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  • 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. Neurogenic Tumors
  • 3. Neurilemoma
  • 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. 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. CLASSIC Case #315 Coronal T-1 MRI gluteus max 55 year male with neurilemoma proximal sciatic nerve
  • 7. Axial T-2 MRI
  • 8. gluteus tumor nerveSagittal T-2 MRI of tumor in sciatic nerve
  • 9. tumor gluteus nerveSurgical exposure of tumor on sciatic nerve
  • 10. Photomic of highly cellular Antoni A area
  • 11. Verocay bodyClose up of Antoni A area
  • 12. Case #316 Axial T-1 MRI 22 year female with neurilemoma sciatic nerve
  • 13. tumorAxial T-2 MRI
  • 14. tumorCoronal T-2 MRI
  • 15. Coronal gad contrast MRI showing large cystic areas
  • 16. cystAxial gad contrast MRI
  • 17. Case #31769 year malecalcifying neurilemomaulnar nerve
  • 18. nerve tumorSagittal T-1 MRI showing tumor in ulnar nerve
  • 19. nerve tumor Sagittal T-2 MRI
  • 20. Axial T-2 MRI showing calcific signal voids
  • 21. Surgical specimen
  • 22. Photomic from Antoni A area
  • 23. Case #318 36 year male with neurilemoma S-1 root
  • 24. Lateral view
  • 25. L-5Sagittal T-1 MRI tumor
  • 26. Sagittal PD MRI
  • 27. S-1 root S-1 tumorCoronal T-2 MRI
  • 28. tumor S-1 foramen Coronal T-1 MRI
  • 29. Case #31924 year female S-2neurilemoma S-2 tumorSagittal PD MRI
  • 30. S-1Another sagittalPD MRI cut tumor
  • 31. tumor cystic area tumor Sagittal T-2 MRI
  • 32. cystic areaSagittal gad contrast MRI
  • 33. cyst S-2Axial gad contrast MRI
  • 34. Case #319.1 66 year male with painless presacral tumor mass for 7 years
  • 35. Axial T-1 T-2Gad
  • 36. LBP MRISag T-1 in 99 Sag T-1 in 06 Sag T-2 in 06
  • 37. Cor Gad C+
  • 38. Case #32045 year maledumbell neurilemomaC-3-4
  • 39. Myelographic studyshowing extraduraldefect
  • 40. Case #32128 year male withossifying ancientneurilemoma tibia
  • 41. Lateral view
  • 42. Case #321.1 86 year female with ancient schwannoma popliteal space
  • 43. Sag T-1 Sag PD Sag Gad
  • 44. Cor T-2 Cor Gad
  • 45. AxialT-1 T-2 Gad
  • 46. Case #1214 CT scan 37 year male with neurilemoma S-1
  • 47. L RBone scan shows signal void S-1 on left side
  • 48. Sagittal PD MRI
  • 49. Coronal PD MRI
  • 50. Case #121542 year male withneurilemoma L-5with lysis of L-5pedicle
  • 51. Lateral view
  • 52. CT scan
  • 53. Another CT scan
  • 54. Case #1216 Coronal T-1 MRI36 year female with neurilemoma brachial plexus C5-6 roots
  • 55. Coronal T-2 MRI
  • 56. Case #1217 Axial T-2 MRI 48 year male with neurilemoma radial nerve
  • 57. Sagittal T-2 MRI
  • 58. Surgical specimen cut in path lab
  • 59. Case #1217.1 Sag T-1 PD Gad 51 year female with 1 yr history of tingling in right thumb
  • 60. Axial T-1 PD Gad
  • 61. Radial N.Cor STIR Surgical excision
  • 62. Case #1217.2 Cor T-1 PD Gad C.46 year female with tender mass located along ulnar nerve
  • 63. Axial T-2Axial Gad
  • 64. Case #1217.3 Axial PD FS Gad Upper Lower 36 yr female with two separate lumps in one leg for 1 year
  • 65. Sag T-1 Gad Gad
  • 66. Surgical specimen
  • 67. Case #121836 year male withneurofibromatosis anda neurilemoma in thesciatic nerveAxial T-1 MRI
  • 68. Sagittal T-1 MRI
  • 69. Sagittal T-2 MRIshowing tumor arisingfrom sciatic nerve
  • 70. Axial T-2 MRI
  • 71. Case #1220.1 Axial PD MRI65 year male with tender lump low in popliteal space 1 yr
  • 72. posteriortibial art Coronal T-1 Coronal PD
  • 73. Post tibial nerveSagittal T-1 Sagittal PD
  • 74. Surgical exposure of the neurilemoma in the post. tib. N.
  • 75. Post tibial N. Surgical removal of the neurilemoma
  • 76. Case #1220.2 Sag T-1 T-2 Neurilemoma 63 year female with tingling on bottom of foot for 6 months
  • 77. Axial T-1 T-2
  • 78. Cor T-1 T-2
  • 79. Surgical excision
  • 80. Case #1220.3 Sag T-142 year male withtender lump bottomof foot for 1 year Gad
  • 81. Axial T-1 T-2 Gad
  • 82. SolitaryNeurofibroma
  • 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. 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. CLASSIC Case #322 Sagittal PD MRI 32 year female with neurofibroma foot
  • 86. Axial STIR MRI
  • 87. Surgical exposure for excision
  • 88. nerveSurgical specimen cut in path lab
  • 89. Photomic showing palisading of schannoma cells
  • 90. Case #323 Sagittal T-1 MRI tumor 3 month old infant with neurofibroma foot
  • 91. Sagittal PD MRI
  • 92. Axial T-2 MRI
  • 93. Another axial T-2 cut
  • 94. Case #324 Coronal PD MRI 53 year male with neurofibroma thigh
  • 95. Coronal T-2 MRI
  • 96. Axial T-1 MRI
  • 97. Axial T-2 MRI
  • 98. nerveResection specimen cut in path lab
  • 99. Photomic
  • 100. Case #1220 Axial T-1 MRI56 year female with neurofibroma on sciatic N at notch level
  • 101. Axial T-2 MRI
  • 102. Axial Gad contrast MRI
  • 103. Veracoy bodyPhotomic in area of Antoni-A tissue
  • 104. Case #1220.1 tumor53 year female with slow growing pelvic neurofibroma 10 yrs
  • 105. Axial T-1 Axial T-2 tumorGad Sciatic N
  • 106. tumorAxial T-2 showing origin from both S-1 roots
  • 107. tumorCoronal Gad
  • 108. Sag T-2
  • 109. Case #325 Plastic cut away model 32 year female with Morton’s neuroma
  • 110. Photomic of traumatic neuroma
  • 111. Case 326 Plastic model cut away 42 year male with amputation neuromata forearm
  • 112. Photomic amputation neuroma
  • 113. Neurofibromatosis
  • 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. 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. CLASSICCase #327 fibroma molluscum35 year male type 1neurofibromatosis withlarge plexiformneurofibroma andelephantiasis left arm
  • 117. Case #32811 year maleneurofibromatosis 1café-au-lait spots &scoliosis
  • 118. Case #329 Axial T-1 MRI 5 year female with plexiform neurofibromata pelvis
  • 119. tumorgluteusOblique coronal T-1 MRI
  • 120. tumor Axial T-2 MRI
  • 121. Case #330 Sagittal T-1 MRI 35 year male with neurofibromatosis S-2 & 3
  • 122. Sagittal T-2 MRI tumor
  • 123. tumorAxial T-1 MRI
  • 124. tumorAxial T-2 MRI
  • 125. Case #331 Sagittal T-1 MRI tumor 19 year female with neurofibromatosis and neurilemoma sciatic nerve
  • 126. Axial T-1 MRI tumor
  • 127. tumorAxial T-2 MRI
  • 128. Case #332 Sagittal T-2 MRI15 year female with plexiform neurofibromatosis LS spine
  • 129. tumorAxial proton density MRI pelvis
  • 130. tumorSagittal T-2 MRI
  • 131. Case #333 Sagittal T-1 MRI tumor 45 year male with plexiform neurofibromatosis knee
  • 132. tumorSagittal T-2 MRI
  • 133. Case #3344 year male withneurofibromatosis andelephantiasis left armcafé-au-lait spots
  • 134. X-ray with frail bone
  • 135. Case #33532 year female with neurofibromatosis hands & gigantism
  • 136. Case #33630 year male withneurofibromatosis withgigantism 2nd & 3rd toes
  • 137. Case #33716 year male withneurofibromatosiscervical spine
  • 138. Severe dysplastic changesin cervical vertebrae
  • 139. Myelographic studyshowing multipledumbell neurofibromata
  • 140. Sagittal laminogramshowing hypoplasticvertebrae
  • 141. Case #338Child with tibialpseuoarthrosis 2ndto neurofibromatosis
  • 142. Case #1221 CT scan 55 year female with neurofibromatosis and large neurofibroma in sciatic nerve at notch level
  • 143. Sagittal T-1 MRIshowing large plexiformneurofibroma in buttockarea
  • 144. Axial T-1 MRI showing plexiform neurofibroma
  • 145. Axial T-1 MRI at higher level
  • 146. Case #1222 45 year female with neurofibromatosis and plexiform neurofibroma about heel and ankle area
  • 147. Axial T-1 MRIshowing tumor betweentalus and heel cord
  • 148. Coronal T-1 MRIshowing tumor behindankle joint
  • 149. Coronal T-2 MRI
  • 150. Sagittal T-2 MRI
  • 151. Case #1223 Sagittal T-2 MRI 8 year male with neurofibromatosis and plexiform neurofibroma of forefoot
  • 152. Axial T-1 MRI
  • 153. Axial T-2 MRI
  • 154. Coronal T-2 MRI
  • 155. Case #1223.126 year male with type Ineurofibromatosis andlarge plexiform neuro-fibroma in foot withbony deformation
  • 156. Sagittal T-1 T-2
  • 157. Coronal T-1 T-2
  • 158. Axial T-1 T-2 Gad
  • 159. Case #1224 27 year male with neurofibromatosis and secondary talar gigantism
  • 160. Gigantism of 3rd toe
  • 161. Plexiform neurofibroma deep post compartment leg
  • 162. Plexiform neurfibromadeep in arch of foot
  • 163. Axial T-2 MRIplexiform neuro-fibroma in forefootarea
  • 164. Sagittal T-2 MRIplexiform neurofibromaforefoot area
  • 165. Resected specimen from posterior leg compartment
  • 166. Case #1225 18 year male with neurofibromatosis and plexiform neurofibroma calf area
  • 167. popliteal vesselsSurgical exposure of tumor in calf
  • 168. Plexiform neurofibromata removed from calf area
  • 169. Surgical incision
  • 170. Case #122655 year male withneurofibromatosisand associateddeformity rib cage
  • 171. Case #122717 year female withneurofibromatosis andsevere spinal kyphosiswith paraplegia
  • 172. Case #12289 year female withneurofibromatosis withassociated pseudarthrosistibia
  • 173. Case #122919 year female withneurofibromatosis andassociated deformationforearm bones andradial head dislocation
  • 174. MalignantSchwannoma
  • 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. 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. CLASSICCase #339 tumor28 year femalemalignant schwannomasciatic nerve nerveSagittal T-2 MRI
  • 178. tumorAxial PD MRI
  • 179. spinal prep siteSubQ plexiform neurofibroma with café-au-lait spot over pelvis
  • 180. peroneal N sciatic N tumorpost tib N Tumor exposed ready for resection
  • 181. benignmalignant Scanning lens photomic
  • 182. Low power malignant photomic
  • 183. Higher power
  • 184. Case #340 tumor tumor 16 year female with malignant schwannoma pelvis
  • 185. necrosis notch Axial CT scan showing large necrotic tumorfilling the pelvis and extruding thru the sciatic notch
  • 186. Large gluteal mass with café-au-lait spots prior to resection
  • 187. Tumor exposed beneath gluteus maximus
  • 188. Large excised tumor on back table
  • 189. Low power photomic
  • 190. Higher power photomic
  • 191. Case #341 46 year male with skin lesions of neurofibromatosis
  • 192. Close up of fibroma molluscum skin lesions
  • 193. CT scan tumorLarge malignant schwannoma in adductor compartment
  • 194. tumorExposed tumor at time of surgery
  • 195. Excised tumor cut open on back table
  • 196. Low power photomic
  • 197. High power photomic
  • 198. Case #342 25 year male with neurofibromatosis and café-au-lait spot over buttock and malignant schwannoma sciatic N
  • 199. Surgical exposure of sciatic nerve tumor
  • 200. malignant benignExcised cut specimen showing benign and malignant parts
  • 201. Photomic of malignant portion
  • 202. Case #343 Autopsy specimenAutopsy specimen of 42 year female with neurofibromatosisand malignant schwannoma in area of severe dorsal kyphosis
  • 203. Sagittal cut autopsy specimen
  • 204. Photomic of tumor
  • 205. Case #344 Sagittal T-2 MRI 26 year female with malignant schwannoma peroneal N
  • 206. Axial T-2 MRI
  • 207. Photomic
  • 208. Case #123047 year male withmalignant schwannomalegCoronal T-1 MRI
  • 209. Axial T-2 MRI
  • 210. Surgical specimen
  • 211. Photomic
  • 212. Higher power
  • 213. Case #1231 CT scan20 year male with malignant schwannoma paraspinous area
  • 214. Axial T-2 MRI
  • 215. Another axial T-2 MRI at different level
  • 216. Case #1232 17 year male with malignant schwannoma thigh
  • 217. CT scan
  • 218. Another CT scan at different level
  • 219. Case #123336 year female withbenign meningiomaarising within theradial nerveSagittal T-1 MRI nerve
  • 220. nerveSagittal T-2 MRI
  • 221. Axial PD MRI
  • 222. Case #1234 49 year female with myxopapillary ependymoma sacrum
  • 223. CT scan
  • 224. CT scan at lower S-2 level
  • 225. Sagittal T-1 MRI
  • 226. Sagittal T-2 MRI
  • 227. Axial T-2 MRI
  • 228. Granular cell Tumor
  • 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. 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. Cor T-1 Gad
  • 232. Sag T-1 T-2 FS Gad
  • 233. Rhabdomyosarcoma
  • 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. 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. 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. CLASSIC Case #345 Axial T-1 MRI 9 month male with embryonal rhabdomyosarcoma pelvis
  • 238. Axial T-2 MRI
  • 239. Coronal gad contrast MRI showing central necrosis
  • 240. Another coronal gad contrast cut with necrosis
  • 241. Photomic showing round cells
  • 242. Higher power
  • 243. Case #3467 year maleembryonalrhabdomysarcomamaxilla
  • 244. AP x-ray
  • 245. Low power photomic
  • 246. High power
  • 247. Case #3477 year female with fungating alveolar rhabdomysarcoma leg
  • 248. Surgical specimen cut in path lab
  • 249. Photomic
  • 250. Case #34845 year male with pleomorphic rhabomyosarcoma biceps
  • 251. Close up of tumor
  • 252. Lateral x-ray of armshowing tumor mass
  • 253. Arteriogram showingtumor blush
  • 254. Surgical exposure during wide resection
  • 255. Tumor cut in path lab
  • 256. Photomic showing strap cells
  • 257. Case #34942 year female withpleomorphicrhabdomysarcomahypothenar hand
  • 258. Ulnar amputationcut in path lab
  • 259. biopsyClose up photo tumor
  • 260. Photomic showing large pleomorphic giant forms
  • 261. Remaining functional radial hand
  • 262. Great cosmetic appeal
  • 263. Alveolar Soft Part Sarcoma
  • 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. CLASSICCase #35022 year femalealveolar soft partsarcoma pelvis
  • 266. Axial T-2 MRI
  • 267. Wide resection specimen
  • 268. Photomic showing alveolar pattern
  • 269. Post op x-ray withinternal hemipelvectomyreconstructed withrebar and cement THA
  • 270. Case #351 Oblique x-ray 45 year female with alveolar soft part sarcoma pelvis
  • 271. CT Scan
  • 272. Sagittal T-1 MRI tumor ischium
  • 273. tumorAxial T-2 MRI
  • 274. Photomic showing alveolar pattern
  • 275. cementPost op x-ray showing rebar and cement reconstruction
  • 276. Case #352 Axial T-2 MRI 26 year male with alveolar soft part sarcoma thigh
  • 277. Arteriogramshowing tumor blush
  • 278. Low power photomic showing alveolar pattern
  • 279. High power
  • 280. Case #353 Axial proton density MRI tumor 9 year female with alveolar soft part sarcoma thigh
  • 281. tumorSagittal proton density MRI
  • 282. Alveolar pattern on photomic
  • 283. Synovial Sarcoma
  • 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. 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. 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. CLASSIC Case #354 20 year female with synovial sarcoma shoulder
  • 288. CT scan showing calcifying tumor
  • 289. 3-D CT reconstruction
  • 290. tumorCoronal T-1 MRI
  • 291. tumorAxial T-1 MRI
  • 292. tumorAxial proton density MRI
  • 293. Surgical specimen
  • 294. Photomic showing biphasic pattern
  • 295. Case #354.1 Synovial sarcoma 34 year old male with tender lump in axilla for 6 months
  • 296. Chest x-ray CT scan
  • 297. T-2Cor T-1 Gad
  • 298. Axial T-1 T-2 FS Gad
  • 299. MRI Arteriogram
  • 300. Resection specimen
  • 301. Case #355 73 year female with synovial sarcoma proximal thigh
  • 302. Close up x-ray
  • 303. tumor Axial T-1 MRI
  • 304. tumor Axial T-2 MRI
  • 305. Photomic showing biphasic pattern
  • 306. Case #355.1 Synovial sarcoma Axial T-1 T-2 67 year old female with injury to thigh 7 years ago
  • 307. Sag T-1 Sag PD
  • 308. Cor STIR
  • 309. Case #355.2 Synovial sarcoma 55 year male with tender mass in anterior thigh 6 months
  • 310. Coronal MRIT-1 T-2 Gad
  • 311. Axial T-2 GadSag T-2
  • 312. Calcific tendonitis gluteus maxCase #355.3Synovial sarcomapseudotumor41 year female withleft hip pain 3 months
  • 313. CT scan
  • 314. Axial T-1 T-2 Gad
  • 315. Cor T-2 FS T-2 FS Gad
  • 316. Sag T-2 Gad
  • 317. Case #355.4 Calcific deltoid tendonitis 46 year male with severe pain left upper arm for 6 weeks
  • 318. Axial T-1 T-2
  • 319. Case #355.5 Pseudo-synovial sarcoma 61 year male with incidental finding in pelvis
  • 320. Case #356 tumor25 year male with heavily calcified synovial sarcoma thigh
  • 321. Macro section showing invasion of adjacent femur
  • 322. epithelioidSpindle cells Photomic showing biphasic pattern
  • 323. Case #356.1 Synovial sarcoma 11 year male with painless lump posterior medial knee 1 yr
  • 324. Sag T-1 Cor T-2
  • 325. Axial GadAxial T-2
  • 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. Sagittal PD MRI Axial T-2 MRI
  • 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. Coronal T-1 T-2
  • 330. Sagittal T-1 T-2
  • 331. Case #357 28 year male with synovial sarcoma foot
  • 332. tumorSagittal T-2 MRI
  • 333. tumorAxial T-2 MRI
  • 334. Photomic showing biphasic pattern
  • 335. Case #35832 year female withossifying synovialsarcoma great toe
  • 336. Case #358.1 28 yr male with fungating tumor in foot for 6 mos
  • 337. AxialT-1 T-2 Gad
  • 338. Sag T-1 T-2 FS
  • 339. CorT-1 T-2 Gad
  • 340. Case #358.2 Axial T-1 T-2 40 year female with painful forefoot for 1 year
  • 341. Cor T-1 T-2 FS
  • 342. Case #359 Sagittal T-1 MRI 36 year female with synovial sarcoma foot
  • 343. Axial gad contrast MRI tumor
  • 344. Case #36030 year female withsynovial sarcoma leg
  • 345. tumorCT scan
  • 346. Coronal T-1 MRI tumor
  • 347. tumorAxial T-2 MRI
  • 348. Case #361 Sagittal T-1 MRI tumor 74 year female with synovial sarcoma ankle
  • 349. Axial STIR MRI tumor
  • 350. Axial gad contrast tumor
  • 351. Case #36222 year maleintra-articularsynovial sarcomaknee tumorSagittal T-1 MRI
  • 352. tumorAxial gad contrast MRI
  • 353. Sagittal gad contrast tumor
  • 354. Resection specimen cut in path lab
  • 355. Scanning lens photomic showing epithelioid lined clefts
  • 356. Case #362.1 Synovial sarcoma 30 year male with 17 year history of slow growing lump lateral retinaculum right knee
  • 357. Axial T-2
  • 358. Cor T-1 T-2
  • 359. Case #362.2 Tophaceous gout synovial sarcoma pseudotumor 51 year male with tender prepatellar lump for one year
  • 360. Axial T-1 T-2 Gad
  • 361. Sag T-1 T-2 Gad
  • 362. Case #362.3 Axial T-2 Synovial sarcoma 40 year male with knee pain for 6 months
  • 363. Cor T-1 T-2 PD FS
  • 364. Sag T-2 FS PD FS
  • 365. Case #36316 year female withossifying synovial sarcomaIn a suprapatellar lipoma
  • 366. Synovial sarcomalipoma CT scan
  • 367. tumorSagittal T-1 MRI lipoma
  • 368. Scanning lens photomic showing lipoma to right and ossifying synovial sarcoma to left
  • 369. Photomic of lipoma portion
  • 370. osteoidPhotomic of ossifying monophasic synovial sarcoma
  • 371. Photomic showing extensive ossification in sarcoma
  • 372. One year after resectionand signs of recurrence
  • 373. Case #1235 Coronal T-1 MRI 34 year female with synovial sarcoma thigh
  • 374. Coronal T-2 MRI
  • 375. Axial T-1 MRI
  • 376. Axial T-2 MRI
  • 377. Photomic showing a biphasic pattern
  • 378. Case #123614 year female withsynovial sarcoma thighwith calcification
  • 379. Axial T-1 MRI
  • 380. Axial T-2 MRI
  • 381. Case #1236.1 Synovial sarcoma 85 year male with soft tissue lump medial thigh 3 mos
  • 382. AxialT-1 T-2 Gad
  • 383. Cor T-2 Gad
  • 384. Case #123735 year male withcalcifying synovialsarcoma thigh
  • 385. Axial PD MRI
  • 386. Axial T-2 MRI
  • 387. Photomic showing a biphasic pattern
  • 388. Case #1238 Coronal PD MRI28 year female with synovial sarcoma adductor compartment
  • 389. Case #1239 Coronal PD MRI 18 year female with synovial sarcoma shoulder
  • 390. Another coronal PD MRI cut thru tumor
  • 391. Axial PD MRI
  • 392. Photomic with biphasic pattern
  • 393. Case #124145 year female withcalcifying synovialsarcoma thigh
  • 394. Sagittal T-1 MRI
  • 395. Axial PD MRI
  • 396. Photomic showing monophasic spindle cell pattern
  • 397. Case #1242 Coronal PD MRI 27 year male with synovial sarcoma elbow
  • 398. Axial PD MRI
  • 399. Case #1242.1 Synovial sarcoma 17 year old male with painful mass in elbow for 3 months
  • 400. Sag T-1 T-2 Gad
  • 401. Axial T-1 T-2Gad