CASE REPORT – OPEN ACCESS

International Journal of Surgery Case Reports 22 (2016) 79–82

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International Journal of Surgery Case Reports

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Calvarial bone cavernous hemangioma with intradural invasion: An

unusual aggressive course—Case report and literature review
Davide Nasi a,∗ , Lucia di Somma a , Maurizio Iacoangeli a , Valentina Liverotti a ,
Antonio Zizzi b , Mauro Dobran a , Maurizio Gladi a , Massimo Scerrati a
a
Department of Neurosurgery, Università Politecnica delle Marche, Umberto I General Hospital, Ancona, Italy
b
Department of Pathology, Università Politecnica delle Marche, Umberto I General Hospital, Ancona, Italy

a r t i c l e i n f o a b s t r a c t

Article history: INTRODUCTION: Cavernous hemangioma of the skull is a rare pathological diagnosis, accounting for 0.2%
Received 31 December 2015 of bone tumors and 7% of skull tumors. Usually calvarial bone cavernous hemangioma are associated
Received in revised form 24 March 2016 with a benign clinical course and, despite their enlargement and subsequent erosion of the surrounding
Accepted 24 March 2016
bone, the inner table of the skull remains intact and the lesion is completely extracranial.
Available online 1 April 2016
PRESENTATION OF A CASE: The authors present the unique case of a huge left frontal bone cavernous
malformation with intradural extension and brain compression determining a right hemiparesis.
Keywords:
DISCUSSION: Calvarial cavernous hemangiomas are benign tumors. They arise from vessels in the diploic
Cavernous hemangioma
Bone tumors
space and tend to involve the outer table of the skull with relative sparing of the inner table. More
Intradural extension extensive involvement of the inner table and extradural space is very unusual and few cases are reported
Skull tumors in literature. To the best of our knowledge, intradural invasion of calvarial hemangioma has not been
previously reported.
CONCLUSION: Our case highlights the possibility of an aggressive course of this rare benign pathology.
© 2016 The Authors. Published by Elsevier Ltd. on behalf of IJS Publishing Group Ltd. This is an open
access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction been previously reported. This case highlights the possibility of an

Cavernous hemangioma of the skull is a rare pathological diag-
nosis, accounting for 0.2% of bone tumors and 7% of skull tumors [1].
They can occur at any age, but are commonly found in females who
are in the fourth or fifth decades of life [2]. These cranial lesions are
usually solitary and most often found in frontal and parietal bones,
but the lesion could occur in almost any skull region (occipital,
sphenoidal, clival and ethmoidal regions) [3].
Calvarial cavernous hemangiomas arise from vessels in the
diploic space and are supplied by the branches of the external
carotid artery. The middle meningeal and superficial temporal
arteries are the main sources of blood supply [4].
Calvarial hemangiomas tend to involve the outer table of the
skull and the diploe, with relative sparing of the inner table [5].
More extensive involvement of the inner table and extradural space
is very unusual [6].
The authors present the unusual case of a frontal bone cav-
ernous malformation with intradural extension. To the best of our
knowledge, intradural invasion of calvarial hemangioma has not
∗ Corresponding author at: Department of Neurosurgery, Università Politecnica
delle Marche, Umberto I General Hospital, Via Conca #71, Ancona 60020, Italy.
E-mail addresses: davidenasi83@gmail.com, neurotra@tiscali.it (D. Nasi).
aggressive course of this rare benign pathology.
2. Presentation of a case
2.1. History and examination
A 60-year-old man with a left frontal mass was admitted to our
Department after 2 week of increasing headaches, nausea, right arm
weakness with poor coordination, and difficulty walking because
of a “clumsy” right leg. A clinical history of uncontrolled hyperten-
sion, diabetes mellitus and prostatic hypertrophy is reported. The
patient denied previous trauma. The mass had been present for
at least 6 months and had shown gradual growth. Physical exam-
ination revealed an approximately four cm in diameter palpable
and non-tender mass in the left frontal region. Neurologic exami-
nation revealed a patient with right hemiparesis. The patient had
3/5 strength in the right upper and lower extremities. The deep
tendon reflexes were slightly increased on the right side. Magnetic
resonance imaging (MRI) revealed a large extra-axial lesion with
epicenter in left frontal bone, predominantly solid (Fig. 1A–C). It
measured 4 cm in its largest dimension and approximately 3 cm
in depth. There was discontinuity in the cortical surfaces of both
the inner and outer tables of the skull with intracranial inva-
sion. The intracranial component of the lesion compressed the left

http://dx.doi.org/10.1016/j.ijscr.2016.03.041
2210-2612/© 2016 The Authors. Published by Elsevier Ltd. on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://
creativecommons.org/licenses/by-nc-nd/4.0/).
 CASE REPORT – OPEN ACCESS
80 D. Nasi et al. / International Journal of Surgery Case Reports 22 (2016) 79–82

frontal lobe. The extracranial component was also seen in the left
frontal scalp region. The lesion was predominantly isointense on
T1-weighted images (Fig. 1A) and hyperintense on T2-weighted
images (Fig. 1B). After the administration of Gadolinium, there was
heterogeneous enhancement of the mass (Fig. 1C). A surgical plan
was made under the assumption that this lesion was a meningioma.
2.2. Surgical treatment
mal, with complete recovery from his previous motor weakness.
Postoperative CT scan showed a good restoration of the cranial
deformity (Fig. 1D).
He was discharged home on postoperative Day 3 after an
uneventful course. The patient has been followed up for 48 months
post-operatively and remains asymptomatic with an excellent cra-
nial contour and no evidence of recurrence on serial MRI imaging
(Fig. 1E and F).

During surgery, the patient was positioned in the supine decu-
bitus position and head was fixed in 3-pin head holder and rotated
to the right. A question mark-shaped incision was given just behind
the posterior margin of the mass and the skin flap was raised sub-
galeally. Soft, reddish-brown, highly vascular lesion was seen in
the left frontal region with erosion of the outer table. The whole
extracranial portion was removed. Then we proceed to remove
the intracranial portion of the tumor. After placing a burr hole
behind the lesion, a circular craniotomy of about 6 cm was per-
formed around the lesion. At this point, the tumor was found to
be not completely extradural. In fact, the lesion eroded the dural
mater with invasion of intradural compartment. The lesion is then
coagulated and followed up to identify the opening of the dural
mater. A defect of the dural plane caused by the tumor has been
used to remove the residual intradural part of the lesion. At this
stage, the lesion did not have arterious pial afferents anymore. The
pia mater was thick and whitish. After total removal of the lesion,
a polymethyl methacrylate cranioplasty was performed.
2.4. Pathological findings
Macroscopically, the pathologic bone presented a huge purple-
red blush mass that eroded completely the inner table. The dura
appeared eroded throughout its thickness by a bluish red spongy
lesion.
Histological examination of the bone revealed a capillary
hemangioma consisting of thin-walled blood vessels, some of
which were distended with blood (Fig. 2A). Microscopic examina-
tion of the dura showed pathological vessels perforating the dura
mater (Fig. 2B). The vessels were separated by scarce connective
tissue intensely blue stained (Fig. 2C). Immunohistochemical stain-
ing with CD34 highlights the contours of the malformed vessels
(Fig. 2D).
The present work has been reported in line with the CARE cri-
teria [7].
3. Discussion

2.3. Postoperative course Hemangiomas are benign tumors of blood vessels and are his-
tologically classified as cavernous and capillary [8]. Most of the
Postoperatively, the patient presented immediate relief of calvarial hemangiomas are of the cavernous type. The cavernous
symptoms, and results of his neurological examination were nor- type is composed by a group of large, dilated blood vessels sepa-

Fig. 1. (A–C) Pre-operative MRI imaging revealed a large extra-axial lesion with epicenter in left frontal bone and intradural invasion, predominantly isointense on axial
T1-weighted image (A), hyperintense on coronal T2 weighted image (B) and with contrast enhancement (C). (D) Postoperative CT scan showed a good restoration of the
cranial deformity. (E, F) Follow-up MRI axial and coronal T1 weighted images with Gadolinium demonstrated no evidence of recurrence.
 CASE REPORT – OPEN ACCESS
D. Nasi et al. / International Journal of Surgery Case Reports 22 (2016) 79–82
Fig. 2. A Cavernous angioma made of a proliferation of thin-walled vessels with dilated lumina. The vessels are seen substituting the bone tissue of the cranial vault (black
arrow). H&E∗ × 100. (B) The thin-walled vessels are seen perforating the dura (black arrows). H&E∗ × 100. (C) The vessels are separated by scarce connective tissue intensely
blue stained (white arrow). Picro Mallory ×100. (D) Immunohistochemical staining with CD34 highlights the contours of the malformed vessels (black arrows). ×100.
∗ H&E: haematoxylin and eosin stain.
rated by fibrous tissue. On the contrary, vertebral hemangiomas are
most frequently of the capillary type that lacks fibrous septa and
have smaller vascular lumen.
Calvarial cavernous hemangiomas arise from vessels in the
diploic space and tend to involve the outer table of the skull with
relative sparing of the inner table. In fact, despite their enlargement
and subsequent erosion of the surrounding bone, the inner table
remains intact [5]. More extensive involvement of the inner table
and extradural space is very unusual [6]. Some authors reported
the grow of intraosseous cavernous hemangiomas into the internal
skull with dural sparing [6]. Uemura et al. described a patient with
a calvarial cavernous hemangioma who presented with an epidu-
ral haemorrhage in which the inner table was completely eroded
and the lesion was in direct contact with intact dura mater [9].
Khanam et al. [2] reported a right sphenoid wing hemangioma with
involvement of adjacent dura, which was also resected and sent for
pathologic analysis. Finally, Gottfried et al. [10] presented a case
of a 50-year-old man in whom a symptomatic subdural hematoma
resulting from a cavernous hemangioma of the calvaria had hem-
orrhaged and eroded through the inner table of the skull and dura
mater. During surgery, the authors found that the dura mater was
eroded all the way through in the area immediately subjacent to
the skull cavernous hemangioma, making the subdural space con-
tiguous with the calvarial lesion.
Nevertheless, in all of these cases a clear extension of the tumor
into the subdural space was not documented [1–13].
The peculiarity of our case consists in the complete erosion of
dural plane with invasion of subdural space by the skull cavernous
hemangioma. During surgery, after durotomy, the lesion did not
present pial vascular afferents and both the arachnoid and the pia
mater appeared thickened and whitish, but intact. To the best of our
knowledge, this extensive intradural invasion of calvarial heman-
gioma has not been previously reported.
These lesions are usually small and asymptomatic but can cause
a variety of symptoms depending on their location [8]. Patients
most often report a slowly enlarging mass that may become tender,
81
but most often is not [11]. Headaches are reported to occur as the
lesion expands. Neurological symptoms can occur especially hear-
ing loss, pulsatile tinnitus, and facial paralysis when the petrous or
sphenoid bones are involved [12].
Another peculiarity of our patient was the presence of hemi-
paresis related the brain compression. In fact, the presence of motor
deficit was not previously described in frontal bone cavernous
hemangioma.
The classic appearance of a hemangioma on radiographs are a
“honeycomb” pattern, denoting rounded area of rarefaction [3].
This pattern can be also appreciated on bone windows of a CT
scan. Enhancement with contrast material is intense on both CT and
MRI. On MRI imaging, these lesions show an intermediate to high
T1 signal intensity and a high, hetereogeneousT2 signal intensity
[1–6].
Benign lesions with similar findings include aneurysmal bone
cyst, osteoma, giant cell tumor or fibrous dysplasia. More aggres-
sive lesions include meningioma, multiple myeloma, metastatic
disease and osteosarcomas [1–10]. Therefore, classic MRI features
may not be sufficient to distinguish the different pathologic entities.
Sometimes, the classic radiographic appearances are not evident.
Consequently, the diagnosis is established in most of the cases only
after surgery and biopsy [1–5].
The standard therapy for cranial hemangiomas is the surgical
resection with or without cranioplasty [1–12]. To prevent recur-
rence, removal of the lesion plus a 1-cm-wide margin of uninvolved
bone is always recommended [5]. This method allows the removal
of the tumor intact, obviating the risk of significant bleeding
because the sinusoids are undisturbed. In our case, after removal
of whole extracranial, intraosseous and extradural components of
the lesion in one piece, we proceed to the excision of the infiltrated
dura mater and intradural part of the tumor.
Reconstruction of the defect with methylmethacrylate quite
often yields favorable cosmetic results [5], as seen in our patients.
Before cranioplasty, in this case we perfomerd a duroplasty with
bovine pericardium.
 CASE REPORT – OPEN ACCESS
82 D. Nasi et al. / International Journal of Surgery Case Reports 22 (2016) 79–82
Alternative therapies used for the treatment of cranial heman-
giomas include surgical percutaneous embolization, curettage, and
radiotherapy [13]. Curettage is associated with an increased risk
of haemorrhage and recurrence, since residual tumor can be left
behind. Radiotherapy, typically used when resection is not possi-
ble, has been shown to decrease the size of hemangiomas, but not
reduce the risk of haemorrhage and is associated with radiation-
induced carcinoma [7–13].
4. Conclusion
The authors present the unusual case of a huge frontal bone
cavernous malformation with intradural extension and brain com-
pression determining a hemiparesis. To the best of our knowledge,
intradural extension of calvarial hemangioma has not been previ-
ously reported. On the basis of this report, neurosurgeon must be
aware on the possibility of an aggressive course of this rare benign
pathology
Conflict of interest
The authors declare that there is no conflict of interest regarding
the publication of this paper.
Funding
No involvement of any funding to be mentioned.
Ethical approval
Approval obtained from local research committee. Approval was
given by consenting the patient.
Consent
We obtained written informed consent from subject of this
study.
Author contribution
Davide Nasi: Author,
Lucia di Somma: Co-author,
Maurizio Iacoangeli: Co-author–literature review,
Valentina Liverotti: Data collection,
Open Access
This article is published Open Access at sciencedirect.com. It is distributed under the IJSCR Supplemental terms and conditions, which
permits unrestricted non commercial use, distribution, and reproduction in any medium, provided the original authors and source are
credited.
Antonio Zizzi: Pahtological findings collection and interpreta-
tion,
Mauro Dobran: Discussion of the case,
Maurizio Gladi: Discussion of the case,
Massimo Scerrati: Data analysis or interpretation–literature
review.
Guarantor
Davide Nasi and Maurizio Iacoangeli.
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