Vol 13 ppt
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The document discusses eosinophilic granuloma, a benign histiocytic disorder most commonly seen in children ages 5-15. It often presents as inflammatory bone pain and can resemble conditions like Ewing's sarcoma on x-ray with its permeative, onion-skin appearance. Microscopically, biopsies show large pale histiocytes speckled with eosinophils. Treatment involves surgical curettage or resection for symptomatic relief with a good prognosis.
Vol 13 ppt
- 1. Volume 13 Adamantinoma----------------Case 214-218 & 1094-1098 Chordoma----------------------Case 219-224 & 1099-1106 Histiocytoses Eosinophillic granuloma--Case 417-444 Hand-Schiller-Christian --Case 445-446 Letter Siwe disease--------Case 447-450 Sinus histiocytosis---------Case 450.1 Rosai-Dorfman’s disease
- 2. Adamantinoma
- 3. Adamantinoma The adamantinoma of bone is an extremely rare primary sarcoma of bone, accounting for about .3% of all malignant tumors of bone, and in 90% of the cases it will be seen in the diaphyseal portion of the tibia, especially in the anterior cortex. It occurs equally in males and females, typically in the second and third decade of life. The tissue of origin still remains a mystery but immunohistochemical stains suggest an epithelial origin that might account for why they are more common just beneath the skin in the anterior cortex of the tibia. Radiographically, the adamantinoma takes on the appearance of a fibrous dysplastic lesion or perhaps osteofibrous dysplasia of the tibia. The lesion appears benign with a lytic core surrounded by dense, reactive fibro-osseous bone that frequently dilates the anterior cortex and may be multiloculated in appearance. The tumor is quite slow growing and is usually painful, whereas in fibrous dysplasia and osteofibrous dysplasia, the patients remain asymptomatic. If a
- 4. so-called fibrous dysplastic lesion continues to grow past maturity, a physician should suspect adamantinoma, especially if the lesion is painful, and look for the characteristic nests and cords of epithelial- looking cells surrounded by fairly benign-appearing fibro-osseous tissue on histological specimens. It is very rare for this tumor to metastasize to different sites but occasionally it will metastasize to regional lymph nodes and the lung. Treatment for this low grade lesion is purely surgical, consisting of a wide local resection, frequently a segmental resection of the mid portion of the tibia, and reconstruction with a large bone allograft over an intramedullary nail. There have been a few rare cases in the literature where adamantinoma has arisen out of a pre- existing osteofibrous dysplastic lesion.
- 5. CLASSIC Case #214 25 year male with adamantinoma tibia
- 6. Lateral view
- 7. Wide resection specimen opened on back table
- 8. Photomic
- 9. Surgical specimen after 3 mins in autoclave
- 10. Autoclaved tibia replaced over IM nail and rotated 180 degrees
- 11. Cancellous autogenous iliac chips placed between tibia and roughed up fibular
- 12. 1 year later showing union of prox tib-fib step ladder synostosis
- 13. 3 years post op with solid union to autoclaved bone
- 14. 7 years post op with symptoms of ankle pain Turns HIV positive
- 15. One year later with osteopenia about painful and swollen ankle
- 16. Sagittal T-1 MRI shows recurrent tumor above ankle
- 17. Cut amputation specimen showing excellent osseointegration between fibula & autoclaved tibia tumor
- 18. autoclaved tibia reactive live bone Photomic at tibia-fibula interphase
- 19. Case #215 22 year female adamatinoma distal tibia
- 20. Photomic
- 21. Another photomic
- 22. Wide resection distal tibia biopsy site
- 23. Autoclaved specimen cemented over IM nail including total ankle
- 25. Post op lateral x-ray
- 26. AP x-ray showing fibular recurrence resulting in BK amp 2 years later
- 27. Case #216 11 year female with adamantinoma arising from osteofibrous dysplasia tibia
- 28. AP view
- 29. Bone scan
- 30. Low power photomic with osteofibrous dysplasia to right and adamantinoma to left
- 31. Photomic with cords and nests of epithelioid cells
- 32. Higher power of cords and nests of epithelioid cells
- 33. 4 years following segmental resection and allograft recon over IM nail
- 34. Case #217 25 year female adamantinoma tibia
- 35. Close up AP view
- 37. Case #218 58 year female adamantinoma proximal tibia
- 38. Lateral view
- 39. Bone scan
- 42. Axial T-2 MRI
- 43. Another axial T-2 MRI showing cystic fluid-fluid levels
- 44. Photomic
- 45. Another photomic
- 46. Case #1094 15 year female with adamantinoma arising from osteofibrous dysplasia
- 47. AP view several years later showing slow progression and fibular involvement as well
- 48. Lateral view showing path fracture
- 49. Photomic showing combined fibro-osseous and adamantinoma histology
- 50. Photomic showing mostly adamantinoma histology
- 51. Another photomic
- 52. 6 months following curettement and cancellous allograft placement
- 53. 9 months later
- 54. AP view same time
- 55. 3.5 years later showing good healing and no progression of disease
- 56. Lateral view at same time
- 57. Case #1095 58 year female with adamantinoma tibia
- 58. Sagittal T-1 MRI
- 59. Sagittal T-2 MRI
- 60. Case #1096 18 year male with adamantinoma tibia
- 61. Bone scan
- 62. Sagittal T-2 MRI
- 63. Axial T-2 MRI
- 64. Case #1096.1 Pseudo adamantinoma 78 year male with chronic osteomyelitis tibia since age 2 years with recent fungating lesion anterior tibia
- 65. Case #1097 14 year male with adamantinoma distal tibia and fibula
- 66. Coronal T-1 MRI
- 67. Photomic
- 68. Case #1098 16 year male with adamantinoma mandible
- 69. AP x-ray
- 70. Oblique view
- 73. Photomic
- 74. Another photomic
- 75. Chordoma
- 76. Chordoma The chordoma is a very rare malignant tumor of bone that accounts for only 4% of all malignant bone tumors. It arises from the primitive notochord of the axial skeleton and is most commonly seen in the lower portion of the sacrum, accounting for 50% of the chordomas. 37% arise in the spheno-occipital area and a small number occur in the cervical and lumbar spine. The more common sacral lesions are seen in an older age group between the ages of 40 and 80 years, compared to the spheno-occipital chordomas that occur in a younger adult age group. The later carry a much worse prognosis because of the location at the base of the skull. The chordoma is clinically similar to a mucinous type of chondrosarcoma. In the sacral area, chordomas are usually attached to the anterior portion of the distal three segments of the sacrum and grow in the retroperitoneal space, pressing up against the rectum where eventually they will present with clinical symptoms related to constipation and can be picked up on a rectal examination. Because
- 77. the tumor is very slow growing, it rarely causes significant pain symptoms. The radiographic findings are frequently not very obvious, even with large tumors that are better evaluated by soft tissue technique, such as CT scan or, better yet, MRI. Microscopically, the chordoma has a mucinous appearance similar to a low grade myxoid chondrosarcoma, but the diagnostic feature is the “signet ring” appearance of the physaliferous cells that have a peripheral nucleus, a large cytoplasmic inclusion of physaliferous mucinous material that can look a bit like a liposarcoma. Treatment for the chordoma consists primarily of a wide surgical resection, which sometimes is very difficult, especially with lesions extending into the upper sacral segments where the nerve roots become a problem and may result in significant neurogenic bowel and bladder complications. Even with surgical treatment, the local recurrence rate is very high so that post op radiation therapy is recommended. It has cut the local recurrence rate to about 30% compared to 65% without RT. Recurrences can occur locally up to
- 78. fifteen years after the original surgery. Pulmonary metastases are extremely rare and systemic chemotherapy is not indicated for this tumor.
- 79. CLASSIC Case #219 45 year male with chordoma sacrum
- 80. Lateral view
- 81. Sagittal T-1 MRI
- 82. Coronal T-1 MRI
- 83. Axial T-1 MRI
- 84. Axial T-2 MRI
- 85. Resected specimen cut in path lab
- 86. Photomic showing physaliferous cells
- 87. Case #220 Sagittal T-1 MRI 74 year male with chordoma sacrum
- 88. Sagittal T-2 MRI
- 89. Case #220.1 CT scan Sacral chordoma 48 year male with 4 mo history of sacral pain
- 90. Bone scan
- 91. Axial MRI T-1 T-2
- 92. Sag T-2
- 93. Cor T-2 Gad
- 94. Post op x-ray
- 95. Case #221 82 year male with chordoma lower sacrum
- 96. Lateral view
- 97. Sagittal T-1 MRI
- 98. Sagittal T-2 MRI tumor
- 100. Case #222 CT scan 65 year female with chordoma sacrum and buttock
- 101. tumor Axial T-2 MRI
- 102. Axial T-2 MRI at higher level
- 103. femur tumor Sagittal T-2 MRI
- 104. Photomic
- 105. Case #222.1 T-2 MRI Sacral chordoma 65 yr male
- 106. Rebar and cement reconstruction
- 107. Case #223 49 year male with recurrent chordoma sacrum
- 108. tumor CT scan
- 109. Sagittal T-2 MRI tumor
- 110. tumor Axial T-2 MRI
- 111. Wide resection entire CD rod sacrum and recon with L-5 CD rods and Steinman pins prior to cementation Steinman pins
- 112. CD rods After cementation cement sacrum
- 113. Post op x-ray cement
- 114. L-4 L-5 Lateral view cement cement coccyx
- 115. Case #224 Sagittal T-1 MRI L-3 S-1 55 year male with chordoma lumbar spine
- 116. Resection L-4, L-5 and part of S-1
- 117. Resected vertebrae
- 118. Photomic
- 119. sacrum Anterior recon with large bone allograft and plate
- 120. allograft CD rods Post op lateral x-ray
- 121. Case #1099 53 year female with chordoma sacrum
- 122. Bone scan
- 123. Oblique bone scan
- 124. tumor CT scan one year later
- 125. Sagittal T-2 MRI
- 126. Sagittal T-2 MRI tumor
- 127. tumor Axial T-2 MRI
- 128. Photomic
- 129. Case #1100 CT scan 61 year male with chordoma distal sacrum
- 130. Bone scan bladder
- 131. Sagittal T-1 MRI tumor
- 132. Sagittal T-2 MRI tumor
- 133. Axial T-1 MRI
- 134. Photomic
- 135. Case #1101 Axial T-1 MRI 60 year female with chordoma sacrum
- 136. Axial T-2 MRI
- 137. Sagittal T-2 MRI
- 138. Case #1102 Axial and coronal CT scan 37 year male with chordoma in body of L-4
- 139. CT scan myelogram with block at L-4
- 140. Myelogram showing block at the L-4 level
- 142. Photomic
- 143. Post op x-ray following removal of L-4 body and recon with fibular strut from L-3 to L-5
- 144. Lateral view of fibular strut reconstruction
- 145. 8 mos later with collapse of L-3 on L-5
- 146. 10 more months and further collapse of L-3 on L-5
- 147. Sagittal T-1 MRI showing collapse and kinked dural sac
- 148. X-ray following restoration of collapsed vertebral space with iliac strut graft in front and CD rods behind
- 149. AP view
- 150. Case #1103 34 year male with chordoma L-5
- 151. tumor CT scan at L-5 shows chondroid like tumor arising from the postero-lateral elements of L-5
- 152. tumor Another CT cut
- 153. Another
- 154. tumor Axial T-1 MRI
- 155. Axial T-2 MRI
- 156. Another axial T-2 MRI
- 157. Sagittal T-2 MRI
- 158. Another sagittal T-2 cut
- 159. Photomic
- 160. Case #1104 65 year male with a chordoma of L-2 Myelographic study showing complete block at L-2 level
- 161. Surgical exposure of paraspinous mass at L-2 level
- 162. Post op x-ray showing recon with cement and pins in tumor defect
- 163. Lateral view
- 164. Case #1104.1 Chordoma L-1 49 year male with back pain for 6 mos and recent paraparesis
- 165. Axial CT L-1 level
- 166. Axial T-1 T-2 Gad
- 167. Sag T-1 T-2
- 168. PO x-ray
- 169. PO Cor CT Sag
- 170. Case #1105 54 year male with chordoma C-spine
- 171. Case #1106 42 year female with chordoma base of skull
- 172. Autopsy specimen showing lobulated chondroid looking mass in post fossa
- 173. Macro section
- 174. Photomic
- 175. Photomic
- 176. Histiocytoses Of Bone
- 178. Eosinophilic Granuloma (Langerhans Histiocytosis) The so-called benign histiocytoses, sometimes referred to as histiocytosis X, include various disease conditions such as eosino- phillic granuloma, Hand-Schuller-Christian disease and Letterer- Siwe disease. Eosinophillic granuloma is the most benign of the histiocytic disorders, followed next by Hand-Schuller-Christian disease that presents with an intermediate diffuse process of both bone and soft tissue that can be fatal. Letterer-Siwe disease is the most aggressive and fatal form of the histiocytoses, presenting like leukemia with a very poor prognosis for survival. Eosinophillic granuloma, now referred to as Langerhans histio- cytosis, is a benign histiocytic disorder that frequently presents in children between the ages of 5 and 15 years with a clinical picture that can masquerade as a malignant neoplasm such as Ewing’s sarcoma. It occurs twice as often in males than females. It is usually a monostotic disorder of the skeletal system, however, in
- 179. 10% of cases it will be seen in two or three separate sites. The etiology of this histiocytic process is still unknown but some have postulated a viral etiology. Patients present with inflammatory pain, more severe at night, that may be associated with a low grade fever or elevated sed rate. The most common location is in the skull, followed next by the ribs, pelvis, maxilla, vertebral body, clavicle and scapula, in that order. Besides flat bone involvement, it is commonly seen in the diaphyses of long bones where it can masquerade as Ewing’s sarcoma, but can also occur in metaphyseal bone and is found least commonly in epiphyseal bone. In young children, the condition can be extremely permeative and destructive in nature, taking on the appearance of Ewing’s sarcoma, metastatic neuroblastoma, or acute osteomyelitis. On x-ray, eosinophillic granuloma has an onion-skin appearance similar to a Ewing’s sarcoma. In an older age group, the condition tends to be more focal and more granulomatous in appearance with less permeative change. Microscopically, there are large, pale-
- 180. staining histiocytes speckled with small, bright-staining eosinophils and an occasional giant cell. Eosinophillic granuloma tends to involute spontaneously without treatment and therefore symptomatic treatment should be conservative--simple curettement for diagnostic purposes and perhaps cortical steroid injections can be beneficial to inhibit the inflammatory process. In more difficult parts of the body, such as the spine or pelvis, very low grade radiation therapy can be considered, realizing that this could convert the process to a malignant sarcoma at a later date. In more aggressive forms with multi-focal involvement, especially if there is soft tissue involvement of the skin, lymph nodes or lung, a low dosage chemotherapy program can be considered. Sometimes the low grade eosinophillic granuloma histiocytosis can upgrade to a more aggressive and dangerous form such as Hand-Schiller-Christian disease or even Letterer-Siwe disease. With spinal lesions, spinal cord compression can result in paraparesis requiring laminectomy decompression. However, kyphotic deform- ities in younger patients tend to correct spontaneously without surgery.
- 181. CLASSIC Case #417 17 year male EG distal femur
- 182. Lateral view
- 183. Coronal T-1 MRI
- 184. Sagittal T-2 MRI
- 185. Coronal STIR MRI
- 186. eosinophil histiocyte Biopsy photomic
- 187. Case #417.1 6 yr male with pain in left thigh for 6 months
- 188. CT scan
- 189. Axial T-1 T-2 Gad
- 190. Cor Gad Sag Gad
- 191. Case #417.2 10/08 11/08 12/08 6 year male with pain in thigh for 1 mo.
- 192. 11/08 1/09 Cor T-1 T-2 T-1 T-2
- 193. Case #418 2 year male EG mid femur
- 194. Case #419 8 year male EG mid femur
- 195. Case #420 3 year female path fracture EG femur
- 196. Lateral view
- 197. Case #421 3 year male EG mid femur
- 198. Lateral view
- 199. Case #422 7 year female with EG femur
- 200. Case # 423 14 year male with EG clavicle
- 201. Case #424 16 year male with EG mid clavicle
- 202. Macro section of resection specimen
- 203. Photomic
- 204. Case #425 6 year male with EG proximal humerus
- 205. Case # 426 12 year male with EG mid humerus
- 206. Arteriogram
- 207. Bone Scan
- 208. Case #427 2.5 year male EG distal humerus
- 209. Lateral view
- 210. Case #428 2.5 female with EG proximal ulna
- 211. AP view
- 212. CT scan showing reactive involucrum formation
- 213. More proximal CT cut
- 214. edema Axial T-2 MRI
- 215. Case #429 23 year female with EG right ilium
- 216. Bone scan
- 217. CT scan
- 218. Axial STIR MRI
- 219. Case #429.1 51 year female with 6 mos pelvic pain second to EG
- 220. Bone scan
- 221. Axial T-2 MRI
- 222. Case #429.2 EG pelvis 39 year male with dull aching pain left hip for 9 mos.
- 223. Axial CT scan
- 224. Cor and Sag CT
- 225. Axial T-1 T-2 T-1 T-2
- 226. Cor T-1 Sag T-2
- 227. Case #430 12 year male EG ilium
- 228. Case #431 20 year male with EG scapula
- 229. CT scan
- 230. Coronal T-2 MRI
- 231. Axial T-2 MRI
- 232. Case #432 4 year male EG mid fibula
- 233. Case #433 12 year male with EG posterior rib
- 234. Case #434 x-ray resection specimen 24 year male with EG skull
- 235. CT scan of EG skull
- 236. Photomic
- 237. Case #435 4 year female EG right mandible
- 238. Oral photo of submucosal mass
- 239. Mandibular view
- 240. Full mouth dental x-ray view
- 241. Axial CT scan
- 242. Photomic
- 243. Case #436 3 year male vertebra plana from EG thoracic vertebra
- 244. Sagittal CT scan
- 245. Sagittal T-1 MRI
- 246. Sagittal T-2 MRI
- 247. Sagittal gad contrast MRI
- 248. Case #438 7 year male with EG T-11
- 249. Sagittal gad contrast MRI
- 250. Sagittal T-2 MRI showing protrusion into vertebral canal
- 251. Case #439 new collapse 19 year male with EG collapse new collapse at 3 different levels but at old different times old The lower levels show height restoration as a spontaneous healing process seen in children old old
- 252. Case # 440 7 year female with healing collapsed lumbar vertebra
- 253. Case # 441 15 year male with EG lumbar vertebra
- 254. Case #442 5 year female EG C-3
- 255. Case #443 10 year female EG C-7
- 256. Case #444 skull defect 8 year male EG skull with EG dermatitis
- 257. Severe EG dermatitis back
- 258. Case #444.1 Multifocal osteomyelitis EG pseudotumor 5 year old male with pain in heel and knee for 6 mos
- 259. Sag T-1 T-2 Gad
- 260. Cor T-1 Sag T-2 Axial T-2
- 261. Case #444.2 EG 2 year old male with painful foot for 2 months
- 262. Sag T-1 T-2 Gad
- 263. Axial T-1 T-2 Gad
- 265. Hand-Schiller-Christian Disease Hand-Schiller-Christian disease is the intermediate form of histiocytosis-X that involves predominantly children, two-thirds of the cases being younger than five years of age. The classic triad for this syndrome is diabetes insipitus, exophthalmos, and single geographic lesions involving mostly the skull and pelvic bones. The initial lesions appear like eosinophillic granuloma and, in fact, eosinophillic granuloma can progress into a Hand-Schiller-Christian type syndrome as the disease advances. It is common to have soft tissue involvement of lymph nodes, liver, spleen, lung, brain and kidney as well as skin changes that can be seen in eosinophillic granuloma. Histologically, the same histiocytic cells as are seen with eosinophillic granuloma are present, along with eosinophils. In the later stages, foam cells and cholesterol deposits are typical. as the disease progresses and more and more soft tissue organs are affected, the prognosis worsens with an overall fatally rate of
- 266. 10-30%. Treatment consists of local surgical treatment plus systemic treatment consisting of therapeutic protocols similar to those used in leukemic patients.
- 267. CLASSIC Case # 445 5 year male with HSC disease skull
- 268. reactive bone granuloma Photomic from edge of skull lesion
- 269. Photomic showing foam cells
- 270. Higher power of foam cells
- 271. Photomic showing cholesterol deposits
- 272. Case # 446 6 year female with HSC disease skull, spine and pelvis
- 273. Pelvis
- 274. Spinal lesions
- 275. Biopsy photomic
- 276. Photomic showing cholesterol deposits
- 278. Letterer-Siwe Disease Letterer-Siwe disease is the least common of the histiocyoses comprising about 10% of all histiocytic disorders. It is a pro- gressive, acute syndrome in children under three years of age, involving multiple visceral organ systems, such as the spleen, lymph nodes and skin, associated with purpura, bleeding gums, and multiple lesions similar to those seen in Hand-Schiller- Christian disease. The skull and pelvis are frequently involved. The skeletel lesions tend to be more diffuse than with the other histiocytoses and take on a picture similar to that of leukemia or diffuse lymphoma. These patients usually die of bacterial infections within one or two years of their acute clinical onset because of bone marrow suppression. Histologically, the lesions look very similar to eosinophillic granuloma or Hand-Schuller-Christian disease, although it is unusual to see foam cells in this form of histiocytosis. Treatment consists of chemotherapeutic agents similar to those used in leukemia.
- 279. CLASSIC Case #447 3 year female with LS disease skull
- 280. AP view
- 281. Chest x-ray with diffuse involvement ribs, scapulae & humeri
- 282. Diffuse involvement pelvis and hips
- 284. Photomic showing histiocytes and eosinophils
- 285. giant cell histiocyte poly Photomic showing giant cell, polys & histiocytes
- 286. Case # 448 15 month male with LS skull
- 287. T-12 collapse
- 288. Femoral disease
- 289. Photomic
- 290. Case #449 3 year male with LS skull
- 291. Femoral disease
- 292. Case #450 2 year old female with LS skull
- 293. Sinus Histiocytosis Rosai-Dorfman’s Disease
- 294. Sinus Histiocytosis Rosai-Dorfman’s Disease Sinus histiocytosis is a rare and new variant of the histiocytoses which is characterized by enlarged lymph nodes in the head and neck area in 80% of cases along with bony involvement in 40% of cases. It is an aggressive form of the histiocytoses that is seen in teen agers and young adults. Symptoms may include fever, weight loss and malaise. The bony lesions may be solitary or multifocal and suggest inflammatory disease such as chronic osteomyelitis or EG. The pathology shows mononuclear or multinuclear giant cells with lymphs in the cytoplasm of the giant cells. Other inflammatory cells such as plasma cells, lymphocytes and foamy histiocytes may be seen. 10% of those with bone lesions die of the disease from extra- skeletal involvement of the lungs and kidneys.
- 295. Case #450.1 Rosai-Dorfman’s disease 52 year old female with knee pain for 1 year
- 296. Sag T-1 T-2 Gad
- 297. Axial T-1 T-2 Gad
- 298. Cor T-1 T-2 Gad
- 299. Post op X-ray