Volume 6

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Volume 6

  1. 1. Volume 6 Fibrous Tumors of BoneFibrous cortical defects------------Case 130-131 & 700-702Non-ossifying fibroma-------------Case 132-133 & 703-713Fibrous dysplasia Monostotic------------------------Case 134-135 & 714-748 Polyostotic------------------------Case 136 & 749-757 Mazabraud syndrome-----------Case 758-759 Albright’s disease---------------Case 137 & 760-764Desmoplastic fibroma-------------Case 138-139 & 765-771Fibrosarcoma-----------------------Case 140-141 & 772-783Malignant fibrous histiocytoma--Case 142-143 & 784-800Leiomyosarcoma-------------------Case 144-45
  2. 2. Fibrous Bone Tumors
  3. 3. Fibrous Cortical Defects
  4. 4. Fibrous Cortical Defects The fibrous cortical defect is a very small metaphyseal lesion seenin long bones, typically around the knee joint in children. They areusually picked up on a routine radiograph as an incidental finding.They are considered dysplastic or hamartomatous lesions and arevery common, with about a 25% incidence in normal childrenaround the age of four to five years. As the child matures, thelesions will disappear by a process of skeletal remodeling and,in most cases, the diagnosis is obvious based on a simple radio-graphic study. Microscopically, the lesions are filled with benignfibroblastic cells forming collagen and usually demonstrate nomacrophage or inflammatory activity.
  5. 5. CLASSICCase #13016 year femalefibrous corticaldefect distal femur
  6. 6. Photomic
  7. 7. Case #13118 year malefibrous cortical defectdistal femur
  8. 8. Lateral view
  9. 9. Case #70018 year malefibrous cortical defectdistal femur
  10. 10. Lateral view
  11. 11. Bone scan
  12. 12. Case #701 15 year male with fibrous cortical defect distal femur
  13. 13. Case #70242 year female with fibrous cortical defect femoral neck
  14. 14. Frog leg lateral
  15. 15. Bone scan
  16. 16. CT scan shows bilateral lesions
  17. 17. Non-ossifying Fibroma
  18. 18. Non-ossifying Fibroma The non-ossifying fibroma is considered to be a larger form of thefibrous cortical defect seen in the lower extremities of children.The lesions are usually eccentrically located but the larger ones canbecome more central. Radiographically, they have a soap-bubblyappearance with slight thinning and dilitation of the adjacentcortical structure. The diagnosis can usually be established by x-rayalone without biopsy. These lesions are a common cause for path-ologic fracture in children. They are usually asymptomatic untilthe day of injury. There can be multiple lesions in the same patient,similar to fibrous dysplasia, and can even be associated with café-au-laite skin lesions. Histologically, the lesion consists of benign fibrous tissuespeckled with giant cells, histiocytes, and occasional foam cellsand even cholesterol deposits. Treatment usually consists of curette-ment and bone grafting in high risk athletic males.
  19. 19. CLASSIC Case #13222 year female with non-ossifying fibroma proximal tibia
  20. 20. Close up lateral
  21. 21. Photomic showing giant cells
  22. 22. Post op x-ray afterbone grafting fibular strut
  23. 23. Case #13312 year malenon-ossifying fibromadistal tibia withpathologic fracture
  24. 24. Case #70316 year malenon-ossifying fibromadistal tibia
  25. 25. Coronal T-2 MRI
  26. 26. giant cellsfoam cells Photomic with foam cells and giant cells
  27. 27. Case #704 stress fracture 9 year female with non-ossifying proximal tibia
  28. 28. stress fracture Lateral view
  29. 29. Coronal GRE T-2* MRI showing stress fracture
  30. 30. Coronal GRE T-2 MRI showing stress fracture
  31. 31. Case #70511 year femalenon-ossifying fibromatibia with path fracture
  32. 32. Case #70613 year malenon-ossifying fibromadistal tibia
  33. 33. Case #707 18 year male with non-ossifying fibroma distal tibia
  34. 34. Case #7089 year malenon-ossifying fibromadistal fibula
  35. 35. Case #70912 year malenon-ossifying fibromadistal fibula
  36. 36. Case #710 xanthofibroma34 year femalexanthofibroma andABC prox fibula ABC
  37. 37. Lateral view
  38. 38. Case #711 17 year male with non-ossifying fibroma distal femur
  39. 39. Bone scan
  40. 40. Photomic
  41. 41. Case #7125 year malenon-ossifying fibromafemur with pathfracture
  42. 42. Case #71316 year femalenon-ossifying fibromafemur with path fracture
  43. 43. Fibrous Dysplasia
  44. 44. Fibrous Dysplasia Fibrous dysplasia of bone is a relatively common hamartomatousdevelopmental process in the skeletal system that can be eithermonostotic or polyostotic. In the case of polyostotic, there can alsobe endrocrine abnormalities such as precocious puberty in femaleswith the McCune-Albright syndrome. This condition occursprimarily in females and can be associated with café-au-laite spotson the skin which have a rough serrated edge compared to thesmooth edges in neurofibromatosis. The monostotic form has anappearance similar to non-ossifying fibroma except that the lyticcomponent has a typical smokey appearance caused by metaplasticbone formation in the fibrous tissue. In the more severe polyostoticform, representing only about 10% of fibrous dysplasia, the diseasetends to amplify on one side of the body more than the othercausing leg length discrepancy, especially when there is a character-istic chronic deformity of the proximal femur; referred to in the
  45. 45. literature as the shepherd’s crook deformity; that frequentlyrequires surgical correction. The most common locations for fibrous dysplasia are theproximal femur, followed by the tibia, humerus, ribs and,occasionally, caranio-facial anatomy. Other endrocrinopathiesassociated with polyostotic fibrous dysplasia include hyperpara-thyroidism, acromegaly, Cushing’s disease and, occasionally,hypophosphatemic osteomalacia. Occasionally, one will see softtissue myxomas with fibrous dysplasia which is referred to as theMazabraud’s syndrome. Radiographically, there will be diffusedysplastic changes throughout the involved bones with gradualdilation and thinning of the cortices, and a soap-bubbly appear-ance but with a smoky look compared to non-ossifing fibroma.Histologically, there is evidence of benign fibrous tissue matrixstudded with islands of metaplastic bone with a so-calledalphabet soup appearance but without osteoblastic rimming of the
  46. 46. trabeculae. Occasionally one will see macrophages, osteoclasts,foam cells and even cholesterol deposits similar to what is foundin non-ossifying fibroma. In approximately 1% of cases, there willbe a conversion to a secondary sarcoma in adult life that couldpresent as an osteosarcoma, or malignant fibrous histiocytoma oreven a chondrosarcoma. Treatment usually consists of corrective osteotomies for secondarydevelopmental deformities, along with bone grafting, includingfibular strut grafts and occasionally intramedullary devices to preventrepeated stress fractures. Recently there has been enthusiasm forthe use of biphosphonates to inhibit the osteoclastic activity ingrowing children that produces weakening of the bone. It is bestnot to perform bone grafting procedures in children because of thehigh recurrence rate in children under 16 years of age.
  47. 47. MonostoticFibrous Dysplasia
  48. 48. CLASSICCase #13445 year femalemonostoticfibrous dysplasiaproximal tibia
  49. 49. Bone scan
  50. 50. Coronal T-1 MRI
  51. 51. Sagittal T-2 MRI
  52. 52. Photomic showing alphabet soup metaplastic bone
  53. 53. metaplastic osteoclastsbone fibrous tissue Close up photomic
  54. 54. Case #13559 year femalemonostotic fibrousdysplasia humerus
  55. 55. Bone scan
  56. 56. Sagittal T-1 MRI
  57. 57. Case #135.124 year female with monostotic fibrous dysplasia humerus
  58. 58. Case #71430 year malemonostotic fibrousdysplasia femur
  59. 59. Lateral view
  60. 60. Coronal T-1 MRI
  61. 61. Photomic with metaplastic bone
  62. 62. Monostotic fibrous dysplasiaCase #714.1 Bone scan 46 year male with mild stress pain right hip
  63. 63. Cor T-1 STIR
  64. 64. Sag T-1 PD Gad
  65. 65. P.O. DHS and cancellousallograph
  66. 66. Case #71518 year femalemonostotic fibrousdysplasia prox femur
  67. 67. Bone scan
  68. 68. metaplastic bone Photomic
  69. 69. Case #71621 year femalemonostotic fibrousdysplasia prox femur
  70. 70. Case #71769 year femalemonostotic fibrousdysplasia femur
  71. 71. Lateral view
  72. 72. Bone scan
  73. 73. Coronal T-1 MRI
  74. 74. Coronal T-2 MRI
  75. 75. Photomic
  76. 76. Case #717.125 year malemonostotic fibrousdysplasia femur
  77. 77. Post op x-rayfollowing surgicalcorrection of varusdeformity
  78. 78. Case #71827 year malemonostotic fibrousdysplasia femur
  79. 79. Lateral view
  80. 80. Case #71947 year malemonostotic fibrousdysplasia femurLateral view
  81. 81. Bone scan
  82. 82. Case #72010 year femalemonostotic fibrousdysplasia proximalfemur with pathologicfracture
  83. 83. Case #72116 year femalesolitary bone cystfemoral neck
  84. 84. X-ray picture oneyear following bonegrafting procedure
  85. 85. Then developes tibial pain because of fibrous dysplasia
  86. 86. Macro cyst seen in fibrous dysplasia beneath cortical window
  87. 87. cortical window macro cyst Surgical specimen following curettement
  88. 88. Photomic with alphabet soup bone
  89. 89. Appearance following curettement
  90. 90. Placement of ipsilateral fibular bone strut
  91. 91. fibular strutX-ray a year later
  92. 92. Case #72238 year femalemonostotic fibrousdysplasia tibia
  93. 93. Close up view
  94. 94. Bone scan
  95. 95. Case #72339 year femalemonostotic fibrousdysplasia tibia
  96. 96. CT scan
  97. 97. Case #724 20 year female with monostotic fibrous dysplasia tibia
  98. 98. Coronal T-2 MRI
  99. 99. Axial T-2 MRI
  100. 100. osteoclastsmetaplastic bone Photomic
  101. 101. Case #725 27 year male monostotic fibrous dysplasia tibia
  102. 102. Another view
  103. 103. Case #72637 year malemonostotic fibrousdysplasia fibula
  104. 104. Bone scan
  105. 105. Sagittal T-1 MRI
  106. 106. Sagittal T-2 MRI
  107. 107. Case #7278 year femalemonostotic fibrousdysplasia humeruswith path fracture
  108. 108. X-ray one year laterwith 2nd path fracture
  109. 109. CT scan
  110. 110. Case #72837 year femalemonostotic fibrousdysplasia humerus
  111. 111. Lateral view
  112. 112. Bone scan
  113. 113. Coronal T-1 MRI
  114. 114. Post op x-ray fibularfollowing bone strutgraft
  115. 115. Case #72945 year female withmonstotic fibrous dysplasiahumerus
  116. 116. Lateral viewdistal humerus
  117. 117. Surgical debriedment of fibrous tissue
  118. 118. Photomic
  119. 119. Placement of IM nail prior to cementation
  120. 120. Cementation completed
  121. 121. nailPost op x-ray2.5 years later
  122. 122. Case #73028 year femalemonostotic fibrousdysplasia humerus
  123. 123. Case #731 36 year male with monostotic fibrous dysplasia scapula
  124. 124. Case #73219 year femalemonostotic fibrousdysplasia prox radius
  125. 125. Sagittal T-1 MRI
  126. 126. Sagittal T-2 MRI
  127. 127. Case 73314 year malemonostotic fibrousdysplasia radius
  128. 128. Case #734 Lateral view 13 year female with monostotic fibrous dysplasia ulna
  129. 129. AP view
  130. 130. Case #735 16 year female with monstotic fibrous dysplasia ulna
  131. 131. Case #73611 year femalemonostotic fibrousdysplasia 1stmetatarsal
  132. 132. Case #737 26 year female with monstotic fibrous dysplasia 3rd rib
  133. 133. Macro section of resected rib
  134. 134. Photomic
  135. 135. Case #73839 year femalemonostotic fibrousdysplasia 12th rib gall stones
  136. 136. Case #739Monostotic fibrousdysplasia 10th ribin a 25 year female
  137. 137. Case #740 46 year female with monostotic fibrous dysplasia pelvis
  138. 138. CT scan
  139. 139. 3-D CT anterior oblique reconstruction
  140. 140. 3-D CT posterior oblique reconstruction
  141. 141. Case #741 14 year male with monostotic fibrous dysplasia pelvis
  142. 142. CT scan
  143. 143. Lower cut
  144. 144. Case #741.137 year female withincidental finding onX-ray of pelvis
  145. 145. Axial T-1 T-2 FS Gad
  146. 146. Cor T-1 T-2 FS Gad
  147. 147. Sag T-2 FS Gad
  148. 148. Case #742 22 year male with fibrocartilaginous dysplasia sacrum
  149. 149. Bone scan
  150. 150. Axial T-1 MRI
  151. 151. tumorAxial T-2 MRI
  152. 152. Coronal T-1 MRI
  153. 153. tumorCoronal T-2 MRI
  154. 154. cartilage fibrous bone Photomic
  155. 155. cartilagefibrous Photomic
  156. 156. Photomic showing chondroid matrix
  157. 157. Case #743 CT scan23 year female with monostotic fibrous dysplasia L-spine
  158. 158. Another cut
  159. 159. Another cut
  160. 160. Case #744 17 year female with monostotic fibrous dysplasia skull
  161. 161. Case #74516 year malemonostotic fibrousdysplasia maxilla
  162. 162. Skull x-ray
  163. 163. Photomic
  164. 164. Case #746 Monostotic fibrous dysplasia frontal bone
  165. 165. Case #747 22 year male monostotic fibrous dysplasia mandible
  166. 166. Case #748 23 yr female Monostotic fibrous dysplasia mandible
  167. 167. PolyostoticFibrous dysplasia
  168. 168. CLASSICCase #13625 year malepolyostotic fibrousdysplasia
  169. 169. Photo of left faceinvolvement includingeye displacement fromorbital disease
  170. 170. Skull x-ray showingchanges left side ofskull and face
  171. 171. Shepherd’s crookdeformity proximalfemur
  172. 172. Macro section ofshepherd’s crookdeformity
  173. 173. Photomic showing alphabet soup metaplastic bone
  174. 174. Soap-bubblyhumeral defect
  175. 175. R LMajor changes left and minor changes right hand
  176. 176. Case #749 Pelvis & hip x-ray 25 year male with polyostotic fibrous dysplasia
  177. 177. tibiaX-ray femur and femur femurtibia
  178. 178. Autopsy specimen offemur cut in path lab
  179. 179. Macro section ofproximal femur
  180. 180. Photomic with punctate metaplastic bone
  181. 181. Case #75032 year male withpolyostotic fibrousdysplasia
  182. 182. Views front and back
  183. 183. Shepherd’s crookdeformity femur
  184. 184. Skull deformities
  185. 185. feetHand and footX-rays hands
  186. 186. Case #75120 year femalepolyostotic fibrousdysplasia
  187. 187. Side view
  188. 188. path fractureX-rays hand and humerus
  189. 189. AP x-ray pelvis and hips with path fracture femoral neck
  190. 190. Leg x-ray withvarus deformity
  191. 191. Case # 752 large café-au-lait spot 29 year male with polyostotic fibrous dysplasia
  192. 192. AP x-ray showingextensive thinningand dilatation oftibial cortex
  193. 193. Lateral view
  194. 194. Amputation specimen cut in path lab
  195. 195. Photomic
  196. 196. Case #75358 year male withpolyostotic fibrousdysplasia of femoriAnd feet
  197. 197. Femoral head specimen from total hip surgery
  198. 198. Photomic
  199. 199. Post op x-ray withlong stem femoralcomponent
  200. 200. Lateral view showingold healed pathfracture
  201. 201. Opposite hip withsimilar defects L
  202. 202. Involvement of both feet
  203. 203. Case #753.1 31 year female with mid thigh pain for 1 year
  204. 204. Bone scan
  205. 205. Coronal T-1 Coronal T-2
  206. 206. Coronal T-1 Coronal T-2
  207. 207. Axial T-1 Axial T-2
  208. 208. Case #753.2 Polyostotic fibrous dysplasia 16 year male with deformity of proximal thumb for years
  209. 209. X-rays of right upper extremity
  210. 210. Post op x-ray following curettement and bone grafting
  211. 211. Case #754 34 year female with polyostotic fibrous dysplasia
  212. 212. Case #75519 year malepolyostotic fibrousdysplasia withshepherd’s crookdeformity femur
  213. 213. Skull involvement
  214. 214. L R Changes in right hand more than left
  215. 215. Case #75626 year malepolyostotic fibrousdysplasia left hip
  216. 216. Case #756.1 40 yr male with fibrous dysplasia humerus and prox ulna with prior surg 20 yrs ago and now aneurysmal changes
  217. 217. Sagittal T-1 MRI Sagittal T-2 MRI
  218. 218. Coronal T-1 Coronal STIR Coronal Gad MRI
  219. 219. Case #75728 year femalepolyostotic fibrousdysplasia LS spine
  220. 220. Oblique views LS spine
  221. 221. Lateral view
  222. 222. Sagittal T-1 MRI
  223. 223. Axial T-1 MRI sacrum
  224. 224. Sagittal T-2 MRI with cystic changes
  225. 225. Axial T-2 MRI with fluid-fluid level in sacrum
  226. 226. cystAxial gad contrast MRI with rim enhancement
  227. 227. Sagittal gad contrast MRI with lumbar cysts
  228. 228. Photomic from sacral biopsy
  229. 229. MazabraudSyndrome
  230. 230. Case #75839 year femaleMazabraud syndromepolyostotic fibrousdysplasia with softtissue myxomasCT skull
  231. 231. fibrous dysplasia myxomamyxoma Coronal T-1 MRI
  232. 232. Coronal T-2 MRI showing high signal myxomas
  233. 233. Case #759 fibrous dysplasia 62 year female with Mazabraud’s syndrome
  234. 234. AP shoulder
  235. 235. Bone scan
  236. 236. Bone scan skull
  237. 237. Bone scan femur
  238. 238. fibrousCoronal T-1 MRI showing multiple myxomas
  239. 239. fibrousCoronal T-2 MRIshowing high signalmyxomas
  240. 240. fibrousAxial T-1 MRI with low signal myxomas
  241. 241. fibrousAxial T-2 MRI with high signal myxomas
  242. 242. Coronal T-2 MRI with high signal myxomas
  243. 243. Albright’s Disease
  244. 244. CLASSICCase #1377 year femaleAlbright’s disease
  245. 245. X-ray at 13 yearsminimal changes offibrous dysplasia
  246. 246. More changes at 18 years
  247. 247. Stress symptoms in both hips at age 24
  248. 248. Close up right hipage 24
  249. 249. cancellous graftPost op x-ray with fibular struts in both femoral necks
  250. 250. Cibagram showing thedifference between therough serrated edges ofthe café-au-lait spots offibrous dysplasia on topcompared to the smoothedges of neurofibromatosisseen on the bottom
  251. 251. Case #760 35 year female with Albright’s disease
  252. 252. Close up left hip
  253. 253. Frog lateral left hip
  254. 254. R L CT scan shows more disease on left side
  255. 255. Café-au-lait spot in left axilla
  256. 256. Photomic from proximal femoral biopsy
  257. 257. fibrousHigher power showing metaplastic bone
  258. 258. Case #760.1 40 year female a short left leg and early menarchy
  259. 259. Case #76127 year femaleAlbright’s diseasehumerus
  260. 260. Shepherd’s crookdeformity proximalfemur
  261. 261. Post op x-ray showingattempt to correct thevarus deformity
  262. 262. X-ray 1 year later withfailure of fixation dueto nail cut out
  263. 263. Side plate on left tibiato reduce stress pain
  264. 264. One year later with new DHS fixation to replace failed nail
  265. 265. Case #762 10 year female with Albright’s of pelvis and spine
  266. 266. Coronal CT of LS spine
  267. 267. Sagittal CT scan
  268. 268. Axial CT scan
  269. 269. Sagittal T-1 MRI LS spine with multi level disease
  270. 270. Axial T-2 MRIof LD spine
  271. 271. Case #763 mandibular lesion 37 year female with Albright’s disease
  272. 272. View of opposite side of mandible
  273. 273. AP pelvis
  274. 274. IM nail for stresspain
  275. 275. Case #76464 year femaleAlbright’s diseasewith stress painproximal femur
  276. 276. Bone scan
  277. 277. Post op bipolarprosthesis
  278. 278. DesmoplasticFibroma of Bone
  279. 279. Desmoplastic Fibroma of Bone Desmoplastic fibroma of bone is an extremely aggressive, butbenign, fibrous lesion seen in long bones as well as the mandible.It represents 1% of all bone tumors and occurs typically in childrenand young adults with equal sex distribution. The long bonesinvolved include the distal femur, proximal tibia, proximalhumerus and the lower half of the pelvis. Radiographically, theselesions have an aggressive and moth-eaten appearance, erodinginto cortical bone and breaking out into the adjacent soft tissue,much like the low grade fibrosarcoma. Histologically, the tumorappears identical to aggressive fibromatosis seen in soft tissue withintense collagen formation by aggressive-appearing fibroblasts withan occasional mitotic figure. With curettement alone, the recurr-ence rate is very high, in the neighborhood of 40%, and for thisreason most authorities suggest a wide resection and possibleprosthetic replacement. This tumor will not metastasize to the lung.
  280. 280. CLASSICCase #13827 year femaledesmoplastic fibromadistal femur
  281. 281. Lateral view
  282. 282. Photomic
  283. 283. Case #13913 year femaledesmoplastic fibromaproximal fibula
  284. 284. Lateral view
  285. 285. Coronal T-1 MRI
  286. 286. Coronal T-2 MRI
  287. 287. Proximal fibular resection specimen
  288. 288. growth plate Cut specimen in path lab
  289. 289. Case #765 25 year female with desmoplastic fibroma prox tibia
  290. 290. Lateral view
  291. 291. Bone scan
  292. 292. CT scan
  293. 293. Another CT scan
  294. 294. Coronal T-1 MRI
  295. 295. Axial PD MRI
  296. 296. Post op x-ray followingcurettement andcementation
  297. 297. Lateral view
  298. 298. Case #76625 year femaledesmoplastic fibromaproximal femur
  299. 299. Post op x-ray followingcurettage, IM nail andbone graft
  300. 300. X-ray 3 years laterand nail removed
  301. 301. Case #76728 year maledesmoplastic fibromadistal femur
  302. 302. 6 months later withprogression of diseaserequiring distal femoralresection and TKA
  303. 303. Lateral view
  304. 304. Case #76819 year femaledesmoplastic fibromadistal femur
  305. 305. Case #769 30 year male with desmoplastic fibroma distal radius
  306. 306. Lateral view
  307. 307. Photomic
  308. 308. Case #770 6 year male with desmoplastic fibroma distal radius
  309. 309. Case #771 22 year female with desmoplastic fibroma prox ulna
  310. 310. Fibrosarcoma of Bone
  311. 311. Fibrosarcoma of Bone Fibrosarcoma of bone is a malignant spindle cell sarcoma seen inan older age group with a peak incidence in the fourth decade oflife. It is ten times less frequent than osteosarcoma but involvessimilar bones including the distal femur, proximal tibia, pelvis,proximal femur and proximal humerus. It is rare in the spine, handor foot. Radiographically, the fibrosarcoma has a permeative lyticappearance in the metaphyseal ends of long bones, similar to thatseen in lymphomas or malignant fibrous histiocytoma. Histo-logically, the tumor demonstrates aggressive fibroblastic activitywith collagen formation similar to what is seen in desmoplasticfibromas but with a much higher mitotic index. The lesions can below grade or high grade. The low grade lesions carry a goodprognosis with a low incidence of metastasis to the lung, whereasthe high grade lesions, just like with osteosarcoma, are moreaggressive with a worse prognosis and a higher potential for
  312. 312. metastasis. Treatment consists of wide local resection of the tumor,if possible. If the lesion is high grade, adjuvant therapies includinglocal radiation therapy and chemotherapy are frequently implementedto reduce the chance of pulmonary metastasis. Limb salvageprosthetic reconstructions are frequently utilized as in the case ofosteosarcoma.
  313. 313. CLASSICCase #14061 year malepathologic fracture thrufibrosarcoma distal femur
  314. 314. Lateral view
  315. 315. 3 months later afterORIF not knowingthis was a sarcoma tumor
  316. 316. Another 3 monthsgo by with continuedtumor growth
  317. 317. Photomic from biopsy and amputation 6 months out
  318. 318. Case #141 29 year female with fibrosarcoma 5th metatarsal
  319. 319. Case #77226 year malefibrosarcomadistal femur
  320. 320. Cut specimen in pathlab after amputation
  321. 321. Macro section tumor
  322. 322. Photomic
  323. 323. Case #773 44 year female with fibrosarcoma femoral neck
  324. 324. Cut specimen in path lab after internal hemipelvectomy
  325. 325. Macro section tumor
  326. 326. Photomic
  327. 327. Case #774 43 year female with fibrosarcoma femoral neck
  328. 328. Laminogram cut
  329. 329. Marginal resection of head and neck cut in path lab
  330. 330. Photomic
  331. 331. 1 year post op withbipolar hip recon
  332. 332. Case #77562 year femalepath fracture prox femurthru primary fibrosacomathought to be metastaticbreast disease
  333. 333. Post op x-ray followingORIF at which time theunexpected biopsydiagnosis of primarysarcoma was made
  334. 334. Surgical specimenafter wide resectionentire proximal femurwith fixation devise
  335. 335. Specimen cut inpath lab tumor
  336. 336. Photomic
  337. 337. Proximal femoral recon with custom bipolar arthroplasty
  338. 338. Post op x-ray
  339. 339. Case #776 48 year female with fibrosarcoma distal femur
  340. 340. Resected distalfemur cut in path lab tumor
  341. 341. Photomic
  342. 342. Post op x-ray withcustom prosthesis
  343. 343. Case #77742 year femalefibrosarcoma prox tibia
  344. 344. 2 years later
  345. 345. 4 more years withfinal diagnosis andamputation
  346. 346. Lateral view
  347. 347. Photomic
  348. 348. Pulmonary met 4 years after amputation
  349. 349. metmet Autopsy photo of multiple lung mets
  350. 350. Case #77864 year malefibrosarcomaproximal humerus
  351. 351. Case #77973 year malefibrosarcomadistal humerus
  352. 352. Lateral view
  353. 353. Case #780 69 year female with fibrosarcoma proximal ulna
  354. 354. AP view
  355. 355. tumor CT scan
  356. 356. tumorradialheadProximal radius and ulna resection with custom implant
  357. 357. Photomic
  358. 358. Prosthesis in place ready for skin closure
  359. 359. median nerve radial nerve Another view of implant prior to closure
  360. 360. Post op x-ray
  361. 361. Case #781 tumor 20 year female with fibrosarcoma sacrum
  362. 362. Case #78221 year malefibrosarcomamandible
  363. 363. Case #78338 year femalefibrosarcomaarising from monostoticfibrous dysplasiaproximal tibia
  364. 364. Previous fibrous dysplasianew fibrosarcoma
  365. 365. Lateral view
  366. 366. Malignant Fibrous Histiocytoma of Bone
  367. 367. Malignant Fibrous Histiocytoma of Bone Malignant fibrous histiocytoma of bone is a relatively new patho-logical entity in bone tumors, compared to soft tissue tumorswhere malignant fibrous histiocytoma is a more common tumor.Malignant fibrous histiocytoma (MFH) of bone occurs in a middleor older age group, more commonly in males than females. It isfound typically in the same locations as fibrosarcoma or osteo-sarcoma, including the distal femur, proximal tibia, and proximalhumerus. Radiographically, it is an aggressive permeative lesionin metaphyseal bone with significant cortical destruction that canbreak out into the adjacent soft tissue. Histologically, there isevidence of malignant fibroblasts as well as atypical histiocytesand giant cells, exactly as we see in soft tissue MFH. Because thisis usually a high grade tumor, the prognosis is quite guarded witha high potential for metastasis to the lung, similar to fibrosarcomaand osteosarcoma. Treatment consists of a wide surgical resection
  368. 368. with prosthetic reconstruction when possible, along withadjuvant chemotherapy to reduce the chance of pulmonarymetastases.
  369. 369. CLASSIC Case #14226 year male with pathfracture thru MFHfemoral neck
  370. 370. Coronal T-1 MRI
  371. 371. Coronal T-2 MRI
  372. 372. Axial T-1 MRI
  373. 373. Cut femoral head in path lab
  374. 374. Photomic
  375. 375. Case # 143 50 year female MFH distal femur
  376. 376. Sagittal T-1 MRI
  377. 377. tumor tumorAxial proton density MRI
  378. 378. Photomic
  379. 379. Post op x-raycustom TKA
  380. 380. Lateral view proxend of implant
  381. 381. Case #143.1 Multifocal MFH 37 year male with painful lump behind knee for 3 months
  382. 382. Cor T-1 T-2 Gad
  383. 383. Axial T-1 T-2Gad
  384. 384. 1906Cor T-2 Axial T-2
  385. 385. tumorWide surgical resection and rotating hinge recon
  386. 386. Post op X-ray
  387. 387. Case #784 27 year female MFH proximal femur
  388. 388. Surgical incision to include biopsy site over trochanter
  389. 389. biopsyProximal femoral resection with biopsy site
  390. 390. Proximal femoral resection cut in path lab
  391. 391. Photomic
  392. 392. Custom proximal femoral resection bipolar prosthesis
  393. 393. Ready for abductor attachment
  394. 394. Closure soft tissue flaps
  395. 395. Abductor tendon sutured to implant
  396. 396. Post op x-ray
  397. 397. 10 years later withstem loosening due tostress shielding
  398. 398. X-ray 2 years afterrevision with theCompress system toavoid further stressshielding
  399. 399. 5 years out showinghypertrophic bonyresponse at the bonespindle interphase
  400. 400. Case #78527 year femaleMFH distal femur
  401. 401. Lateral view
  402. 402. X-ray of cementedstem of customimplant soon aftersurgery
  403. 403. 11 years later withstem loosening fromstress shielding
  404. 404. 4 years after revisionsurgery with stemfracture which wasrevised to a Compresssystem which becameinfected resulting inamputation
  405. 405. Case #78630 year femaleMFH distal femur
  406. 406. Bone Scan
  407. 407. Coronal T-1 MRI
  408. 408. Coronal gad contrast MRI
  409. 409. Another coronal gad contrast MRI
  410. 410. Case #787 35 year male with MFH femoral head
  411. 411. Frog lateral
  412. 412. CT scan
  413. 413. Bone scan
  414. 414. Case #78837 year malepath fracture thruMFH proximal femur
  415. 415. Bone scan
  416. 416. Biopsy site at time of surgical resection
  417. 417. biopsy siteProximal femoral resection specimen
  418. 418. Tumor site uncovered on back table
  419. 419. Photomic
  420. 420. Custom proximal femoral bipolar prosthesis ready for implant
  421. 421. Post op x-ray
  422. 422. Case #789 49 year male with path fracture thru MFH prox femur
  423. 423. Bone scan
  424. 424. Post op x-ray afterORIF for what thesurgeon thought wasa metastatic tumor
  425. 425. Surgical specimenafter wide resectionproximal femur withentire fixation devicefor primary sarcoma
  426. 426. Photomic
  427. 427. Reconstruction withcustom proximalfemoral bipolarprosthesis
  428. 428. Case #790 36 year male with myxoid MFH distal femur
  429. 429. Lateral view
  430. 430. Oblique view
  431. 431. CT scan
  432. 432. Bone scan
  433. 433. Coronal T-1 MRI
  434. 434. Sagittal T-1 MRI
  435. 435. Sagittal T-2 MRI
  436. 436. Case #79175 year maleMFH patella
  437. 437. CT scan
  438. 438. tumorSoft tissue window CT scan
  439. 439. Axial T-1 MRI
  440. 440. Sagittal T-1 MRI
  441. 441. Case #79234 year maleMFH proximal tibia
  442. 442. biopsy siteWide resection proximal tibia
  443. 443. Custom long stemspherocentric TKAcomponents
  444. 444. Post op x-ray afterautoclaving the surgicalspecimen and replacingit over a long stemtibial component
  445. 445. Case #793 infarct36 year femaleMFH at site of aprevious bone infarct
  446. 446. infarct Different rotation
  447. 447. glenoidWide resectionproximal humerus biopsy
  448. 448. Resected surgicalspecimen biopsy
  449. 449. Photomic
  450. 450. Close up
  451. 451. Autoclaved specimencemented over longstem Neer prosthesis
  452. 452. Reimplanted
  453. 453. Post op x-ray
  454. 454. 11 years laterwith proximal migrationand loss of autoclavedbone
  455. 455. Case #795 tumor 41 year female with MFH pelvis
  456. 456. tumorCoronal T-1 MRI
  457. 457. tumor Axial T-1 MRI
  458. 458. Case #795.1 54 year old male with right hip pain for 3 months
  459. 459. CT scan
  460. 460. Bone scan
  461. 461. Cor T-1 STIR Gad
  462. 462. Axial T-1 T-2
  463. 463. Chest CT
  464. 464. Case #79643 year femaleMFH pelvis
  465. 465. Case #79775 year femaleMFH sacrum tumor
  466. 466. Case #798 43 year female with multifocal MFH tibia and radius
  467. 467. Sagittal T-1 MRI
  468. 468. Coronal T-2 MRI
  469. 469. Distal radius
  470. 470. Case #79955 year malemetal associatedMFH with pathfracture at tip offemoral component
  471. 471. Close up x-rayshowing permeativelysis at fracture site
  472. 472. OR x-ray showing cortical lysis at fracture site
  473. 473. Biopsy photomic at time of surgery
  474. 474. Immediate post opX-ray
  475. 475. X-ray several weekslater tumor
  476. 476. Case #80084 year malemetal associated MFHaround femoralcomponent THA loose stem
  477. 477. X-ray 3 months afterrevision surgery
  478. 478. 3 months later we seecortical lysis medially
  479. 479. 5 months after anotherrevision surgery withextensive corticallysis 2nd to MFH
  480. 480. More lysis at tipof stem from MFHHemipelvectomywas carried outand died soon afterwith pulmonary mets
  481. 481. Leiomyosarcoma of Bone
  482. 482. Leiomyosarcoma of Bone Leiomyosarcoma of bone is an extremely rare diagnosis withfewer than 50 cases in the world literature. It occurs typically inmiddle to older age adults in the same location in the skeleton asfibrosarcoma, MFH, and osteosarcoma are found in; distal femur,proximal tibia and upper humerus. Radiographically, these lesionshave a permeative lytic appearance, similar to a lymphoma,withextensive cortical breakdown and extension into the surroundingsoft tissue. Histologically, the malignant spindle cells with collagenformation look like a fibrosarcoma or MFH so that the only waya diagnosis can be established is by using the specific immuno-histochemical staining for smooth muscle protein. Treatment usuallyconsists of wide surgical resection and prosthetic implantation.Because of the poor prognosis with high grade lesions, they arefrequently treated with adjuvant systemic chemotherapy andradiation which is not very effective, especially in old patients.
  483. 483. CLASSIC Case #144 69 year female with leiomysarcoma proximal humerus
  484. 484. Rotational view
  485. 485. Bone scan
  486. 486. Surgical photo ready for wide resection including biopsy site
  487. 487. tumor bulge humeralcut head glenoid Wide resection proximal humerus
  488. 488. cutSurgical defect ready for reconstruction
  489. 489. Resected specimen
  490. 490. Specimen cutin path lab
  491. 491. Photomic
  492. 492. Custom long stemNeer prosthesis inposition
  493. 493. Cement reconstructionaround proximal stem
  494. 494. Immediate post opX-ray cement
  495. 495. Case #14546 year femaleleiomyosarcomaproximal humerus
  496. 496. Coronal proton density MRI

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