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CHAPTER 18
Vascular Tumours
Primary vascular tumours of bone are rare. Haemangiomas
occur as incidental findings in the skull or in the spine. The
roentgenographic features are almost always diagnostic. They
rarely cause clinical symptoms.
The terminology for malignant vascular tumours has been
controversial. Angiosarcoma is the most acceptable term for
malignant vascular tumours. They have a peculiar tendency to
involve multiple bones. Histological grading correlates well with
prognosis.
Epithelioid haemangioendothelioma is a distinct entity with
histological features identical to those of the soft tissue counter-
part and is associated with an favourable clinical course.
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C.P. Adler
Haemangioma and related lesions
L. Wold
Table 18.01
Definition dental radiographic findings. However,
Variants of haemangiomas.
A benign vasoformative neoplasm or large vertebral tumours may cause cord
developmental condition of endothelial compression, pain and neurological
origin. symptoms. Symptomatic tumours occur-
Haemangioma:
ing elsewhere are painful and may cause cavernous,
capillary,
ICD-O code 9120/0 a pathologic fracture. Haemangiomas
epithelioid,
present as a well demarcated lucent
histiocytoid,
Synonyms mass that frequently contains coarse tra-
sclerosing
Capillary haemangioma, cavernous hae- beculations or striations. In flat bones
Papillary vegetant endothelial proliferation
mangioma, venous haemangioma, like the calvarium, the tumour is expan-
(Masson type)
angioma, histocytoid haemangioma, sile and lytic and produces a sunburst
Angiolymphoid hyperplasia with eosinophilia
angiomatosis. pattern of reactive bone formation.
(Kimura disease)
Clinically, indolent lesions frequently
Epidemiology contain fat and sclerotic trabeculae on
Haemangiomas are relatively common CT and MRI. Symptomatic tumours usu- Angiomatosis:
non-aggressive: regional,
lesions; autopsy studies have identified ally show loss of fat and reveal a low sig-
disseminated: cystic angiomatosis
them in the vertebrae of approximately nal on T1-weighted images and a high
aggressive: massive osteolysis
10% of the adult population {18}. signal on T2 {539,644,1280,1354,1875,
(Gorham-Stout syndrome)
However, clinically significant sympto- 2287}.
matic tumours are very uncommon and
account for less than 1% of primary bone Macroscopy
Osseous glomus tumour (glomangioma)
tumours {539}. Haemangiomas occur at Haemangioma manifests as a soft well
Lymphangioma
any age, but most are diagnosed during demarcated dark red mass. It may also
Lymphangiomatosis
middle and late middle age with the have a honey-comb appearance with
peak incidence in the 5th decade of life intralesional sclerotic bone trabeculae
{1875}. The male to female ratio is about and scattered blood-filled cavities.
2:3 {18,539,1875,2153,2249}.
Histopathology
Sites of involvement Haemangiomas have variable histologi-
Vertebral bodies are the most common cal features. Capillary and cavernous
site, followed by the craniofacial skeleton, haemangiomas are composed of thin-
and then the long bones where they tend walled blood-filled vessels lined by a sin-
to involve the metaphyses {18,539,2249}. gle layer of flat, cytologically banal
endothelial cells. The vessels permeate
Clinical features / Imaging the marrow and surround preexisting tra-
The majority of haemangiomas, espe- beculae. When capillary or cavernous
cially those arising in the spine, are inci- haemangiomas involve a large localized
A B
Fig. 18.02 Haemangioma of bone. Gross specimen of
Fig. 18.01 Haemangioma of bone. A Plain radiographs show a lesion with multiple cystic defects within the
a tumour of the proximal fibula with a focus of
distal tibia. B CT cross-sectional appearance of a vertebral haemangioma where the coarse trabeculae
brown-red appearance without marginal sclerosis.
result in a  polka-dot pattern.
320 Vascular tumours
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composed of large polyhedral neoplastic Immunophenotype
endothelial cells that have vesicular The endothelial cells uniformly express
nuclei and abundant eosinophilic cyto- vimentin and many cells stain with anti-
plasm. Some tumour cells have round bodies to F. VIII, CD31, and CD34.
clear cytoplasmic vacuoles that may Epithelial haemangiomas may also
contain intact or fragments of red blood express keratins and EMA. FLI1 has also
cells. Vacuoles in neighbouring cells been observed in haemangiomas.
often fuse forming vascular lumena. The
epithelioid cells may line well formed Ultrastructure
vascular spaces or grow in solid cords or The endothelial cells contains Weibel-
sheets. The stroma consists of loose con- Palade bodies. Cytoplasmic filaments are
Fig. 18.03 Haemangioma of bone. The radiated spicules
nective tissue and may contain a mixed abundant in epithelioid endothelial cells.
are demonstrated on this macerated specimen.
inflammatory infiltrate including
eosinophils. Prognostic factors
region or are widespread throughout the The vessels in lymphangioma are dilat- Haemangiomas have an excellent prog-
skeleton, it is known as angiomatosis. ed, sinusoidal, filled with lymph fluid and nosis and have a low rate of local recur-
Gorham disease may be associated with lined by a single layer of flat, banal rence. Progression to an angiosarcoma
a histological picture that resembles hae- endothelial cells. The surrounding stroma is an extraordinarily rare event {528,611,
mangioma. Epithelioid haemangioma is may contain lymphocytes. 641,649,1628}.
A B
Fig. 18.04 Haemangioma of bone. A This bony haemangioma shows the morphology commonly associated with cavernous lesions which have been curetted. The
spaces often become collapsed, and blood is no longer present because of the processing. B Histological pattern of a cavernous haemangioma showing broad thin-
walled blood vessels, lined by a single layer of flat endothelial cells and filled with blood, within the medullary cavity between the bone trabeculas.
Haemangioma and related lesions 321
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A. Roessner
Angiosarcoma
T. Boehling
Definition Sites of involvement gioma to poorly differentiated tumours
Angiosarcomas of bone are composed Malignant vascular tumours of bone which may be difficult to identify as a
of tumour cells which show endothelial show a wide skeletal distribution. They vascular tumour. Histo-logically, reactive
differentiation. tend to affect the long tubular bones of bone formation can sometimes be
the extremity and the axial skeleton, observed in angiosarcoma of bone. This
ICD-O code 9120/3 mainly the spine. These tumours reveal is more pronounced in the periphery, but
the tendency to develop multicentric can also be found in the more central
Synonyms lesions in bone. About a third of these portions of the lesion.
Haemangiosarcoma, haemangioen- lesions are multifocal. Poorly differentiated angiosarcomas are
dothelioma, haemangioendothelial sar- composed of more atypical endothelial
coma, epithelioid angiosarcoma. Clinical features / Imaging cells. They exhibit very prominent nu clear
Malignant vascular tumours most com- atypia and a considerably increased
Epidemiology monly present as painful lesions which number of mitoses with atypical mitotic
Malignant vascular tumours of bone are may be associated with a mass. figures. Formation of intraluminal buds
very rare and account for less than 1% of Angiosarcoma usually develops purely can often be observed. Areas with
malignant bone tumours. Age distribu- lytic bone lesions. They are poorly mar- necroses may be present. Some tumours
tion shows a wide range with nearly ginated but can occassionally have a may show epithelioid cytological features
equal distribution from the second to the sclerotic rim. A soft tissue mass is often and mimic the appearance of metastatic
eighth decade. Epithelioid haeman- associated with less well differentiated carcinoma. Others show spindle cell cyto-
gioendothelioma tends to occur during tumours. The radiological appearence of logical features and mimic other primary
the second and third decades of life. epithelioid hemangioendothelioma is bone sarcomas.
Males and females are affected approxi- also non-specific. They also present as Epithelioid haemangioendothelioma is
mately equally. purely lytic lesions with varying degrees composed of anastomosing cords, solid
of peripheral sclerosis. Although the nests, and strands of endothelial cells
radiographic feature of malignant vascu- that may sometimes form narrow vascu-
lar tumours of bone are nonspecific, lar channels. The small capillary-sized
clustering of multifocal lesions in a single tumour vessels can mimic small reactive
anatomic location suggests the diagno- vessels of granulation tissue. The epithe-
sis of a vascular neoplasm. lioid cells tend to have eosinophilic cyto-
plasm which may show vacuolization
Aetiology and sometimes signet ring-like appear-
Angiosarcomas may arise at sites of ance. Of remarkable significance is the
prior radiation {338,452,1716}. The aeti-
ology of the majority of malignant vascu-
lar tumours is unknown.
Macroscopy
Angiosarcomas are bloody and general-
ly firm in their consistency. Necrosis is
generally not observed. Epithelioid hae-
mangioendotheliomas tend to be firm
and tan-white. Both tumours can erode
the cortex and extend into the soft tis-
sue.
Histopathology
Tumour cells forming vascular spaces
constitute the general histological fea-
ture of angiosarcoma of bone.
Fig. 18.05 Angiosarcoma. Plain X-ray of a tumour
Angiosarcoma of bone shows a wide
involving the proximal femur, featuring a purely
range of histology, ranging from well dif-
lucent destructive process in the intertrochanteric Fig. 18.06 Angiosarcoma. T2 MRI of a multicentric
region. The radiological appearance is nonspecific. ferentiated cases mimicking haeman- tumour involving multiple bones of the foot.
322 Vascular tumours
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myxoid and hyalinized appearance of
the connective tissues stroma. The
nuclei of the neoplastic cell show vary-
ing degrees of pleomorphism and
anaplasia.
Immunophenotype
The endothelial cells uniformly express
vimentin and many cells stain with anti-
bodies to Factor VIII, CD31, CD34, and
Ulex Europaeus. Epithelioid malignan-
cies may also express cytokeratins and
EMA {1134,2249}.
Ultrastructure
The endothelial cells contain Weibel-
Palade bodies, but are generally difficult
to find in poorly differentiated tumours.
Cytoplasmic filaments are abundant in
epithelioid neoplasms.
Fig. 18.07 Epithelioid haemangioendothelioma. The tumour cells are arranged in a cording fashion in a myxoid
stroma. Note the occasional cytoplasmic vacuoles. Genetics
Two epithelioid haemangioendothe-
liomas have shown an identical chromo-
somal translocation involving chromo-
somes 1 and 3 {1403}.
Prognostic factors
The histological degree of differentiation
is the most significant factor in the prog-
nosis of patients with malignant vascular
tumours of bone {300,2288}. Some stud-
ies have also suggested that multifocal
tumours show a survival advantage. This
A B
survival advantage may in part be relat-
Fig. 18.08 A Epithelioid angiosarcoma. The tumour cells form anastomosing channels, have large nuclei and
ed to the multifocal tumours showing
prominent nucleoli. B High grade angiosarcoma showing atypical cells with poorly formed papillae present
better differentation {1134,2142}.
within spaces.
Angiosarcoma 323