This document summarizes different types of vascular tumors of bone including hemangioma, cystic angiomatosis, hemangiomatosis, lymphangiomatosis, Gorham's disease, hemangioendothelioma, and high grade angiosarcoma. Specific cases are presented with clinical information, imaging features, histology, and treatment details. Key characteristics of each tumor type are highlighted such as common locations, age distribution, radiographic appearance, histologic findings, and prognosis.

1. Volume 7
Vascular Tumors of Bone
Hemangioma---------------------Case 146-151 & 801-832
Cystic angiomatosis-------------Case 152
Hemangiomatosis----------------Case 833-834
Lymphangiomatosis-------------Case 835-838
Gorham’s disease----------------Case 153 & 839-840
Hemangioendothelioma---------Case 154-158 & 841-847
High grade angiosarcoma-------Case 159 & 848-851
Hemangiopericytoma------------Case 160-162 & 852-855
Lipid Tumors of Bone
Parosteal lipoma------------------Case 163-165 & 859-60
Intramedullary lipoma-----------Case 166-168 & 856-860
Intracortical lipoma--------------Case 860.3

2. Vascular Tumors
of Bone

3. Hemangioma of
Bone

4. Hemangioma of Bone
Hemangioma of the skeletal system is rare and accounts for only
about 1% of all skeletal tumors. It is slightly more common in
females with the most common location being the skull, and spinal
column, and the least common location being the appendicular
skeleton where it is seen typically in long bones such as the
humerus, femur and tibia. The presents of asymptomatic incidental
hemangiomas of the vertebral bodies at autopsy have been noted
in as high as 10% of autopsy studies. The hemangioma is considered
A hamartomatous dysplastic process similar to fibrous dysplasia.
It can be monstotic or polyostotic. The lesions occur during the
developmental years but are frequently not diagnosed until middle
age when they are picked up as incidental findings, perhaps during
the performance of an MRI study of the axial skeleton.
Radiographically, the hemangioma is a lytic process with fairly
geographic borders and the lesions tend to have a motheaten or

5. honeycomb appearance in both the axial and long bones due to
reactive bone formation surrounding the vascular spaces. In the
case of the axial skeleton, the characteristic radiographic feature is
a vertically oriented honeycomb pattern. In flat bones, such as the
iliac crest or the calvarium, the lesion may have a soap-bubbly
or sunburst appearance, especially in the calvarium where these
lesions are typically seen. Because of the osteoblastic response to
the vascular dysplasia, the radiographic appearance can be similar
to that of an osteoid osteoma or an osteoblastoma. With MRI
imaging, one may find a soft tissue component with the bony lesion,
especially in the appendicular skeleton. Histologically, these lesions
tend to be grossly bloody in appearance with large vascular sinusoids
lined by a single layer of flat-appearing endothelial cells filled
with blood. Occasionally, one will see an epithelioid pattern to
the endothelial cells that gives them a more cube-like appearance
similar to the histological appearance of an epithelioid low grade
hemangioendothelioma.

6. As far as treatment is concerned, many of these smaller lesions
are incidental findings that require no treatment whatsoever. How-
ever, with larger lesions, especially in the vertebral column, there
may be mechanical collapse associated with spinal cord compression
that might necessitate curettement of the lesion with bone grafting
and instrumentation. Also in the spinal area,larger lesions may
require embolization therapy prior to surgery to reduce hemorrhage
at the time of exploration. Occasionally, low dose radiation therapy
can reduce the lytic process produced by these dysplastic lesions.

7. CLASSIC
Case #146
14 year male
hemangioma tibia

8. Photomic

9. Case #147
45 year female with hemangioma ilium

10. Case #148
47 year male
hemangioma tibia

11. Bone scan

12. Axial T-2 MRI

13. Photomic

14. Higher power

15. Case #149
49 year male
hemangioma L-4
vertebra

16. Lateral view

17. Coronal PD MRI

18. Sagittal PD MRI

19. Axial PD MRI

20. Bone scan

21. Case #150
Coronal CT scan
T-11
30 year female with hemangioma thoracic spine

22. Axial CT scan

23. Case #151
25 year female with hemangioma skull

24. Case #801
13 year female
hemangioma femur

25. Proximal end

26. Coronal T-1 MRI

27. Sagittal T-1 MRI

28. Axial proton density MRI

29. Photomic

30. 2 months post op
pins, cement and
cancellous allograft

31. allograft
Lateral view
cement

32. Case #802
27 year male
parosteal hemangioma
femur

33. AP view

34. Bone scan

35. Sagittal T-2 MRI

36. Axial T-2 MRI

37. Case #803
Parosteal sclerosing
hemangioma femur
28 year female

38. Hemicortical resection
specimen

39. Masson stain photomic

40. Placement
IM nail

41. Cement augmentation

42. Post op x-ray
radiolucent
cement

43. 2.5 years later

44. Case #804
Parosteal
hemangioma femur
26 year male

45. Bone scan

46. Axial T-2 MRI

47. Case #805
14 year male
hemangioma femur

48. Another view

49. Case #806
31 year female
sclerosing parosteal
hemangioma femur

50. Case #807
4 year female
hemangioma humerus

51. Case #808
35 year male
path fracture thru
hemangioma femur

52. Skin overlying femur fracture

53. Close up cavernous hemangioma

54. Healed fracture
at later date

55. Case #809
26 year female
hemangioma tibia

56. AP view

57. Case #810
21 year female
hemangioma tibia

58. Case #811
26 year male
hemangioma tibia

59. Axial T-2 MRI

60. Case #812
19 year female with hemangioma scapula

61. Axial T-2 MRI

62. Case #813
21 year female with hemangioma scapula

63. Case #814
31 yr female with sclerosing hemagioma mid clavicle

64. Case #815
27 year female
hemangioma humerus

65. Case #816
30 year male with hemangioma radius & ulna

66. Lateral view

67. Case #817
41 year male with hemangioma distal radius

68. Case #818
bone
phlebolith
soft tissue
24 year male with bone & soft tissue hemangioma hand

69. Case #819
14 year female
hemangioma foot

70. Case #820
17 year male with hemangioma C-2 spine

71. Axial CT scan

72. Another axial cut

73. Axial T-1 MRI

74. Sagittal T-2 MRI

75. Case #821
47 year female with hemangioma C-4

76. Axial CT scan

77. Sagittal T-1 MRI

78. Sagittal T-2 MRI

79. Case #822
Sagittal T-1 MRI
18 year male with hemangioma lumbo-dorsal spine

80. Sagittal T-2 MRI

81. Axial T-1 MRI

82. Case #823
32 year male
hemangioma T-11

83. CT scan

84. sagittal T-1 MRI

85. Sagittal T-2 MRI

86. Case #824
60 year female with
hemangioma lumbo-
dorsal spine

87. Case #825
15 year female with hemangioma lumbar spine

88. Case #826
24 year female
hemangioma T-12

89. Case #827
25 year female
hemangioma skull

90. Lateral view

91. CT scan

92. Photomic

93. Case #828
23 year male
hemangioma skull

94. Case #829
19 year male
hemangioma
frontal bone

95. Case #830
31 year female with hemangioma skull

96. Case #831
36 year male with hemangioma superior pubic ramus

97. Bone detail

98. CT scan showing parosteal soft tissue component

99. Coronal T-2 MRI showing parosteal lesion

100. Axial gad contrast MRI

101. Photomic

102. Case #832
21 year male with hemangioma pubic bone

103. Cystic
Angiomatosis

104. CLASSIC
Case #152
37 year female
cystic angiomatosis
starting 1st in prox humerus

105. Coronal proton
density MRI

106. Coronal T-2 MRI

107. CT scan

108. Photomic

109. Higher power

110. Post op ORIF with
pins and cement

111. 5 years later with
osteoblastic spontaneous
healing response

112. Same healing response
in dorsal spine multi-
focal disease over 5 yrs

113. Lateral view of spine
at 5 yrs

114. CT scan chest at 6 years

115. CT scan with blastic response in dorsal spine

116. Another CT cut

117. Bone scan at 5 yrs

118. Blastic response in pelvis at 5 years

119. 5 year response
in opposite right humerus

120. 8 years later with IM nail in
femur for stress pain

121. Hemangiomatosis

122. CLASSIC
Case #833
5 year female
multi-focal bony
hemangiomatosis

123. Skull changes

124. Rib changes

125. Photomic

126. Higher power

127. Case #834
5.5 year male
hemangiomatosis
femur

128. Distal femoral lesion

129. Proximal femoral
lesions

130. Coronal proton
density MRI

131. Photomic

132. Lymphangiomatosis
of Bone

133. CLASSIC Case #835
15 year female with lymphangiomatosis of pelvis

134. Coronal T-2 MRI with bone & soft tissue lesions

135. Coronal gad contrast MRI

136. Axial T-2 MRI

137. Coronal T-2 MRI
cystic changes in thigh

138. Axial proton density MRI

139. Photomic

140. Case #836
24 year female with lymphangiomatosis pelvis

141. Lymphangiogram showing
large soft tissue disease

142. Case #837
12 year female with lymphangiomatosis pelvis

143. Case #838
16 year female
IM nail
lymphangiomatosis
femur with pre and
post op x-rays of
IM nailing procedure
pre op
bowing

144. Gorham’s disease

145. Gorham’s Disease
Gorham’s disease, sometimes referred to as disappearing bone
disease, is characterized by massive osteolysis in children or young
adults and is usually associated with the presence of benign
cavernous hemangiomas or lymphangiomas of bone. This strange
condition usually affects a particular area (such as the spine or the
hip) but can involve multiple bones of that area and tends to resolve
spontaneously.

146. CLASSIC
Case #153
36 year male
Gorham’s disease
seen initially left hip
Spontaneous osteolysis
femoral head and neck

147. CT scan 3 years later shows massive osteolysis both hips

148. Coronal T-1 MRI at time of initial disease in left hip

149. Initial axial T-2 MRI with high signal changes left hip

150. Bone biopsy left hip
osteoid
Photomic shows changes similar to hemangiomatosis

151. Higher power showing capillary vascular pattern

152. Changes in right hip
3 years after onset
in left hip

153. Case #839
12 year male with Gorham’s disease skull and spine

154. AP view

155. Sagittal T-1 MRI showing skull defects

156. Another sagittal T-1 cut

157. Axial CT scan showing defects at C-1 level

158. bone
Bone biopsy photomic changes similar to lymphangiomatosis

159. Case #840
46 year female with Gorham’s disease L hip & pelvis

160. Hemangioendothelioma

161. Hemangioendothelioma
The hemangioendothelioma or epithelioid hemangioendothelioma
is considered an intermediate grade vascular sarcoma arising from
endothelial cells. It occurs more commonly in males than females
and is found typically in the femur, tibia ,axial skeleton and ribs.
The most common age group is 20 thru 50 years. The lower grade
lesions behave clinically very much like a hemangioma of bone
but the higher grade hemangioendotheliomas are more aggressive,
require more aggressive treatment surgically, and can be helped
with adjuvant radiation therapy.

162. CLASSIC
Case #154
35 year female
low grade
hemangioendothelioma
distal femur

163. AP x-ray following
open biopsy

164. Photomic

165. Case #155
11 year male
hemangioendothelioma
distal tibia and talus

166. Curettement and
bone grafting distal
tibia

167. Photomic showing epitheliod cells

168. Case #156
35 year male with hemangioendothelioma rib

169. Photomic

170. Case #157
T-7
46 year female with hemangioendothelioma T-7

171. CT scan T-7 level

172. Sagittal T-2 MRI showing evidence of cord compression

173. Photomic

174. Post op x-ray following
posterior decompression
and spinal instrumentation

175. Case #158
69 year male high grade
hemangioendothelioma
distal tibia and fibula

176. Lateral view

177. bone
Low power photomic

178. Another photomic

179. Case #841
35 year female with hemangioendothelioma distal femur

180. Bone scan

181. Coronal T-1 MRI

182. Sagittal T-1 MRI

183. Axial T-2 MRI

184. Photomic

185. Case #842
19 year male with hemangioendothelioma distal femur

186. Coronal T-1 MRI

187. Case #843
40 year male
hemangioendothelioma
with path fracture
mid shaft humerus

188. Photomic

189. 6 years later and
conversion to OGS

190. Different view at 6 yrs

191. Case #844
48 year female with hemangioendothelioma foot

192. Bone scan

193. Axial T-1 MRI

194. Sagittal T-1 MRI

195. Sagittal STIR MRI

196. Case #845
19 year male with hemangioendothelioma 4th metatarsal

197. Sagittal T-2 MRI

198. Case #846
52 year male
hemangioendothelioma
hand

199. Bone scan

200. Case #847
T-4
40 year female
hemangioendothelioma T-5
T4 and 5

201. T-4
Lateral view
T-5

202. Myelogram 4 mos later
showing cord compression
At T-5 level

203. Rib lesion same patient

204. Photomic

205. Immediate post op
lateral x-ray with
anterior rib graft

206. Solid fusion 4 yrs later
no recurrence

207. 5 years post op
lateral view showing
anterior rib graft

208. High Grade
Angiosarcoma of
Bone

209. High Grade angiosarcoma of Bone
High grade hemangiosarcoma of bone differs from the low grade
hemangioendotheliomas of bone in that they are very aggressive,
lytic, destructive tumors usually occurring in the lower extremities
of young adults that carry an extremely poor prognosis because
of the high incidence of pulmonary metastases. These lesions, as
opposed to the hemangioendotheliomas, have very little osteoblastic
response to the infiltrate and take on the radiographic appearance of
a high grade spindle cell sarcoma such as a malignant fibrous histio-
cytoma or a fibrosarcoma of bone. As with the hemangioendo-
thelioma, they can be multifocal in nature but the more aggressive
lesions tend to be solitary and lytic with permeative lysis throughout
the bone. There are only about 30 cases described in the world
literature showing the extreme rarity of this lesion. This aggressive
sarcoma requires aggressive surgical treatment along with radiation
and chemotherapy with a 50% chance of a five year survival.

210. CLASSIC Case #159
21 year male with angiosarcoma pelvis

211. Progressive disease at a later date

212. CT scan above sciatic notch level

213. CT scan just below the sciatic notch

214. CT scan at femoral head level

215. tumor
Coronal T-1 MRI

216. tumor
Axial T-1 MRI

217. Photomic

218. Higher power

219. Case #848
50 year male with angiosarcoma pelvis

220. CT scan

221. Bone scan

222. Coronal T-1 MRI

223. Axial T-2 MRI

224. Photomic

225. Immediate post op internal hemipelvectomy with THA

226. Case #849
34 year male
angiosarcoma fibula

227. tumor
Coronal proton density MRI

228. tumor
Axial proton density MRI

229. Photomic

230. Case #849.1 Bone Angiosarc
Cor T-1 T-2 Gad
46 year male smoker with fibular head mass for 3 months

231. Sag T-1 T-2 Gad

232. Axial T-1 T-2
Gad

233. Case #850
66 year male
angiosarcoma
tibia and fibula

234. Oblique view

235. Femur involved also

236. Photomic

237. Case #851
60 year male
angiosarcoma femur

238. 9 mos. Following
segmental resection
autoclaving and
replacement over IM
nail

239. Multifocal radial lesion at a later date

240. Low power photomic

241. Hemangiopericytom
a of Bone

242. Hemangiopericytoma of Bone
Hemagiopericytoma of bone is an extremely rare tumor arising
from the hemangiopericytes of Zimmerman, which are smooth
muscle contractile cells that lie outside the capillary tubes of the
vascular system peripherally and control the flow of blood to
peripheral tissue. Hemangiopericytomas can range from very low
grade tumors, such as the glomus tumor seen in the distal
phalanges of young adults, to the more aggressive, malignant
hemangiopericytoma seen in the more proximal parts of the body,
such as the pelvis, spine or femur. The later can behave like a
sarcoma and metastasize to the lung. There have only been a few
cases of this tumor reported in the literature in large bones.

243. CLASSIC Case #160
25 year female with benign hemangiopericytoma talus

244. AP view

245. Mortice view

246. Low power photomic

247. High power

248. Post op x-ray after
curettement and
cementation

249. 22 years later with minimal pain with degenerative OA and
beaking at the talo-narvicular joint

250. Case #161
25 year female
hemangiopericytoma
(glomus tumor) distal
phalanx index finger

251. Case #162
55 year male
malignant
hemangiopericytoma
pelvis

252. Higher power

253. Post op x-ray following wide surgical resection

254. Case #852
47 year female with metastatic hemangiopericytoma
Proximal humerus

255. Bone scan

256. Coronal T-1 MRI

257. Photomic

258. Case # 853
67 year female
malignant
hemangiopericytoma
humerus with path
fracture

259. Another view

260. Photomic

261. Case #853.1
49 yr male with malignant hemangiopericytoma mid femur

262. Case #854
39 year female with benign hemangiopericytoma rib
3 years apart

263. Case #855
29 year female
malignant
hemangiopericytoma
thoracic spine

264. Laminogram cut

265. CT scan

266. Lipid Bone
Tumors

267. Parosteal Lipoma

268. Parosteal Lipoma
Lipid tumors of bone, unlike soft tissue fatty tumors, are extremely
rare clinical entities. The most common of these rare tumors is the
parosteal lipoma that is usually found lying on the surface of the
metaphyseal portion of a long bone such as the humerus, femur or
tibia. They usually occur in middle-aged patients with no sex
dominance. Radiographically these lesions stand out because of
an exophytic bony spur arising from the surface of the metaphyseal
bone similar to the appearance of a small osteochondroma or bone
spur, or in larger cases they can take on the appearance of a parosteal
sarcoma. However, the diagnostic feature of the parosteal lipoma
is the presence of a radiolucent cap of benign fatty tissue surrounding
the bone spur that extends out into the soft tissue a distance of 3-5
cm. These lesions are very benign, are usually asymptomatic and do
not require surgical treatment, similar to the situation with a soft
tissue lipoma.

269. CLASSIC
Case #163
64 year female
parosteal lipoma
distal humerus spur

270. Axial T-1 MRI

271. Axial T-2 MRI

272. Case #163.1
AP & lat x-ray of a parosteal lipoma distal femur in
A 67 yr male showing diagnostic bony spur at base

273. spur
Coronal and axial T-1 MRI

274. Axial T-2 FS MRI Axial Gad

275. Case #164
16 year female
parosteal lipoma spur
mid femur

276. Bone scan

277. spur
Axial CT scan

278. Case #165
spur
25 year female with parosteal lipoma 5th metacarpal

279. Case #859
29 year female spur
parosteal lipoma
proximal humerus

280. spur
Axial T-1 MRI

281. spur
Coronal T-1 MRI

282. Case #860
spur
74 year female with parosteal lipoma prox humerus

283. Intramedullary
Lipoma

284. Intramedullary Lipoma
Intramedullary lipomas are extremely rare conditions with approx-
imately 30 cases in the world literature. They are usually located
in the central area of the diaphysis of a long bone such as the femur,
tibia, or fibula. For some reason, a large 30% of cases of this rare
tumor are seen in the os calcis. These lesions are usually asympto-
matic and picked up as an incidental finding. They take on the
radiographic appearance of fibrous dysplasia of a long bone
because of slight fusiform dilatation of the surrounding cortex,
which is slightly thinned out and in the central area there is evidence
of degenerative calcification and occasionally even bone formation
in the lipoma. However, a T-1 weighted MRI image will show the
high signal features of a lipoma to help differentiate the tumor from
fibrous dysplasia that has a low signal on a T-1 weighted image.
These lesions are asymptomatic and do not require surgical treatment.
There is no threat to the patient of pathologic fracture.

285. CLASSIC
Case #166
40 year female
intramedullary lipoma
humerus

286. Coronal T-1 MRI

287. Intramedullary lipoma
Case #166.1
31 year male with
painless lytic lesion
in proximal humerus

288. CT scan

289. Cor T-1 T-2 Gad

290. Axial T-2 Gad

291. Sag T-2 Gad

292. Case #166.2 Intramedullary lipoma
45 year male with incidental finding in right shoulder

293. Sag T-1 T-2
Gad

294. Axial T-1 T-2
Gad

295. Case #166.2 Bone and soft tissue lipoma
CT scan of a 54 year old female
with a painless mass in axilla for
one year

296. 3D CT scan recon

297. Sag T-1 Gad

298. Axial T-1 T-2
Gad

299. Case #166.3 Combined bone and soft tissue lipoma
69 year female with incidental findings in thigh and femur

300. Cor T-1 Gad

301. Axial T-1 T-1
Gad Gad

302. Case #167
CT scan
53 year male with
ossifying lipoma
os calcis

303. Another CT scan

304. Case #167.1 Cystic lipoma
31 year old male with incidental finding in foot

305. Sag T-1 T-2
Gad

306. Axial T-1 T-2
Gad

307. Cor T-1 Gad

308. Case #167.2 Lipoma
50 year male with mild heal pain for 1 year

309. Sag T-1 STIR
Gad

310. Case #168
50 year female
ossifying lipoma
distal femur

311. Case #168.1 Intramedullary lipoma
31 year male with incidental finding in distal femur

312. Cor T-1 T-2 FS

313. Axial T-1 T-2 FS

314. Sag T-1 T-2 FS

315. Case #860.1
33 year female with intramedullary lipoma prox tibia

316. Coronal T-1 MRI

317. Axial T-1 MRI

318. Case #860.2
24 year male with intramedullary lipoma os calcis

319. Sagittal T-1 MRI

320. Coronal T-1 MRI

321. Case #860.3
Sagittal CT scan
55 year female with intracortical lipoma

322. Coronal T-1 MRI