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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
Vascular Tumors
of Bone
Hemangioma of
Bone
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
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.
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.
CLASSIC
Case #146
14 year male
hemangioma tibia
Photomic
Case #147
45 year female with hemangioma ilium
Case #148
47 year male
hemangioma tibia
Bone scan
Axial T-2 MRI
Photomic
Higher power
Case #149
49 year male
hemangioma L-4
vertebra
Lateral view
Coronal PD MRI
Sagittal PD MRI
Axial PD MRI
Bone scan
Case #150
30 year female with hemangioma thoracic spine
Coronal CT scan
T-11
Axial CT scan
Case #151
25 year female with hemangioma skull
Case #801
13 year female
hemangioma femur
Proximal end
Coronal T-1 MRI
Sagittal T-1 MRI
Axial proton density MRI
Photomic
2 months post op
pins, cement and
cancellous allograft
Lateral view
cement
allograft
Case #802
27 year male
parosteal hemangioma
femur
AP view
Bone scan
Sagittal T-2 MRI
Axial T-2 MRI
Case #803
Parosteal sclerosing
hemangioma femur
28 year female
Hemicortical resection
specimen
Masson stain photomic
Placement
IM nail
Cement augmentation
Post op x-ray
radiolucent
cement
2.5 years later
Case #804
Parosteal
hemangioma femur
26 year male
Bone scan
Axial T-2 MRI
Case #805
14 year male
hemangioma femur
Another view
Case #806
31 year female
sclerosing parosteal
hemangioma femur
Case #807
4 year female
hemangioma humerus
Case #808
35 year male
path fracture thru
hemangioma femur
Skin overlying femur fracture
Close up cavernous hemangioma
Healed fracture
at later date
Case #809
26 year female
hemangioma tibia
AP view
Case #810
21 year female
hemangioma tibia
Case #811
26 year male
hemangioma tibia
Axial T-2 MRI
Case #812
19 year female with hemangioma scapula
Axial T-2 MRI
Case #813
21 year female with hemangioma scapula
Case #814
31 yr female with sclerosing hemagioma mid clavicle
Case #815
27 year female
hemangioma humerus
Case #816
30 year male with hemangioma radius & ulna
Lateral view
Case #817
41 year male with hemangioma distal radius
Case #818
24 year male with bone & soft tissue hemangioma hand
bone
soft tissue
phlebolith
Case #819
14 year female
hemangioma foot
Case #820
17 year male with hemangioma C-2 spine
Axial CT scan
Another axial cut
Axial T-1 MRI
Sagittal T-2 MRI
Case #821
47 year female with hemangioma C-4
Axial CT scan
Sagittal T-1 MRI
Sagittal T-2 MRI
Case #822
18 year male with hemangioma lumbo-dorsal spine
Sagittal T-1 MRI
Sagittal T-2 MRI
Axial T-1 MRI
Case #823
32 year male
hemangioma T-11
CT scan
sagittal T-1 MRI
Sagittal T-2 MRI
Case #824
60 year female with
hemangioma lumbo-
dorsal spine
Case #825
15 year female with hemangioma lumbar spine
Case #826
24 year female
hemangioma T-12
Case #827
25 year female
hemangioma skull
Lateral view
CT scan
Photomic
Case #828
23 year male
hemangioma skull
Case #829
19 year male
hemangioma
frontal bone
Case #830
31 year female with hemangioma skull
Case #831
36 year male with hemangioma superior pubic ramus
Bone detail
CT scan showing parosteal soft tissue component
Coronal T-2 MRI showing parosteal lesion
Axial gad contrast MRI
Photomic
Case #832
21 year male with hemangioma pubic bone
Cystic
Angiomatosis
CLASSIC
Case #152
37 year female
cystic angiomatosis
starting 1st in prox humerus
Coronal proton
density MRI
Coronal T-2 MRI
CT scan
Photomic
Higher power
Post op ORIF with
pins and cement
5 years later with
osteoblastic spontaneous
healing response
Same healing response
in dorsal spine multi-
focal disease over 5 yrs
Lateral view of spine
at 5 yrs
CT scan chest at 6 years
CT scan with blastic response in dorsal spine
Another CT cut
Bone scan at 5 yrs
Blastic response in pelvis at 5 years
5 year response
in opposite right humerus
8 years later with IM nail in
femur for stress pain
Hemangiomatosis
CLASSIC
Case #833
5 year female
multi-focal bony
hemangiomatosis
Skull changes
Rib changes
Photomic
Higher power
Case #834
5.5 year male
hemangiomatosis
femur
Distal femoral lesion
Proximal femoral
lesions
Coronal proton
density MRI
Photomic
Lymphangiomatosis
of Bone
CLASSIC
Case #835
15 year female with lymphangiomatosis of pelvis
Coronal T-2 MRI with bone & soft tissue lesions
Coronal gad contrast MRI
Axial T-2 MRI
Coronal T-2 MRI
cystic changes in thigh
Axial proton density MRI
Photomic
Case #836
24 year female with lymphangiomatosis pelvis
Lymphangiogram showing
large soft tissue disease
Case #837
12 year female with lymphangiomatosis pelvis
Case #838
16 year female
lymphangiomatosis
femur with pre and
post op x-rays of
IM nailing procedure pre op
bowing
IM nail
Gorham’s disease
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.
CLASSIC
Case #153
36 year male
Gorham’s disease
seen initially left hip
Spontaneous osteolysis
femoral head and neck
CT scan 3 years later shows massive osteolysis both hips
Coronal T-1 MRI at time of initial disease in left hip
Initial axial T-2 MRI with high signal changes left hip
Photomic shows changes similar to hemangiomatosis
Bone biopsy left hip
osteoid
Higher power showing capillary vascular pattern
Changes in right hip
3 years after onset
in left hip
Case #839
12 year male with Gorham’s disease skull and spine
AP view
Sagittal T-1 MRI showing skull defects
Another sagittal T-1 cut
Axial CT scan showing defects at C-1 level
Bone biopsy photomic changes similar to lymphangiomatosis
bone
Case #840
46 year female with Gorham’s disease L hip & pelvis
Hemangioendothelioma
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.
CLASSIC
Case #154
35 year female
low grade
hemangioendothelioma
distal femur
AP x-ray following
open biopsy
Photomic
Case #155
11 year male
hemangioendothelioma
distal tibia and talus
Curettement and
bone grafting distal
tibia
Photomic showing epitheliod cells
Case #156
35 year male with hemangioendothelioma rib
Photomic
Case #157
T-7
46 year female with hemangioendothelioma T-7
CT scan T-7 level
Sagittal T-2 MRI showing evidence of cord compression
Photomic
Post op x-ray following
posterior decompression
and spinal instrumentation
Case #158
69 year male high grade
hemangioendothelioma
distal tibia and fibula
Lateral view
Low power photomic
bone
Another photomic
Case #841
35 year female with hemangioendothelioma distal femur
Bone scan
Coronal T-1 MRI
Sagittal T-1 MRI
Axial T-2 MRI
Photomic
Case #842
19 year male with hemangioendothelioma distal femur
Coronal T-1 MRI
Case #843
40 year male
hemangioendothelioma
with path fracture
mid shaft humerus
Photomic
6 years later and
conversion to OGS
Different view at 6 yrs
Case #844
48 year female with hemangioendothelioma foot
Bone scan
Axial T-1 MRI
Sagittal T-1 MRI
Sagittal STIR MRI
Case #845
19 year male with hemangioendothelioma 4th metatarsal
Sagittal T-2 MRI
Case #846
52 year male
hemangioendothelioma
hand
Bone scan
Case #847
40 year female
hemangioendothelioma
T4 and 5
T-4
T-5
Lateral view T-4
T-5
Myelogram 4 mos later
showing cord compression
At T-5 level
Rib lesion same patient
Photomic
Immediate post op
lateral x-ray with
anterior rib graft
Solid fusion 4 yrs later
no recurrence
5 years post op
lateral view showing
anterior rib graft
High Grade
Angiosarcoma of
Bone
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.
CLASSIC Case #159
21 year male with angiosarcoma pelvis
Progressive disease at a later date
CT scan above sciatic notch level
CT scan just below the sciatic notch
CT scan at femoral head level
Coronal T-1 MRI
tumor
Axial T-1 MRI
tumor
Photomic
Higher power
Case #848
50 year male with angiosarcoma pelvis
CT scan
Bone scan
Coronal T-1 MRI
Axial T-2 MRI
Photomic
Immediate post op internal hemipelvectomy with THA
Case #849
34 year male
angiosarcoma fibula
Coronal proton density MRI
tumor
Axial proton density MRI
tumor
Photomic
Case #849.1
Cor T-1 T-2 Gad
46 year male smoker with fibular head mass for 3 months
Bone Angiosarc
Sag T-1 T-2 Gad
Axial T-1 T-2
Gad
Case #850
66 year male
angiosarcoma
tibia and fibula
Oblique view
Femur involved also
Photomic
Case #851
60 year male
angiosarcoma femur
9 mos. Following
segmental resection
autoclaving and
replacement over IM
nail
Multifocal radial lesion at a later date
Low power photomic
Hemangiopericytom
a of Bone
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.
CLASSIC Case #160
25 year female with benign hemangiopericytoma talus
AP view
Mortice view
Low power photomic
High power
Post op x-ray after
curettement and
cementation
22 years later with minimal pain with degenerative OA and
beaking at the talo-narvicular joint
Case #161
25 year female
hemangiopericytoma
(glomus tumor) distal
phalanx index finger
Case #162
55 year male
malignant
hemangiopericytoma
pelvis
Higher power
Post op x-ray following wide surgical resection
Case #852
47 year female with metastatic hemangiopericytoma
Proximal humerus
Bone scan
Coronal T-1 MRI
Photomic
Case # 853
67 year female
malignant
hemangiopericytoma
humerus with path
fracture
Another view
Photomic
Case #853.1
49 yr male with malignant hemangiopericytoma mid femur
Case #854
39 year female with benign hemangiopericytoma rib 3 years apart
Case #855
29 year female
malignant
hemangiopericytoma
thoracic spine
Laminogram cut
CT scan
Lipid Bone
Tumors
Parosteal Lipoma
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.
CLASSIC
Case #163
64 year female
parosteal lipoma
distal humerus spur
Axial T-1 MRI
Axial T-2 MRI
Case #163.1
AP & lat x-ray of a parosteal lipoma distal femur in
A 67 yr male showing diagnostic bony spur at base
Coronal and axial T-1 MRI
spur
Axial T-2 FS MRI Axial Gad
Case #164
16 year female
parosteal lipoma
mid femur
spur
Bone scan
Axial CT scan
spur
Case #165
25 year female with parosteal lipoma 5th metacarpal
spur
Case #859
29 year female
parosteal lipoma
proximal humerus
spur
Axial T-1 MRI
spur
Coronal T-1 MRI
spur
Case #860
74 year female with parosteal lipoma prox humerus
spur
Intramedullary
Lipoma
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.
CLASSIC
Case #166
40 year female
intramedullary lipoma
humerus
Coronal T-1 MRI
Case #166.1
31 year male with
painless lytic lesion
in proximal humerus
Intramedullary lipoma
CT scan
Cor T-1 T-2 Gad
Axial T-2 Gad
Sag T-2 Gad
Case #166.2
45 year male with incidental finding in right shoulder
Intramedullary lipoma
Sag T-1 T-2
Gad
Axial T-1 T-2
Gad
Case #166.2
CT scan of a 54 year old female
with a painless mass in axilla for
one year
Bone and soft tissue lipoma
3D CT scan recon
Sag T-1 Gad
Axial T-1 T-2
Gad
Case #166.3
69 year female with incidental findings in thigh and femur
Combined bone and soft tissue lipoma
Cor T-1 Gad
Axial T-1 T-1
Gad Gad
53 year male with
ossifying lipoma
os calcis
Case #167
CT scan
Another CT scan
Case #167.1
31 year old male with incidental finding in foot
Cystic lipoma
Sag T-1 T-2
Gad
Axial T-1 T-2
Gad
Cor T-1 Gad
Case #167.2
50 year male with mild heal pain for 1 year
Lipoma
Sag T-1 STIR
Gad
Case #168
50 year female
ossifying lipoma
distal femur
Case #168.1
31 year male with incidental finding in distal femur
Intramedullary lipoma
Cor T-1 T-2 FS
Axial T-1 T-2 FS
Sag T-1 T-2 FS
Case #860.1
33 year female with intramedullary lipoma prox tibia
Coronal T-1 MRI
Axial T-1 MRI
Case #860.2
24 year male with intramedullary lipoma os calcis
Sagittal T-1 MRI
Coronal T-1 MRI
Case #860.3
55 year female with intracortical lipoma
Sagittal CT scan
Coronal T-1 MRI