sought. Histologically, this tumor has a very low mitotic index and in many cases can be confused with a normal healing fracture callous with occasional areas of cartilage being seen. Because this tumor is extremely low grade, it is not
- 50. responsive to adjuvant therapy such as chemotherapy or radiation 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 after the 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.
- 51. CLASSIC Case #111 32 year male parosteal OGS distal femur
- 52. AP view
- 53. Bone scan
- 54. Sagittal T-1 MRI
- 56. Axial T-1 MRI
- 58. Photomic
- 59. Higher power
- 60. Case #504 18 year male parosteal OGS distal femur
- 61. tumor AP view
- 62. Bone scan
- 63. tumor Axial T-2 MRI
- 65. tumor Macro section
- 66. Photomic
- 67. osseointegration Compress total knee reconstruction 2 years later
- 68. 10 years later with recurrence as a high grade dedifferentiated parosteal OGS tumor
- 69. Another view tumor
- 71. Close up of osseointegration of Compress implant
- 72. Case #505 32 year male parosteal OGS tumor proximal humerus
- 74. tumor CT scan
- 75. Amputation specimen cut in path lab
- 76. Photomic
- 77. Case #506 25 year male parosteal OGS distal femur
- 78. Distal femoral resection specimen tumor
- 79. tumor Cut specimen in path lab fatty marrow
- 80. Case #507 13 year male parosteal OGS mid femur AP view
- 81. Lateral view
- 82. CT scan
- 84. Autoclaved bone replaced with IM nail fixation
- 85. Post op x-ray 2 years later autoclaved bone
- 86. Case #508 17 year male with parosteal OGS mid tibia
- 87. Lateral x-ray
- 88. tumor CT scan
- 89. Bone scan
- 90. biopsy site Segmental resection mid tibial lesion
- 91. tumor Surgical specimen cut in path lab
- 92. Allograft reconstruction over IM nail
- 93. X-ray 1 year later
- 94. Case #509 41 year female parosteal OGS humerus
- 95. CT scan
- 96. tumor Resected cut specimen in path lab
- 97. Case #509.1 Parosteal OGS pseudotumor M.O. 10/06 3/07 17 year male with football injury 9/06
- 98. Sag T-1 Sag Gad
- 100. Case #510 32 year female with high grade parosteal OGS femur
- 101. Macro section tumor
- 102. Photomic
- 104. Periosteal Osteosarcoma The periosteal osteosarcoma is another surface type OGS that tends to be low grade to intermediate with potential for pulmonary metastasis in about 25% of cases. It accounts for 2% of all OGS’s and, compared to the parosteal OGS, has a much higher percentage of cartilagenous tissue in the tumor to the point where it can look like 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 life and 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 utilized unless the clinical picture is more aggressive than usual.
- 105. CLASSIC Case #112 15 year female with periosteal OGS tibia
- 106. CT scan
- 108. Axial T-2 MRI
- 109. Photomic
- 110. Post op x-ray following wide resection and allograft reconstruction
- 111. Case #511 30 year male with periosteal OGS prox tibia
- 112. CT scan
- 113. Sagittal T-2 MRI
- 114. edema tumor Axial T-1 MRI
- 115. Wide resection proximal tibia tumor bulge
- 116. tumor Cut specimen in path lab
- 117. Photomic
- 118. Proximal tibia resected ready for reconstruction
- 119. Post op x-ray with alloprosthetic TKA reconstruction allograft
- 120. Case # 512 9 year female periosteal OGS tibia
- 121. AP x-ray
- 122. Lateral view
- 123. Cut specimen in path lab following AK amputation
- 124. Photomic
- 125. Higher power
- 126. Case #513 14 year male periosteal OGS
- 127. Sagittal T-2 MRI
- 128. Axial T-1 MRI
- 129. Axial T-2 MRI
- 130. Case #514 26 year female periosteal OGS distal femur
- 131. Lateral view
- 132. stress shielding X-ray 10 years following wide resection and cemented prosthetic reconstruction
- 133. Case #515 12 year female periosteal OGS tibia
- 134. Bone scan
- 135. Axial T-1 MRI
- 136. Photomic
- 137. Case #516 15 year male periosteal OGS distal tibia
- 138. CT scan
- 139. Bone scan
- 140. Photomic
- 141. Case #517 39 year female periosteal OGS pseudotumor In fact is a Nora’s lesion or bizarre parosteal osteochondromatous proliferation (BPOP)
- 142. Bone scan
- 143. CT scan
- 144. edema Axial Gad contrast MRI
- 145. Sagittal PD
- 146. Sagittal T-2 MRI edema
- 147. Axial gad contrast MRI
- 148. Pagetic Sarcoma
- 149. Pagetic Sarcoma There are multiple diseases of the skeletal system that can result in a secondary form of OGS most likely brought about by a second mutation 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’s disease, 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 Pagetic OGS 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’s disease but then suddenly develop an acute new pain in the area of the older pain with x-ray evidence of recent lysis and destruction
- 150. of old Pagetic reactive bone. The prognosis for survival in this secondary form of OGS is extremely poor with only about 8% surviving, mainly because the older age group in which the disease occurs make it impractical to implement the aggressive protocols used in younger age groups.
- 151. CLASSIC Case #113 tumor 80 year female with Pagetic sarcoma pelvis
- 152. Bone scan
- 153. tumor Axial T-2 MRI
- 154. osteoid Photomic
- 155. Post op internal hemipelvectomy
- 156. Case#518 tumor 83 year female Pagetic sarcoma pelvis
- 157. tumor CT scan
- 158. tumor Another CT cut
- 159. Photomic
- 160. Case #519 85 year female with Paget’s disease pelvis
- 161. Same disease in lumbar spine
- 163. Same disease in tibia Advancing osteolytic wedge
- 164. old Paget’s Same patient with Pagetic sarcoma humerus new tumor
- 165. Macro section from amputation specimen tumor
- 166. Photomic
- 167. Post op x-ray following forequarter amputation
- 168. Case # 520 73 year female Pagetic sarcoma skull ready for resection
- 169. Lateral view of skull
- 170. Occipital view
- 171. Tangential view
- 172. tumor Resected specimen cut in path lab
- 173. Photomic
- 174. Case #521 old Paget’s with prior fracture 82 year male Pagetic sarcoma distal humerus
- 175. tumor Close up of new tumor
- 176. Photomic
- 177. Case #522 80 year female Pagetic sarcoma distal humerus
- 178. Case #523 femur humerus 84 male with multi focal Pagetic sarcoma
- 179. Case #524 83 year male Pagetic sarcoma femur
- 180. Case #525 60 year male Pagetic sarcoma femur
- 181. Lateral view
- 182. Photomic
- 183. Photomic
- 184. Case #526 78 female Pagetic sarcoma proximal tibia
- 185. Lateral view tumor
- 186. Case #527 92 year male Pagetic sarcoma tibia
- 187. Case #528 78 year female Pagetic sarcoma lumbar spine
- 188. Low Grade Intramedullary Osteogenic Sarcoma
- 189. 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 confined within 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 cortical anatomy. The smoky appearance of metaphyseal bone suggests the diagnosis of chronic osteomyelitis or perhaps fibrous dysplasia. Microscopically, the tumor has a histological appearance similar to 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.
- 190. CLASSIC Case #114 63 year female intramedullary OGS distal femur
- 191. Lateral view
- 192. Bone scan
- 193. tumor CT scan
- 194. tumor Macro section from resected specimen
- 195. Photomic
- 196. Photomic
- 197. Case #528.1 51 year female tumor low grade intramedullary OGS distal femur
- 198. Bone scan
- 199. Coronal T-1 MRI tumor
- 200. tumor Axial T-1 MRI
- 201. tumor Resected distal femur cut in path lab
- 202. Photomic
- 203. Case #529 32 year female tumor low grade intramedullary OGS distal femur
- 204. Lateral view
- 205. CT scan
- 206. Photomic
- 207. Case #530 56 year male low grade tumor intramedullary OGS distal tibia
- 208. Lateral view tumor
- 209. Bone scan
- 210. Photomic
- 211. Radiation-induced Osteogenic Sarcoma
- 212. Radiation-induced Osteosarcoma One of the most malignant forms of OGS is the secondary type induced 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. The average delay for the occurrence of secondary OGS is 15 years, with a range from 3 to 55 years. The prognosis for this variant is extremely poor, similar to Pagetic OGS. It has a very high rate of metastasis to the lung for which chemotherapy is not very effective.
- 213. CLASSIC Case #115 33 year female radiation-induced sarcoma scapula
- 214. tumor Widely resected specimen cut in path lab
- 215. scapula tumor Close up
- 216. Photomic
- 217. Higher power
- 218. Post op x-ray following scapular wing resection
- 219. Case #531 tumor 35 year female with radiation sarcoma prox femur prior radiation treatment for Hodgkin’s 20 yrs ago
- 220. Frog leg lateral tumor
- 221. Shortly after with pathologic fracture
- 223. Higher power
- 224. Case #532 72 year male radiation sarcoma pelvis Prior radiation therapy for prostate cancer 3 years before
- 225. Another view at a different date with hip dislocation
- 226. Photomic
- 227. Case #532.1 Radiation induced OGS 79 yr male with prior prostate CA radiation therapy and now presents with radiation OGS
- 228. Coronal Anterior CT Posterior CT
- 229. L Sagittal CT scan R Sagittal CT scan
- 230. Low axial CT cut thru L hip showing large tumor Upper CT cut thru SI area showing tumor R post ilium
- 231. Metastatic disease seen on chest x-ray
- 232. Case #533 56 year female with radiation sarcoma scapula Prior history of radiation for breast cancer
- 233. Oblique view
- 234. Bone scan
- 235. Photomic
- 236. Case #534 tumor 63 year female with radiation sarcoma scapula with prior radiation treatment for breast CA 12 yrs ago
- 237. Case #535 44 year female with radiation sarcoma proximal humerus 2nd to prior radiation for tumor breast cancer
- 238. Photomic with radiation OGS
- 239. Case #536 76 year male radiation sarcoma femur Prior history of radiation therapy for soft tissue tumor 10 years ago
- 240. Bone scan
- 242. Case #537 Elderly M.D. with long history working under X-ray fluoroscope Now skin cancer and radiation sarcoma index finger
- 243. X-ray of index finger sarcoma tumor
- 246. Multicentric Osteosarcoma The multicentric variant of OGS is an extremely rare variant occurring in approximately 1% of all OGS. It has two distinct categories: (1) Synchronous multicentric OGS occurring in child- hood and adolescence. This is the more severe variant, considered to 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 associated with 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 synchronous form 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.
- 247. CLASSIC Case #116 8 year female multicentric OGS
- 248. Close up distal femur
- 249. Lateral view
- 250. Bone scan
- 251. Close up bone scan
- 252. Upper body bone scan
- 253. Coronal T-1 MRI
- 254. Another coronal cut T-1 MRI
- 255. Sagittal T-1 MRI
- 256. Photomic
- 257. Another photomic
- 258. Case #538 18 year female multicentric OGS pelvis and femur
- 259. tumor tumor Gad contrast coronal MRI
- 260. Another Gad contrast cut
- 261. pelvic tumor Axial T-2 MRI
- 262. Internal Hemipelvectomy Recon plate placed across pelvic ring surgical defect
- 263. Placement of air screws just prior to cementation
- 264. cement Placement of cement around screws and plate
- 265. femoral implant total hip Constrained total hip in and securing muscles to custom proximal femoral replacement implant
- 266. acetabulum tumor bulge ilium Outer face of resected specimen
- 267. tumor bulge ilium Inner face
- 268. Closure
- 269. recon plate and screws Post op x-ray
- 270. Case #539 16 year female multicentric OGS tumor Proximal tibial lesion
- 271. Lateral view with skip lesion in distal tibia
- 272. Bone scan showing two lesions in tibia
- 273. Bone scan showing iliac lesion
- 274. Bone scan showing sternal lesion
- 275. Photomic from tibial biopsy
- 276. Proximal tibial resection and tumor total knee bulge reconstruction
- 277. Proximal tibial prosthesis in position ready for relocation and closure
- 278. Reconstruction completed and ready for closure
- 279. Case #540 Multicentric OGS femur and sacrum 20 year male
- 280. Lateral view
- 281. Coronal T-1 MRI showing tumor at both ends of femur
- 282. Sagittal T-1 MRI distal femur tumor
- 283. tumor Axial T-2 MRI distal femur
- 284. Another axial T-2 MRI
- 285. Bone scan
- 286. Coronal T-1 MRI
- 287. Axial T-1 MRI
- 288. tumor Coronal gad contrast MRI showing sacral lesion
- 289. Photomic from femoral biopsy
- 290. Case #541 10 year female with multicentric OGS femur and tibia
- 291. tumor Lateral view
- 292. skip lesion AP view femur tumor
- 293. tumor Coronal T-2 MRI distal femur
- 294. tumor Sagittal T-2 MRI distal femur tibial lesions
- 295. tumor Coronal T-1 MRI knee joint tumor
- 296. tumor Coronal T-1 MRI showing multicentric involvement
- 297. Case #542 tumor 15 year male with multicentric OGS tibia and femur
- 298. tumor Coronal T-1 MRI tumor
- 299. Coronal T-2 MRI tumor
- 300. Sagittal T-1 MRI tumor
- 301. tumor Axial T-2 MRI view of distal femur
- 303. 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 groups around 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 of the lesion and aggressive, poorly mineralized tissue at the periphery, must be differentiated from myositis ossificans, which has a typical zonal pattern with peripheral maturation of bone formation. As with any soft tissue sarcoma, the treatment consists of 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.
- 304. CLASSIC Case #118 tumor 67 year male soft tissue OGS calf
- 305. AP view
- 306. Sagittal T-1 MRI tumor
- 307. Axial T-1 MRI
- 308. Cut surgical specimen in path lab
- 309. Photomic
- 310. Case #543 76 year female soft tissue OGS calf
- 311. Lateral view
- 312. CT scan
- 313. Bone scan
- 314. Axial T-1 MRI
- 315. Sagittal T-1 MRI tumor
- 316. Photomic
- 317. Case #544 60 year female with soft tissue OGS leg
- 318. Oblique view
- 319. Bone scan
- 320. Case #545 63 year male soft tissue OGS hand tumor
- 321. Lateral view
- 322. tumor Axial T-1 MRI
- 323. tumor Axial T-2 MRI
- 326. Intracortical Osteogenic Sarcoma
- 327. Intracortical Osteosarcoma The intracortical OGS is perhaps the rarest variant of OGS with only 14 cases described in the world literature since 1960. It occurs between the ages of 10 and 47 years, equally between males and females, and is seen most typically in the femur or tibia as a metadiaphyseal lesion with a radiographic appearance very similar to that of osteoid oasteoma. The prognosis is usually quite good with a total of three deaths in the world literature. It is usually treated by wide resection without chemotherapy. A few cases are higher grade and carry a poor prognosis similar to the classic OGS.
- 328. CLASSIC Case #119 42 year female with intracortical OGS femur
- 329. Bone scan
- 330. Axial PD MRI
- 331. Sagittal T-2 MRI
- 333. X-ray 18 months after curettement with recurrence
- 334. Bone scan at time of recurrence
- 335. Axial Gad contrast MRI same time
- 336. tumor Sagittal PD MRI same time
- 337. tumor Unicortical segmental wide resection
- 338. Photomic of resected specimen
- 339. Post op x-ray following allograft unicortical resection and allograft recon
- 340. PD T-2 tumor Sagittal PD & T-2 MRI 18 months later with met to C-spine
- 341. Case #546 43 year female intracortical OGS distal femur
- 342. Lateral view
- 343. Sagittal T-1 MRI
- 345. Biopsy photomic
- 346. Photomic
- 347. Case #547 47 year female intracortical OGS humerus
- 348. Lateral view
- 349. CT scan
- 351. Post op x-ray after wide resection and allograft recon