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Volume 16

  1. 1. Volume 16Neurogenic soft tissue tumors Neurilemoma-----------------Case 315-321 & 1214-1220 Solitary neurofibroma--------Case 322-326 Neurofibromatosis------------Case 327-338 & 1221-1229 Malignant schwannoma------Case 339-344 & 1230-1234Rhabdomyosarcoma------------Case 345-349Alveolar soft part sarcoma-----Case 350-353Synovial sarcoma---------------Case 354-363 & 1235-1242Granular cell tumor-------------Case 1234.1
  2. 2. Neurogenic Tumors
  3. 3. Neurilemoma
  4. 4. Neurilemoma The neurilemoma, sometimes referred to as a benign schwanoma,is a nerve sheath tumor arising from the schwann cell surroundingthe axons of peripheral nerves. It affects individuals between theages of 20 and 50 years and occurs equally in males and females. Itis seen most commonly in spinal roots and superficial nerves,especially on the flexor surface of both the upper and lower limbs.The lesions are usually solitary but in the case of neurofibromatosismultiple lesions may occur. These benign lesions are slow growingand very rarely cause neurological symptoms or defect becauseof their benign behavior. As opposed to the more common neuro-fibroma, the neurilemoma is typically a round structure attached tothe periphery of a nerve whereas the neurofibroma is fusiform inshape and is located in the center of a peripheral nerve. The MRIstudy is the best diagnostic study for this lesion and shows lowsignal abnormality on the T-1 weighted image and demonstrates
  5. 5. a very bright, well-marginated spherical lesion on the T-2 weightedimage. Histologically, the neurilemoma has a mixture of dense,fibrotic, Antoni A substance mixed with a more mucinous Antoni Bsubstance. In the Antoni A fibrotic tissue, the histological picture is apalisade of schwann cells and a characteristic and almost diagnosticVerocay body will be seen demonstrating a palisade of schwann cellsas seen in a Japanese lantern. Treatment for this lesion consists of asimple blunt dissection of the tumor from a peripheral nerve whereit is located eccentrically and easy to remove without damaging thesubadjacent nerve. The lesion has a characteristic yellow colorcontrasting with the white strand-like appearance of the adjacentnerve. These tumors have a very low recurrence rate and only rarelywill they convert into a malignant schwannoma. Sometimes the neurilemoma will develop hemorrhagic cysticchanges in patients over the age of 65 years, taking on the appearanceof a hematoma. The lesions can even calcify or form bone as part of adegenerative process, and are sometimes given the name of ancientschwannoma in this case.
  6. 6. CLASSIC Case #315 Coronal T-1 MRI gluteus max 55 year male with neurilemoma proximal sciatic nerve
  7. 7. Axial T-2 MRI
  8. 8. gluteus tumor nerveSagittal T-2 MRI of tumor in sciatic nerve
  9. 9. tumor gluteus nerveSurgical exposure of tumor on sciatic nerve
  10. 10. Photomic of highly cellular Antoni A area
  11. 11. Verocay bodyClose up of Antoni A area
  12. 12. Case #316 Axial T-1 MRI 22 year female with neurilemoma sciatic nerve
  13. 13. tumorAxial T-2 MRI
  14. 14. tumorCoronal T-2 MRI
  15. 15. Coronal gad contrast MRI showing large cystic areas
  16. 16. cystAxial gad contrast MRI
  17. 17. Case #31769 year malecalcifying neurilemomaulnar nerve
  18. 18. nerve tumorSagittal T-1 MRI showing tumor in ulnar nerve
  19. 19. nerve tumor Sagittal T-2 MRI
  20. 20. Axial T-2 MRI showing calcific signal voids
  21. 21. Surgical specimen
  22. 22. Photomic from Antoni A area
  23. 23. Case #318 36 year male with neurilemoma S-1 root
  24. 24. Lateral view
  25. 25. L-5Sagittal T-1 MRI tumor
  26. 26. Sagittal PD MRI
  27. 27. S-1 root S-1 tumorCoronal T-2 MRI
  28. 28. tumor S-1 foramen Coronal T-1 MRI
  29. 29. Case #31924 year female S-2neurilemoma S-2 tumorSagittal PD MRI
  30. 30. S-1Another sagittalPD MRI cut tumor
  31. 31. tumor cystic area tumor Sagittal T-2 MRI
  32. 32. cystic areaSagittal gad contrast MRI
  33. 33. cyst S-2Axial gad contrast MRI
  34. 34. Case #319.1 66 year male with painless presacral tumor mass for 7 years
  35. 35. Axial T-1 T-2Gad
  36. 36. LBP MRISag T-1 in 99 Sag T-1 in 06 Sag T-2 in 06
  37. 37. Cor Gad C+
  38. 38. Case #32045 year maledumbell neurilemomaC-3-4
  39. 39. Myelographic studyshowing extraduraldefect
  40. 40. Case #32128 year male withossifying ancientneurilemoma tibia
  41. 41. Lateral view
  42. 42. Case #321.1 86 year female with ancient schwannoma popliteal space
  43. 43. Sag T-1 Sag PD Sag Gad
  44. 44. Cor T-2 Cor Gad
  45. 45. AxialT-1 T-2 Gad
  46. 46. Case #1214 CT scan 37 year male with neurilemoma S-1
  47. 47. L RBone scan shows signal void S-1 on left side
  48. 48. Sagittal PD MRI
  49. 49. Coronal PD MRI
  50. 50. Case #121542 year male withneurilemoma L-5with lysis of L-5pedicle
  51. 51. Lateral view
  52. 52. CT scan
  53. 53. Another CT scan
  54. 54. Case #1216 Coronal T-1 MRI36 year female with neurilemoma brachial plexus C5-6 roots
  55. 55. Coronal T-2 MRI
  56. 56. Case #1217 Axial T-2 MRI 48 year male with neurilemoma radial nerve
  57. 57. Sagittal T-2 MRI
  58. 58. Surgical specimen cut in path lab
  59. 59. Case #1217.1 Sag T-1 PD Gad 51 year female with 1 yr history of tingling in right thumb
  60. 60. Axial T-1 PD Gad
  61. 61. Radial N.Cor STIR Surgical excision
  62. 62. Case #1217.2 Cor T-1 PD Gad C.46 year female with tender mass located along ulnar nerve
  63. 63. Axial T-2Axial Gad
  64. 64. Case #1217.3 Axial PD FS Gad Upper Lower 36 yr female with two separate lumps in one leg for 1 year
  65. 65. Sag T-1 Gad Gad
  66. 66. Surgical specimen
  67. 67. Case #121836 year male withneurofibromatosis anda neurilemoma in thesciatic nerveAxial T-1 MRI
  68. 68. Sagittal T-1 MRI
  69. 69. Sagittal T-2 MRIshowing tumor arisingfrom sciatic nerve
  70. 70. Axial T-2 MRI
  71. 71. Case #1220.1 Axial PD MRI65 year male with tender lump low in popliteal space 1 yr
  72. 72. posteriortibial art Coronal T-1 Coronal PD
  73. 73. Post tibial nerveSagittal T-1 Sagittal PD
  74. 74. Surgical exposure of the neurilemoma in the post. tib. N.
  75. 75. Post tibial N. Surgical removal of the neurilemoma
  76. 76. Case #1220.2 Sag T-1 T-2 Neurilemoma 63 year female with tingling on bottom of foot for 6 months
  77. 77. Axial T-1 T-2
  78. 78. Cor T-1 T-2
  79. 79. Surgical excision
  80. 80. Case #1220.3 Sag T-142 year male withtender lump bottomof foot for 1 year Gad
  81. 81. Axial T-1 T-2 Gad
  82. 82. SolitaryNeurofibroma
  83. 83. Solitary Neurofibroma The solitary neurofibroma, as opposed to the neurolemoma, isusually a fusiform, sweet potato shaped peripheral nerve sheathtumor that arises centrally from the mid-portion of a peripheral nerve.The lesions are usually small and located in a subcutaneous location.They are seen between the ages of 20 and 30 years in males andfemales equally, and are ten times more common than the neuro-fibromas seen in Von Reckinghausen’s neurofibromatosis. On MRI,the lesions are low signal on T-1 and very bright on T-2, and some-times can be seen arising from a small peripheral nerve. Histo-logically, the lesions have dense Antoni A substance with palisadingschwann cells, similar to that seen in the neurilemoma. Treatmentconsists of simple surgical resection and the recurrence rate is low. Aspecific reactive type neurofibroma occurs in the foot between thethird and fourth toes, in the common digital nerve in the web space.It arises as a result of recurrent compression trauma from wearing
  84. 84. tight shoes. This type of neurofibroma, seen typically in females,can be resected surgically for pain. The so-called amputationneuroma is a bulbous traumatic neurofibroma seen at the end ofan amputation stump where the peripheral nerves have beentransected during the amputation.
  85. 85. CLASSIC Case #322 Sagittal PD MRI 32 year female with neurofibroma foot
  86. 86. Axial STIR MRI
  87. 87. Surgical exposure for excision
  88. 88. nerveSurgical specimen cut in path lab
  89. 89. Photomic showing palisading of schannoma cells
  90. 90. Case #323 Sagittal T-1 MRI tumor 3 month old infant with neurofibroma foot
  91. 91. Sagittal PD MRI
  92. 92. Axial T-2 MRI
  93. 93. Another axial T-2 cut
  94. 94. Case #324 Coronal PD MRI 53 year male with neurofibroma thigh
  95. 95. Coronal T-2 MRI
  96. 96. Axial T-1 MRI
  97. 97. Axial T-2 MRI
  98. 98. nerveResection specimen cut in path lab
  99. 99. Photomic
  100. 100. Case #1220 Axial T-1 MRI56 year female with neurofibroma on sciatic N at notch level
  101. 101. Axial T-2 MRI
  102. 102. Axial Gad contrast MRI
  103. 103. Veracoy bodyPhotomic in area of Antoni-A tissue
  104. 104. Case #1220.1 tumor53 year female with slow growing pelvic neurofibroma 10 yrs
  105. 105. Axial T-1 Axial T-2 tumorGad Sciatic N
  106. 106. tumorAxial T-2 showing origin from both S-1 roots
  107. 107. tumorCoronal Gad
  108. 108. Sag T-2
  109. 109. Case #325 Plastic cut away model 32 year female with Morton’s neuroma
  110. 110. Photomic of traumatic neuroma
  111. 111. Case 326 Plastic model cut away 42 year male with amputation neuromata forearm
  112. 112. Photomic amputation neuroma
  113. 113. Neurofibromatosis
  114. 114. Neurofibromatosis (Von Recklinghausen’s disease) Neurofibromatosis is clinically divided into Type I and type II. Type I involves peripheral nerves and will be discussed in this section: type II is the central type consisting of acoustic neuroma that has nothing to do with peripheral neurofibromatosis. The type I disease is a familial dysplasia, inherited as an autosomal dominant trait, with an incidence of about one in every 3000 births. The condition becomes clinically manifest in the first few years of life with the presence of café-au-laite spots that increase in number and size over time. Unlike the café-au-laite spots seen in fibrous dysplasia, the ones in neurofibromatosis have a smooth edge, some- times referred to as the coast of California. If a patient is found with more than six lesions with smooth-edged café-au-laite spots greater than 1-2 cm in diameter, the diagnosis can be made. Later on in life, the patient will develop numerous cutaneous neurofibromas that are referred to as fibroma molluscum and have the appearance of
  115. 115. small, pedunclated lipomas. The most pathognomic feature ofneurofibromatosis is the large, plexiform neurofibroma associatedwith the larger nerves that can involve an entire extremity and canbe associated with loose, hyperpigmented skin that produces anelephant-like, gross distortion of the skin anatomy referred to aselephantiasis neuromatosa or elephant man syndrome. Skeletaldeformation can be associated with neurofibromatosis, includingscoliosis (which can be quite angular resulting in paraparesis),spinal meningoceles, scalloping of vertebral bodies, and pseudo-arthrosis of the tibia. There can also be associated hypertrophyor localized gigantism in the hand or foot. As opposed to thesolitary neurofibroma, patients with neurofibromatosis run a 10%chance of developing a malignant neurofibrosarcoma that usuallyoccurs during adult life and carries an extremely poor prognosisfor survival.
  116. 116. CLASSICCase #327 fibroma molluscum35 year male type 1neurofibromatosis withlarge plexiformneurofibroma andelephantiasis left arm
  117. 117. Case #32811 year maleneurofibromatosis 1café-au-lait spots &scoliosis
  118. 118. Case #329 Axial T-1 MRI 5 year female with plexiform neurofibromata pelvis
  119. 119. tumorgluteusOblique coronal T-1 MRI
  120. 120. tumor Axial T-2 MRI
  121. 121. Case #330 Sagittal T-1 MRI 35 year male with neurofibromatosis S-2 & 3
  122. 122. Sagittal T-2 MRI tumor
  123. 123. tumorAxial T-1 MRI
  124. 124. tumorAxial T-2 MRI
  125. 125. Case #331 Sagittal T-1 MRI tumor 19 year female with neurofibromatosis and neurilemoma sciatic nerve
  126. 126. Axial T-1 MRI tumor
  127. 127. tumorAxial T-2 MRI
  128. 128. Case #332 Sagittal T-2 MRI15 year female with plexiform neurofibromatosis LS spine
  129. 129. tumorAxial proton density MRI pelvis
  130. 130. tumorSagittal T-2 MRI
  131. 131. Case #333 Sagittal T-1 MRI tumor 45 year male with plexiform neurofibromatosis knee
  132. 132. tumorSagittal T-2 MRI
  133. 133. Case #3344 year male withneurofibromatosis andelephantiasis left armcafé-au-lait spots
  134. 134. X-ray with frail bone
  135. 135. Case #33532 year female with neurofibromatosis hands & gigantism
  136. 136. Case #33630 year male withneurofibromatosis withgigantism 2nd & 3rd toes
  137. 137. Case #33716 year male withneurofibromatosiscervical spine
  138. 138. Severe dysplastic changesin cervical vertebrae
  139. 139. Myelographic studyshowing multipledumbell neurofibromata
  140. 140. Sagittal laminogramshowing hypoplasticvertebrae
  141. 141. Case #338Child with tibialpseuoarthrosis 2ndto neurofibromatosis
  142. 142. Case #1221 CT scan 55 year female with neurofibromatosis and large neurofibroma in sciatic nerve at notch level
  143. 143. Sagittal T-1 MRIshowing large plexiformneurofibroma in buttockarea
  144. 144. Axial T-1 MRI showing plexiform neurofibroma
  145. 145. Axial T-1 MRI at higher level
  146. 146. Case #1222 45 year female with neurofibromatosis and plexiform neurofibroma about heel and ankle area
  147. 147. Axial T-1 MRIshowing tumor betweentalus and heel cord
  148. 148. Coronal T-1 MRIshowing tumor behindankle joint
  149. 149. Coronal T-2 MRI
  150. 150. Sagittal T-2 MRI
  151. 151. Case #1223 Sagittal T-2 MRI 8 year male with neurofibromatosis and plexiform neurofibroma of forefoot
  152. 152. Axial T-1 MRI
  153. 153. Axial T-2 MRI
  154. 154. Coronal T-2 MRI
  155. 155. Case #1223.126 year male with type Ineurofibromatosis andlarge plexiform neuro-fibroma in foot withbony deformation
  156. 156. Sagittal T-1 T-2
  157. 157. Coronal T-1 T-2
  158. 158. Axial T-1 T-2 Gad
  159. 159. Case #1224 27 year male with neurofibromatosis and secondary talar gigantism
  160. 160. Gigantism of 3rd toe
  161. 161. Plexiform neurofibroma deep post compartment leg
  162. 162. Plexiform neurfibromadeep in arch of foot
  163. 163. Axial T-2 MRIplexiform neuro-fibroma in forefootarea
  164. 164. Sagittal T-2 MRIplexiform neurofibromaforefoot area
  165. 165. Resected specimen from posterior leg compartment
  166. 166. Case #1225 18 year male with neurofibromatosis and plexiform neurofibroma calf area
  167. 167. popliteal vesselsSurgical exposure of tumor in calf
  168. 168. Plexiform neurofibromata removed from calf area
  169. 169. Surgical incision
  170. 170. Case #122655 year male withneurofibromatosisand associateddeformity rib cage
  171. 171. Case #122717 year female withneurofibromatosis andsevere spinal kyphosiswith paraplegia
  172. 172. Case #12289 year female withneurofibromatosis withassociated pseudarthrosistibia
  173. 173. Case #122919 year female withneurofibromatosis andassociated deformationforearm bones andradial head dislocation
  174. 174. MalignantSchwannoma
  175. 175. Malignant Schwannoma The malignant schwannoma is a very high grade spindle cellsarcoma arising from the nerve sheaths of peripheral nerves. It canarise denovo from a normal appearing peripheral nerve but is morelikely to arise from a solitary neurofibroma. There is a 5 yearsurvival rate of about 75% in patients over the age of 40 years whena malignant schwannoma arises from a peripheral solitary neuro-fibroma. There is a 10% incidence of malignant schwannoma inpatients with neurofibromatosis and the chance for survival at fiveyears is reduced to 30% and is seen in a younger age group. Themalignant schwannoma is is usually larger than 5 cms and occurstypically in spinal roots or in the larger proximal nerves such asthe sciatic nerve. The lesions are best picked up by MRI of a painfulmass that occurs in patients with neurofibromatosis and may beassociated with neurological deficits distal to the involved area.There may also be a café-au-laite spot in the overlying skin. The
  176. 176. tumor is usually treated by a wide resection, including the entirenerve from which it originates, following which local radiationtherapy is recommended to reduce the chance of local recurrence.Adjuvant chemotherapy is advised even though its benefit isquestionable.
  177. 177. CLASSICCase #339 tumor28 year femalemalignant schwannomasciatic nerve nerveSagittal T-2 MRI
  178. 178. tumorAxial PD MRI
  179. 179. spinal prep siteSubQ plexiform neurofibroma with café-au-lait spot over pelvis
  180. 180. peroneal N sciatic N tumorpost tib N Tumor exposed ready for resection
  181. 181. benignmalignant Scanning lens photomic
  182. 182. Low power malignant photomic
  183. 183. Higher power
  184. 184. Case #340 tumor tumor 16 year female with malignant schwannoma pelvis
  185. 185. necrosis notch Axial CT scan showing large necrotic tumorfilling the pelvis and extruding thru the sciatic notch
  186. 186. Large gluteal mass with café-au-lait spots prior to resection
  187. 187. Tumor exposed beneath gluteus maximus
  188. 188. Large excised tumor on back table
  189. 189. Low power photomic
  190. 190. Higher power photomic
  191. 191. Case #341 46 year male with skin lesions of neurofibromatosis
  192. 192. Close up of fibroma molluscum skin lesions
  193. 193. CT scan tumorLarge malignant schwannoma in adductor compartment
  194. 194. tumorExposed tumor at time of surgery
  195. 195. Excised tumor cut open on back table
  196. 196. Low power photomic
  197. 197. High power photomic
  198. 198. Case #342 25 year male with neurofibromatosis and café-au-lait spot over buttock and malignant schwannoma sciatic N
  199. 199. Surgical exposure of sciatic nerve tumor
  200. 200. malignant benignExcised cut specimen showing benign and malignant parts
  201. 201. Photomic of malignant portion
  202. 202. Case #343 Autopsy specimenAutopsy specimen of 42 year female with neurofibromatosisand malignant schwannoma in area of severe dorsal kyphosis
  203. 203. Sagittal cut autopsy specimen
  204. 204. Photomic of tumor
  205. 205. Case #344 Sagittal T-2 MRI 26 year female with malignant schwannoma peroneal N
  206. 206. Axial T-2 MRI
  207. 207. Photomic
  208. 208. Case #123047 year male withmalignant schwannomalegCoronal T-1 MRI
  209. 209. Axial T-2 MRI
  210. 210. Surgical specimen
  211. 211. Photomic
  212. 212. Higher power
  213. 213. Case #1231 CT scan20 year male with malignant schwannoma paraspinous area
  214. 214. Axial T-2 MRI
  215. 215. Another axial T-2 MRI at different level
  216. 216. Case #1232 17 year male with malignant schwannoma thigh
  217. 217. CT scan
  218. 218. Another CT scan at different level
  219. 219. Case #123336 year female withbenign meningiomaarising within theradial nerveSagittal T-1 MRI nerve
  220. 220. nerveSagittal T-2 MRI
  221. 221. Axial PD MRI
  222. 222. Case #1234 49 year female with myxopapillary ependymoma sacrum
  223. 223. CT scan
  224. 224. CT scan at lower S-2 level
  225. 225. Sagittal T-1 MRI
  226. 226. Sagittal T-2 MRI
  227. 227. Axial T-2 MRI
  228. 228. Granular cell Tumor
  229. 229. Granular Cell Tumors The granular cell tumor is a fairly common soft tissue tumorthat was once thought to be of muscle origin. More recent studieshave proved it to be of neural origin. They are more common inin males than females and are seen typically in a subcutaneouslocation. They are usually painless and less than 3 cm. indiameter. They are more common in blacks and are seen mostcommonly in the fourth thru the sixth decade. By MRI studythe tumors are poorly circumscribed nodular lesions that are lowsignal on T-1 and bright on T-2. Histologically the cells have abenign appearance with a granular appearing cytoplasm. There isa rare and malignant variant of the granular cell tumor. In thecase of the more common benign form the treatment consistsof simple local excision with little chance of recurrence.
  230. 230. Case #1234.1 Axial T-1 Granular cell tumor T-2 FS 34 year old female with a painless lump in calf 4 mos Gad
  231. 231. Cor T-1 Gad
  232. 232. Sag T-1 T-2 FS Gad
  233. 233. Rhabdomyosarcoma
  234. 234. Rhabdomyosarcoma Rhabdomyosarcoma accounts for 20% of all soft tissue sarcomas.The embryonal and alveolar types are seen in the pediatric agegroup while the less common pleomorphic rhabdomyosarcoma isseen in adults. The embryonal rhabdomyosarcoma occurs in children betweenthe ages of 0 and 15 years, and is seen more commonly in boysthan girls, with the head and neck area being the most commonlocation for this tumor. Histologically, it is a round cell tumor similarto Ewing’s sarcoma but in 50% of cases there will be evidence ofcross striation in associated spindle cells and the tumor will haveimmunohistochemical markers for desmin, myoglobin, and actin.Prior to the advent of chemotherapy, this tumor was fatal in about90% of patients, but with current chemotherapy protocols, patientssurvive this tumor in over 80% of cases. Treatment consists of localsurgical resection when indicated, along with postoperative radiationtherapy if the surgical margins are considered contaminated.
  235. 235. The alveolar rhabdomyosarcoma affects children between theages of 10 and 25 years, and is more commonly seen in males thanfemales. It occurs in the head and neck area, but can also be seenin the extremities, especially the thigh and calf. Histologically, thisvariant has a typical alveolar pattern of round cells that gives it itsclinical name and only rarely will there be rhabdomyoblasts in thehistological specimens. Treatment consists of surgical resectionfollowed by radiation therapy if the margins are positive, alongwith adjuvant perioperative chemotherapy. The prognosis forsurvival is much worse than that of embryonal rhabdomyosarcoma. The pleomorphic rhabdomyosarcoma is the rarest variant ofthe group, consisting of 5% of all cases. It occurs in middle-agedor older patients and is most commonly located in large musclegroups in the proximal extremities, usually the lower extremities.In the 1940’s the pleomorphic rhabdomyosarcoma was a commonhistological diagnosis that included many cases of MFH whichat that time was an unpopular histologic diagnosis but a common
  236. 236. one by current pathological criteria. Microscopically, this variantconsists of large, bizarre-appearing giant cells with very atypicalnuclei. The cells stain positive for glycogen and cross striations arefrequently seen in the so-called “candy ribbon” cellular patternswith tandem nuclei on the surface of these rectangular cells. Thisvariant carries a very poor prognosis for survival and the use ofadjuvant chemotherapy is not nearly as effective as it is for thepediatric variants. The mainstay of treatment is wide local resectionwhen possible, followed by radiation therapy, even with negativesurgical margins.
  237. 237. CLASSIC Case #345 Axial T-1 MRI 9 month male with embryonal rhabdomyosarcoma pelvis
  238. 238. Axial T-2 MRI
  239. 239. Coronal gad contrast MRI showing central necrosis
  240. 240. Another coronal gad contrast cut with necrosis
  241. 241. Photomic showing round cells
  242. 242. Higher power
  243. 243. Case #3467 year maleembryonalrhabdomysarcomamaxilla
  244. 244. AP x-ray
  245. 245. Low power photomic
  246. 246. High power
  247. 247. Case #3477 year female with fungating alveolar rhabdomysarcoma leg
  248. 248. Surgical specimen cut in path lab
  249. 249. Photomic
  250. 250. Case #34845 year male with pleomorphic rhabomyosarcoma biceps
  251. 251. Close up of tumor
  252. 252. Lateral x-ray of armshowing tumor mass
  253. 253. Arteriogram showingtumor blush
  254. 254. Surgical exposure during wide resection
  255. 255. Tumor cut in path lab
  256. 256. Photomic showing strap cells
  257. 257. Case #34942 year female withpleomorphicrhabdomysarcomahypothenar hand
  258. 258. Ulnar amputationcut in path lab
  259. 259. biopsyClose up photo tumor
  260. 260. Photomic showing large pleomorphic giant forms
  261. 261. Remaining functional radial hand
  262. 262. Great cosmetic appeal
  263. 263. Alveolar Soft Part Sarcoma
  264. 264. Alveolar Soft Part Sarcoma The alveolar soft part sarcoma is a high grade round cell sarcomaaffecting patients between the ages of 15 and 35 years, with a pre-dominance for females. The tumor usually arises from deep muscletissue in the lower extremities, most commonly in the thigh. Thistumor is a slow growing lesion that carries a very poor prognosisbecause of a high potential for pulmonary metastases. It is felt tooriginate from neurogenic stem cells, however, its histologicalappearance is similar to that of the alveolar variant of the rhabdo-myosarcoma which has a similar microscopic appearance. Treatmentusually consists of an attempt at wide surgical resection followedby postoperative radiation therapy, if the margins are positive, andchemotherapy because of the high incidence of pulmonary metastaseswith an overall survival of approximately 50% at five years.
  265. 265. CLASSICCase #35022 year femalealveolar soft partsarcoma pelvis
  266. 266. Axial T-2 MRI
  267. 267. Wide resection specimen
  268. 268. Photomic showing alveolar pattern
  269. 269. Post op x-ray withinternal hemipelvectomyreconstructed withrebar and cement THA
  270. 270. Case #351 Oblique x-ray 45 year female with alveolar soft part sarcoma pelvis
  271. 271. CT Scan
  272. 272. Sagittal T-1 MRI tumor ischium
  273. 273. tumorAxial T-2 MRI
  274. 274. Photomic showing alveolar pattern
  275. 275. cementPost op x-ray showing rebar and cement reconstruction
  276. 276. Case #352 Axial T-2 MRI 26 year male with alveolar soft part sarcoma thigh
  277. 277. Arteriogramshowing tumor blush
  278. 278. Low power photomic showing alveolar pattern
  279. 279. High power
  280. 280. Case #353 Axial proton density MRI tumor 9 year female with alveolar soft part sarcoma thigh
  281. 281. tumorSagittal proton density MRI
  282. 282. Alveolar pattern on photomic
  283. 283. Synovial Sarcoma
  284. 284. Synovial Sarcoma The synovial sarcoma is the fourth most common soft tissuesarcoma seen in the human anatomy. It occurs typically in youngadults between the ages of 15 and 35 years, affecting males slightlymore than females. The name, synovial sarcoma, suggests anorigin in synovial tissue within a major joint. This is a mistakenconcept because only 10% of these tumors will be found inside amajor joint. They usually occur in juxta-articular structures, mostoften around the knee, and are frequently associated with tendonsheaths, bursal sacs, and fascial planes. They can also occur in deepmuscle bellies. These tumors are also commonly seen about thethigh, shoulder, arm, elbow and wrist area, and are also found aboutthe foot area. The synovial sarcoma is unusual in that it usuallystarts off with a very slow growth pattern that may suggest a benignprocess masquerading as an injury to the extremities. In many casesthere is dystrophic calcification or even heterotopic bone within thetumor, again suggesting a benign diagnosis that clinicians may
  285. 285. inject for symptomatic relief. Microscopically, this tumor has a characteristic biphasic patternwith a combination of epithelioid-looking cells that form in nests,clefts, or even in a tubular structure formation associated withmalignant-appearing spindle cells in the same area. There is amonophasic form of this sarcoma, usually a spindle cell form, thathas the appearance of a fibrosarcoma. There is also an epithelioidform of the synovial sarcoma that is quite unusual. Despite thebenign clinical appearance of the tumor, the prognosis for survivalis very poor, with only about 50% of patients surviving for fiveyears and only 25% surviving ten years. There is a high (20%)metastatic involvement of proximal lymph nodes that should belooked for and treated by aggressive resection and local radiationtherapy. In about 30% of cases, there will be heavy calcificationwithin the tumor that indicates a more benign prognosis with an80% survival rate at five years in this group. The treatment for this tumor consists of wide local resection,
  286. 286. if possible, or high level amputation for cases where local controlis difficult. Following a wide resection, local radiation therapy isemployed. If the prognosis seems extremely guarded, chemo-therapy is indicated.
  287. 287. CLASSIC Case #354 20 year female with synovial sarcoma shoulder
  288. 288. CT scan showing calcifying tumor
  289. 289. 3-D CT reconstruction
  290. 290. tumorCoronal T-1 MRI
  291. 291. tumorAxial T-1 MRI
  292. 292. tumorAxial proton density MRI
  293. 293. Surgical specimen
  294. 294. Photomic showing biphasic pattern
  295. 295. Case #354.1 Synovial sarcoma 34 year old male with tender lump in axilla for 6 months
  296. 296. Chest x-ray CT scan
  297. 297. T-2Cor T-1 Gad
  298. 298. Axial T-1 T-2 FS Gad
  299. 299. MRI Arteriogram
  300. 300. Resection specimen
  301. 301. Case #355 73 year female with synovial sarcoma proximal thigh
  302. 302. Close up x-ray
  303. 303. tumor Axial T-1 MRI
  304. 304. tumor Axial T-2 MRI
  305. 305. Photomic showing biphasic pattern
  306. 306. Case #355.1 Synovial sarcoma Axial T-1 T-2 67 year old female with injury to thigh 7 years ago
  307. 307. Sag T-1 Sag PD
  308. 308. Cor STIR
  309. 309. Case #355.2 Synovial sarcoma 55 year male with tender mass in anterior thigh 6 months
  310. 310. Coronal MRIT-1 T-2 Gad
  311. 311. Axial T-2 GadSag T-2
  312. 312. Calcific tendonitis gluteus maxCase #355.3Synovial sarcomapseudotumor41 year female withleft hip pain 3 months
  313. 313. CT scan
  314. 314. Axial T-1 T-2 Gad
  315. 315. Cor T-2 FS T-2 FS Gad
  316. 316. Sag T-2 Gad
  317. 317. Case #355.4 Calcific deltoid tendonitis 46 year male with severe pain left upper arm for 6 weeks
  318. 318. Axial T-1 T-2
  319. 319. Case #355.5 Pseudo-synovial sarcoma 61 year male with incidental finding in pelvis
  320. 320. Case #356 tumor25 year male with heavily calcified synovial sarcoma thigh
  321. 321. Macro section showing invasion of adjacent femur
  322. 322. epithelioidSpindle cells Photomic showing biphasic pattern
  323. 323. Case #356.1 Synovial sarcoma 11 year male with painless lump posterior medial knee 1 yr
  324. 324. Sag T-1 Cor T-2
  325. 325. Axial GadAxial T-2
  326. 326. Case #356.2 MRI Pseudotumor Cor T-1 Sag T-2 33 yr male with painless popliteal Baker’s cyst present for 6 months
  327. 327. Sagittal PD MRI Axial T-2 MRI
  328. 328. Case #356.3 Synovial sarcoma Axial MRIT-1 T-2 16 year female with Gad painful mass anterior to ankle for 6 months
  329. 329. Coronal T-1 T-2
  330. 330. Sagittal T-1 T-2
  331. 331. Case #357 28 year male with synovial sarcoma foot
  332. 332. tumorSagittal T-2 MRI
  333. 333. tumorAxial T-2 MRI
  334. 334. Photomic showing biphasic pattern
  335. 335. Case #35832 year female withossifying synovialsarcoma great toe
  336. 336. Case #358.1 28 yr male with fungating tumor in foot for 6 mos
  337. 337. AxialT-1 T-2 Gad
  338. 338. Sag T-1 T-2 FS
  339. 339. CorT-1 T-2 Gad
  340. 340. Case #358.2 Axial T-1 T-2 40 year female with painful forefoot for 1 year
  341. 341. Cor T-1 T-2 FS
  342. 342. Case #359 Sagittal T-1 MRI 36 year female with synovial sarcoma foot
  343. 343. Axial gad contrast MRI tumor
  344. 344. Case #36030 year female withsynovial sarcoma leg
  345. 345. tumorCT scan
  346. 346. Coronal T-1 MRI tumor
  347. 347. tumorAxial T-2 MRI
  348. 348. Case #361 Sagittal T-1 MRI tumor 74 year female with synovial sarcoma ankle
  349. 349. Axial STIR MRI tumor
  350. 350. Axial gad contrast tumor
  351. 351. Case #36222 year maleintra-articularsynovial sarcomaknee tumorSagittal T-1 MRI
  352. 352. tumorAxial gad contrast MRI
  353. 353. Sagittal gad contrast tumor
  354. 354. Resection specimen cut in path lab
  355. 355. Scanning lens photomic showing epithelioid lined clefts
  356. 356. Case #362.1 Synovial sarcoma 30 year male with 17 year history of slow growing lump lateral retinaculum right knee
  357. 357. Axial T-2
  358. 358. Cor T-1 T-2
  359. 359. Case #362.2 Tophaceous gout synovial sarcoma pseudotumor 51 year male with tender prepatellar lump for one year
  360. 360. Axial T-1 T-2 Gad
  361. 361. Sag T-1 T-2 Gad
  362. 362. Case #362.3 Axial T-2 Synovial sarcoma 40 year male with knee pain for 6 months
  363. 363. Cor T-1 T-2 PD FS
  364. 364. Sag T-2 FS PD FS
  365. 365. Case #36316 year female withossifying synovial sarcomaIn a suprapatellar lipoma
  366. 366. Synovial sarcomalipoma CT scan
  367. 367. tumorSagittal T-1 MRI lipoma
  368. 368. Scanning lens photomic showing lipoma to right and ossifying synovial sarcoma to left
  369. 369. Photomic of lipoma portion
  370. 370. osteoidPhotomic of ossifying monophasic synovial sarcoma
  371. 371. Photomic showing extensive ossification in sarcoma
  372. 372. One year after resectionand signs of recurrence
  373. 373. Case #1235 Coronal T-1 MRI 34 year female with synovial sarcoma thigh
  374. 374. Coronal T-2 MRI
  375. 375. Axial T-1 MRI
  376. 376. Axial T-2 MRI
  377. 377. Photomic showing a biphasic pattern
  378. 378. Case #123614 year female withsynovial sarcoma thighwith calcification
  379. 379. Axial T-1 MRI
  380. 380. Axial T-2 MRI
  381. 381. Case #1236.1 Synovial sarcoma 85 year male with soft tissue lump medial thigh 3 mos
  382. 382. AxialT-1 T-2 Gad
  383. 383. Cor T-2 Gad
  384. 384. Case #123735 year male withcalcifying synovialsarcoma thigh
  385. 385. Axial PD MRI
  386. 386. Axial T-2 MRI
  387. 387. Photomic showing a biphasic pattern
  388. 388. Case #1238 Coronal PD MRI28 year female with synovial sarcoma adductor compartment
  389. 389. Case #1239 Coronal PD MRI 18 year female with synovial sarcoma shoulder
  390. 390. Another coronal PD MRI cut thru tumor
  391. 391. Axial PD MRI
  392. 392. Photomic with biphasic pattern
  393. 393. Case #124145 year female withcalcifying synovialsarcoma thigh
  394. 394. Sagittal T-1 MRI
  395. 395. Axial PD MRI
  396. 396. Photomic showing monophasic spindle cell pattern
  397. 397. Case #1242 Coronal PD MRI 27 year male with synovial sarcoma elbow
  398. 398. Axial PD MRI
  399. 399. Case #1242.1 Synovial sarcoma 17 year old male with painful mass in elbow for 3 months
  400. 400. Sag T-1 T-2 Gad
  401. 401. Axial T-1 T-2Gad