Case Review

Primary haemangioma of the skull

Awadhesh Kumar Jaiswal1, Sushila Jaiswal2, Ritu Verma2, Udit Singhal1, Rabi Narayan Sahu1

Abstract: Intraosseous haemangiomas of skull are rare neoplasms. The authors report a series of three cases of
intraosseous haemangioma of skull. The clinical features, pathological findings and management of these lesions are
discussed in the light of available literature. (p68-70)

Key words: Cavernous haemangioma, parietal, occipital and skull.

Introduction
Primary haemangiomas of the skull are rare skeletal received radiotherapy following surgery. Follow-up after 24
tumours and comprise of 0.7% of all osseous neoplasms.5 months showed no recurrence on clinical examination.
Calvarial involvement is exceedingly rare and account for
0.2% of all bone neoplasms.11,16 Common sites of involve-
ment in skull are frontal and parietal bone.2,4,10 We describe
three cases of intraosseous haemangioma - one in occipital
and the other two in parietal bone.

Illustrative Cases
Case 1: A 25-year-old male presented with slowly
progressive enlarged swelling over the occipital bone on the
left side for the last 3 years with rapid increase in size over
Figure 1 - Non-contrast CT head (bone window) axial section
the last one month. Examination revealed a bony hard mass showing focal widening of intradiploic space of the occipital
of 8 × 6 cm in the occipital bone, more on the left side. It bone with erosion of outer table and preservation of inner table.
was non-tender and overlying skin was normal. Neuro- The histopathology was suggestive of haemangioma.
logical examination was unremarkable. Non-contrast CT
head showed focal expansion of the diploic space of the
occipital bone on the left side with erosion of the outer table
and preserved inner table margin (Fig. 1). The underlying
brain parenchyma was normal. Patient was operated in
prone position under general anaesthesia. Intraoperatively,
the mass has eroded the outer table and was very vascular;
the inner table was intact. The mass was excised piecemeal.
Histopathological examination of the mass showed multiple
dilated vascular spaces, lined by endothelium separated by
fibrocollagenous tissue suggestive of haemangioma (Fig. Figure 2 - Microphotograph of the tumour specimen showing
2). The patient had smooth postoperative recovery. She multiple dilated vascular spaces lined by endothelium sepa-
rated by fibrocollagenous tissue suggestive of haemangioma.

1
Department of Neurosurgery
Case 2: A 24-year-old lady presented with progressive
2
Department of Pathology enlarged swelling on the left parietal region for the last 4
Sanjay Gandhi Postgraduate Institute of Medical Sciences years. It was not associated with vomiting or seizure.
Lucknow
India Physical examination revealed a non-tender, bony hard
swelling measuring 4 × 6 cm in the left parietal region.
Correspondence:
Dr. Awadhesh Kumar Jaiswal Computed tomography scan head showed focally widened
Department of Neurosurgery intradiploic region in the left parietal bone with erosion of
Sanjay Gandhi Postgraduate Institute of Medical Sciences
Lucknow-226014 UP the outer and inner tables (Fig. 3). The underlying brain was
India normal. The bony swelling was excised totally and the bony
Email: akjaiswal@sgpgi.ac.in / awadheshsushila@gmail.com defect was managed with methylmethacrylate cranioplasty.

68 PAN ARAB JOURNAL OF NEUROSURGERY

 PRIMARY HAEMANGIOMA OF THE SKULL • Jaiswal, et al
Histopathology of the mass was suggestive of haeman-
gioma. At follow-up of 11 months, patient was free from
symptoms without any recurrence as evident on clinical
examination.
Figure 3 - Non-contrast CT head (bone window) axial section
showing focal expansion of intradiploic space of the parietal
bone with involvement of both outer and inner tables.
Case 3: A 19-year-old lady presented with progressive
enlarged swelling on the left parietal region for the last 2
years. It was not associated with vomiting or seizure.
Physical examination revealed a non-tender, bony hard
swelling measuring 3 × 3 cm in the left parietal region.
Computed tomography scan head showed focally widened
intradiploic region in the left parietal bone with erosion of
the outer table leaving the inner table intact (Fig. 4). The
underlying brain was normal. The bony swelling was
excised en bloc and methylmethacrylate cranioplasty was
performed. Histopathology of the bony mass was sugges-
tive of haemangioma. The patient had uneventful
postoperative recovery. At follow-up of 14 months, she was
free from symptoms without any recurrence as evident on
clinical examination.
Figure 4 - Non-contrast CT head (bone window) axial section
showing focal widening of intradiploic space of the parietal
bone with erosion of outer table and preservation of inner table.
Discussion
Primary haemangioma of the skull was first described in
1845.16 These are benign vascular tumours of the bone.2
Most common site of involvement is vertebral column.
Calvarial involvement is rare and if occurs the common
sites are parietal and frontal bone, less common sites are
VOLUME 15, NO. 1, APRIL 2011
occipital, sphenoid, temporal and petrous bones.12 Extra-
calvarial involvement of nasal bone, maxilla, mandible,
orbit and zygomatic bone has been also described.3 They
affect the diploie, causing expansion of the outer table
greater than the inner table, thereby producing a palpable
lump. They are usually solitary but multifocal lesions have
also been reported.
The exact origin of the calvarial haemangioma is not
known. There is usually congenital involvement. Trauma is
not a proven aetiological factor.3 Faulty differentiation of
the primordial vessels resulting in an abnormal capillary
bed may be an aetiological factor.7,12 Skull haemangioma
commonly occurs in middle aged women. There is no
known familial association.3,12,16 Usually it presents with
slowly enlarging, painless bony calvarial swelling and in
rare cases it may present as subarachnoid haemorrhage,
proptosis and extradural haematoma8. All our cases pre-
sented with painless bony lump.
Skull x-ray usually shows a lytic lesion in a honeycomb or
sunburst appearance with striations radiating from the
centre. Both outer and inner tables are involved but often
inner table is preserved.5,11,15,16 In our Case 1, the inner table
was preserved while in the other two cases both tables were
involved. Computed tomography scan demonstrates the
effect of lesion on the inner and outer tables and also reveals
the extension of tumour beyond the calvarium.3 Magnetic
resonance imaging shows a non-homogeneous hypo- to
isointense mass on T1-weighted image and a heterogenous
hyperintense mass on T2-weighted images with diffuse
enhancement on contrast administration.3,12 Angiography
shows an intraosseous hypervascular mass with dilated
feeding arteries and veins.13
Histopathologically, intraosseous haemangiomas are classi-
fied as venous, cavernous and capillary type according to
their vascular network. The cavernous type is composed of
large thin-walled vessels and sinusoids lined with a single
layer of endothelium, whereas the capillary type is formed
by a small fine vascular network filled with blood.1
Regarding natural history, skull haemangiomas have a
benign slow growing course and do not normally undergo
spontaneous involution. Only one case had a spontaneous
haemorrhage which resulted in a fatal epidural haematoma.6
Most authors have recommended gross total surgical
excision to treat mass effect and neurological compromise,
to improve a cosmetic deformity, and to obtain a definitive
diagnosis.10,17 The usual treatment of skull haemangioma is
en bloc resection of the tumour.3,9 Curettage with or without
radiation has also been described. Curettage alone is not
sufficient as there is high potential of recurrence following
curettage without radiation.12,14 Radiation decreases tumour
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 PRIMARY HAEMANGIOMA OF THE SKULL • Jaiswal, et al
size and vascularity and has definite role in the treatment of
skull haemangiomas which are unresectable and those
deeply invading the bone and the orbit. In our Case 1, the
inner table was left behind hence patient was advised
radiation following surgery, while in the other two cases, en
bloc resection of tumour and cranioplasty was performed
and hence these two patients were not advised radiation
following surgery.
Conclusions
Primary haemangiomas of skull are rare tumours, present-
ing with painless bony lump. Computed tomography scan
is the choice of investigation. Complete surgical resection is
the recommended treatment with good prognosis. Radiation
is recommended in case of inability to eradicate the lesion
completely.
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