volume 7

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