Hemangioma of The Cavernous Sinus: A Case Series

Download as pdf or txt
Download as pdf or txt
You are on page 1of 5

THIEME

e26 Case Report

Hemangioma of the Cavernous Sinus:


A Case Series
Dylan A. Noblett1 Jennifer Chang1 Atrin Toussi2 Arthur Dublin1 Kiarash Shahlaie2

1 Departments of Radiology, University of California, Davis Medical Address for correspondence Kiarash Shahlaie, MD, PhD, Department
Center, Sacramento, California, United States of Neurological Surgery, University of California, Davis Medical
2 Departments of Neurological Surgery, University of California, Center, 4860 Y Street, Suite 3740, Sacramento, CA 95817,
Davis Medical Center, Sacramento, California, United States United States (e-mail: krshahlaie@ucdavis.edu).

J Neurol Surg Rep 2018;79:e26–e30.

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
Abstract Introduction Cavernous sinus hemangiomas (CSHs) are rare, vascular, extra-axial
tumors that are diagnosed with a combination of imaging and biopsy. We describe the
clinical presentations, imaging findings, and management of two male patients with
CSHs.
Case Report Case 1 describes a 57-year-old man who presented with vision changes
and cranial nerve palsies. Initial imaging and surgical biopsy were nondiagnostic.
Follow-up Tc-99m tagged red blood cell (RBC) imaging supported CSH diagnosis. He
was treated with surgical resection and radiotherapy.
Case 2 describes a 57-year-old man who presented with chronic headache. Imaging
findings were suggestive of CSH. He underwent endoscopic endonasal surgical
resection and a final diagnosis of CSH was made via biopsy.
Keywords Discussion CSHs often present with headache, vision changes, and cranial nerve
► hemangioma palsies. Characteristic findings of a T2 hyperintense lesion with homogeneous contrast
► cavernous sinus enhancement has been described in the literature. There is also a role for tagged RBC
► radiosurgery imaging studies in the setting of nondiagnostic imaging and biopsy. Surgical resection
► nuclear medicine can be difficult due to tumor vascularity and encasement of internal carotid arteries.
► neuroradiology Stereotactic radiosurgery and adjuvant radiotherapy can play a role in the treatment of
► neuroimaging patients who have inoperable lesions or subtotal resections.

Introduction
Case Reports
Cavernous sinus hemangiomas (CSHs) are rare, benign extra-
Case 1
axial tumors, primarily affecting middle-aged women.1
Symptoms vary, but are typically due to mass effect and Clinical Presentation
include headache, vision changes, and cranial nerve palsies. CJ is a 57-year-old man who first presented to ophthalmology
In this case series, we describe the clinical presentation, after noticing a progressive worsening and blurriness of vision
imaging findings, and management of two male patients in his left eye over the last 7 months. Neurological examination
with CSHs encasing the cavernous segment of the internal revealed mild anisocoria, left-sided ptosis, left-sided lid lag,
carotid artery (ICA), making surgical resection challenging. diplopia with upward gaze, and hypesthesia in the distribution
Additionally, we report the use of Tc-99m tagged red blood of the trigeminal nerve. A detailed eye exam demonstrated an
cell (RBC) imaging to assist with diagnosis of cavernous inferior altitudinal visual field defect and a relative afferent
hemangioma in the setting of nondiagnostic surgical biopsy. pupillary defect in the left eye, concerning for ischemic optic

received DOI https://doi.org/ © 2018 Georg Thieme Verlag KG


October 5, 2017 10.1055/s-0038-1641731. Stuttgart · New York
accepted ISSN 2193-6358.
March 2, 2018
Cavernous Sinus Hemangioma Noblett et al. e27

Computed tomography (CT) angiogram (►Fig. 2C)


demonstrated small, scattered arterially enhancing compo-
nents laterally and centrally within the mass. The mass
encased the left cavernous ICA without occlusion. The infer-
ior medial aspect of the mass abutted the inferior right
cavernous ICA, causing mild narrowing.

Management
The patient underwent a left frontotemporal craniotomy
approach for surgical debulking of his mass. After elevating
the bone flap, a high-speed drill was used to resect the lateral
sphenoid wing and remove the anterior clinoid process in an
extradural fashion. The lesion was then identified at the orbital
fissure, extending toward the orbital apex and cavernous sinus.
Fig. 1 CJ. Magnetic resonance imaging (MRI) of the brain with axial T1

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
postcontrast (A) and coronal T2 (B) weighted images of the sellar region The mass was entered along its lateral margin, and found to be
demonstrates heterogeneous enhancement of a well-defined extra-axial extremely hemorrhagic with multiple vascular spaces. After a
mass centered in the left cavernous sinus with encasement of the left subtotal resection, hemostasis was achieved and the bone and
internal carotid artery flow void (red arrow), which remained patent. scalp flap were then closed. The patient reported improved
visual acuity and resolution of diplopia immediately after
neuropathy. The patient had no history of diabetes, but was a surgery. He had an uneventful postoperative course, and was
smoker of 10 years with hypertension managed with amlodi- discharged home on postoperative day 2.
pine and quinapril. Pathology results from the surgical specimen
revealed minute fragments of fibrovascular, focally cellular
Imaging Studies tissue. Immunohistochemistry was noncontributory. A defi-
Magnetic resonance imaging (MRI) scan of the brain (►Fig. 1) nitive diagnosis was not obtained, although a vascular lesion
demonstrated an extra-axial left parasellar mass measuring was favored.
35  41  24 mm. The lesion was iso- to hyperintense on T2- After the patient had recovered from his operation, a
weighted imaging with heterogeneous enhancement after cerebral angiogram was obtained to further explore a vascular
contrast administration. The mass extended medially to etiology. This study revealed abnormal enhancement arterial
involve the left cavernous sinus and encased the left para- flow from the proximal branches of the left ophthalmic,
clinoid ICA; however, there was preservation of the ICA flow recurrent meningeal, and left middle cerebral artery to supply
void, and vessel caliber was normal. The mass displaced the the mass. There was no vascular contribution from external
pituitary gland without invasion. Superiorly, there was some carotid artery and its branches. No arteriovenous shunting was
compression of the temporal lobe, without T2 hyperintensity noted. Findings were inconsistent with meningioma or hema-
to suggest underlying parenchymal edema or subpial exten- giopericytoma, and suggested the possibility of a CSH. The
sion. Anteriorly, the mass surrounded the orbital apex and patient then underwent nuclear medicine scintigraphy, with
extended through the inferior orbital fissure, compressing administration of 20.3mCi Tc-99m RBC (►Fig. 3A). Also known
the prechiasmatic optic nerve. as a tagged RBC scan, this study is generally used to detect

Fig. 2 CJ. Noncontrast computed tomography (CT) in bone (A) and brain (B) algorithm demonstrates a hyperdense mass centered in the left
cavernous sinus extending into left orbital apex with smooth bony remodeling of the adjacent sphenoid sinus wall rather than destruction. CT
angiogram (C) shows scattered arterially enhancing components within the periphery (red arrow).

Journal of Neurological Surgery Reports Vol. 79 No. R2/2018


e28 Cavernous Sinus Hemangioma Noblett et al.

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
Fig. 3 CJ. Nuclear medicine tagged red blood cell scan. Delayed static images of the head after the administration of Tc-99m RBC (A) with
anterior, posterior, and lateral views of the head demonstrate increased uptake within the left cavernous sinus mass. Single photon emission
computed tomography (SPECT) (B) images of the head show avid increased uptake within the left cavernous sinus mass.

lower gastrointestinal (GI) bleeding; however, because ery- decompressing the cavernous sinus by resecting tissue med-
throcytes are labeled with technetium-99m, lesions such as ial to the ICA, the defect was repaired with a pedicled
hemangiomas in the liver can also be confirmed, with nasoseptal flap. The patient had an uneventful postoperative
increased activity on delayed blood pool images. Scintigraphy course, and was discharged home on postoperative day 1.
in addition to single photon emission computed tomography Pathology results from the surgical specimen revealed
(SPECT) localization (►Fig. 3B) revealed increased Tc-99m RBC fibrous tissue with numerous vascular channels, some con-
activity within the known left cavernous sinus mass, highly taining organized thrombi. Differential diagnosis included
suggestive of a hemangioma. angiomatous meningioma but the lack of epithelial mem-
The patient went on to receive radiation therapy in the brane antigen (EMA) expression argued against this diag-
form of 46.8 Gy delivered over 26 fractions. Six months after nosis. A final diagnosis was made of left CSH.
radiation therapy, his vision has significantly improved and The patient initially refused adjuvant radiotherapy. He
he has been able to return to work. underwent surveillance imaging at 3, 6, and 12 months after
surgery, during which time he remained asymptomatic.
Approximately 18 months after surgery, repeat MRI demon-
Case 2
strated slight evidence of radiographic progression and the
Clinical Presentation patient reported recent onset of mild facial dysesthesia.
MB is a 57-year-old man who presented with a 6-year history Physical examination at that time demonstrated mild ptosis
of slowly progressive headache. Noncontrast head CT scan with no diplopia or disconjugate gaze.
(►Fig. 4) demonstrated a 20-mm hyperdense mass within
the left cavernous sinus that extended into the sella turcica
Discussion
and Meckel’s cave, with mild mass effect on the medial
temporal lobe. MRI scan (►Fig. 5) demonstrated heteroge- Cavernous Sinus Hemangioma
neous peripheral enhancement and a central nonenhancing CSHs are rare extra-axial intracranial tumors. They account
region. There was mass effect on the pituitary gland and for 3% of benign cavernous sinus masses and are more
stalk, and complete encasement of the cavernous segment of common in middle-aged women.1 The masses are typically
the ICA. slow-growing collections of thin-walled vascular channels
that can exert mass effect on adjacent neurovascular struc-
Management tures. Pathologically, these masses can be divided into three
The patient underwent an endoscopic endonasal transsphe- major classifications: sponge-like, mulberry-like, and
noidal approach to the sella turcica and cavernous sinus. mixed.2 Sponge-like CSH typically possess an intact pseudo-
Intraoperative navigation and Doppler ultrasonography capsule and demonstrate homogenous contrast enhance-
were used to identify the location of the ICA. The medial ment on MRI. In contrast, mulberry-like and mixed CSH
compartment of the cavernous sinus was opened, where a have an incomplete or absent pseudocapsule and demon-
highly vascular, fibrotic tumor was encountered. The lesion strate heterogeneous contrast enhancement patterns.2
was contained within the cavernous sinus, with compres- Unlike intra-axial cavernous hemangiomas, extra-axial
sion, but no invasion, of the adjacent pituitary gland. After CSH do not typically cause brain hemorrhage.3

Journal of Neurological Surgery Reports Vol. 79 No. R2/2018


Cavernous Sinus Hemangioma Noblett et al. e29

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
Fig. 4 MB. Noncontrast computed tomography (CT) in bone (A) and brain (B) algorithm demonstrates hyperdense mass centered in the left
cavernous sinus with extension into the sella turcica and Meckel’s cave with mild mass effect on the medial temporal lobe.

Diagnostic Workup vascular malformation.3 CSHs generally appear more hyper-


Hemangiomas only rarely occur in the cavernous sinus, intense than meningiomas and pituitary tumors, and the
where the differential diagnosis includes various neoplastic, heterogeneous enhancement pattern of hemangiomas can
infectious, inflammatory, and vascular etiologies.4,5 More differentiate them from the intense enhancement seen with
common neoplastic lesions of the cavernous sinus are schwannomas. Some studies have observed a hyperintense
meningiomas and schwannomas, with chordomas, metasta- peripheral enhancement leading to a central hyperintensity
sis, lymphomas, and cavernous hemangiomas as rare possi- pattern with timed contrast administration that is classically
bilities. Common infectious and inflammatory etiologies seen in hepatic cavernous hemangiomas.6
include abscess, sarcoidosis, and Tolosa–Hunt syndrome.4 Since the first patient in our study did not have a diag-
Vascular etiologies are typically cerebral aneurysms. Since nostic biopsy, a Tc-99m RBC nuclear medicine study was
the symptoms of cavernous sinus lesions are primarily due to obtained to further characterize the lesion and ultimately
mass effect impeding venous outflow and/or compressing make the diagnosis. We observed a dramatic increase in
cranial nerves, they are typically not specific and do not tracer accumulation in the left parasellar region, which is
significantly focus the differential diagnosis. consistent with previous case reports utilizing this technique
MRI plays an important role in the workup of patients with to diagnose hemangioma.7–9 It has been suggested that this
CSH. These lesions typically demonstrate hypo- or isointensity tracer accumulation, which labels erythrocytes, is specific to
on T1-weighted images and hyperintensity on T2-weighted CSH and is not noted in patients found to have other extra-
images; these imaging findings alone lend a differential diag- axial masses in this region, including meningiomas, schwan-
nosis of meningioma, schwannoma, pituitary adenoma, and nomas, chordomas, or chondrosarcomas.7,9 This practice of
CSH. MRI of CSH generally reveals T2 hyperintensity with utilizing tagged RBC nuclear medicine studies may be useful
some intratumor linear hypointenisty that is generally washed in future cases where the diagnosis of CSH is unclear and the
out by homogenous enhancement when intravenous contrast physician requires a diagnosis for surgical planning or defi-
is used, likely representing thin walled vessels within the nitive treatment.

Fig. 5 MB. Axial T1 postcontrast (A) and coronal T1 postcontrast (B), images demonstrates heterogeneous peripheral enhancement with central
nonenhancing portions of a left cavernous sinus mass with mass effect on the pituitary stalk (red arrow). Coronal T2 (C) images show a T2
hyperintense mass encasing the left internal carotid artery (ICA) flow void and axial magnetic resonance angiogram (MRA) (D) confirms normal
flow related enhancement within the preserved ICA.

Journal of Neurological Surgery Reports Vol. 79 No. R2/2018


e30 Cavernous Sinus Hemangioma Noblett et al.

Management Conflict of Interest


Surgical resection is the mainstay of treatment for heman- None.
gioma, reducing the likelihood of disease recurrence drama-
tically. When located in the cavernous sinus, however, the Acknowledgments
decision to perform complete surgical resection has to be The authors did not receive any financial support for the
carefully weighed against the risks of neurovascular injury, work in this article.
where up to 40% of tumors exhibit severe intraoperative
bleeding.10 In one report, of 20 cavernous sinus cases, 7 were
operated on using an intradural approach and within those 7,
References
one died of intracranial hemorrhage postoperatively.11 The
1 Linskey ME, Sekhar LN. Cavernous sinus hemangiomas: a series, a
most common postoperative complication is a transient review, and an hypothesis. Neurosurgery 1992;30(01):101–108
ophthalmoplegia that develops in up to 40 to 86% of 2 Yao Z, Feng X, Chen X, Zee C. Magnetic resonance imaging
patients.10,12 Rarely, permanent abducens nerve palsies characteristics with pathological correlation of cavernous mal-
can occur, with the incidence being 14% as reported in Suri formation in cavernous sinus. J Comput Assist Tomogr 2006;30

This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
et al’s recent study.12 Morbidity and mortality after caver- (06):975–979
3 Shi J, Hang C, Pan Y, Liu C, Zhang Z. Cavernous hemangiomas in the
nous sinus surgery has generally been reported to be 38% in
cavernous sinus. Neurosurgery 1999;45(06):1308–1313, discus-
older reports and as high as 12.5% in more recent reports.13 sion 1313–1314
As a result, many patients with cavernous sinus lesions may 4 Bag AK, Shah R. AJR teaching file: cavernous sinus mass in a woman
undergo subtotal resection followed by adjuvant therapies. presenting with painful ophthalmoplegia. AJR Am J Roentgenol
Stereotactic radiosurgery is a treatment option for patients 2010;195(3, Suppl):WS1–WS4
5 Razek AA, Castillo M. Imaging lesions of the cavernous sinus. AJNR
with inoperable lesions or those that undergo limited subtotal
Am J Neuroradiol 2009;30(03):444–452
resection. Gamma Knife radiosurgery (GKS) has been shown to 6 Anqi X, Zhang S, Jiahe X, Chao Y. Cavernous sinus cavernous
significantly reduce tumor size and volume, as well as to hemangioma: imaging features and therapeutic effect of Gamma
relieve some of the neurological symptoms associated with Knife radiosurgery. Clin Neurol Neurosurg 2014;127:59–64
CSH.13 It is hypothesized that radiotherapy reduces lesion size 7 Salanitri GC, Stuckey SL, Murphy M. Extracerebral cavernous
by inducing endothelial proliferation, hyalinization of vessel hemangioma of the cavernous sinus: diagnosis with MR imaging
and labeled red cell blood pool scintigraphy. AJNR Am J Neuror-
walls, and cytotoxic effects of radiation on microvasculature
adiol 2004;25(02):280–284
and tumor cells themselves. Tang et al’s 2015 study reported a 8 Kawai K, Fukui M, Tanaka A, Kuramoto S, Kitamura K. Extracer-
79% mean reduction in tumor volume in 53 patients treated ebral cavernous hemangioma of the middle fossa. Surg Neurol
with GKS during a 24.5-month follow-up period. Likewise, no 1978;9(01):19–25
tumor showed enlargement after treatment.14 9 Sayit E, Durak I, Capakaya G, Yilmaz M, Durak H. The role of Tc-
99m RBC scintigraphy in the differential diagnosis of orbital
cavernous hemangioma. Ann Nucl Med 2001;15(02):149–151
Conclusion 10 Yin YH, Yu XG, Xu BN, Zhou DB, Bu B, Chen XL. Surgical manage-
ment of large and giant cavernous sinus hemangiomas. J Clin
CSHs are rare extra-axial benign tumors that often present with Neurosci 2013;20(01):128–133
headache, diplopia, proptosis, or extraocular nerve palsies. MRI 11 Zhou LF, Mao Y, Chen L. Diagnosis and surgical treatment of
often demonstrates a T2 hyperintense lesion with homoge- cavernous sinus hemangiomas: an experience of 20 cases. Surg
Neurol 2003;60(01):31–36, discussion 36–37
neous contrast enhancement with administration of contrast.
12 Suri A, Ahmad FU, Mahapatra AK. Extradural transcavernous
Although imaging characteristics are often highly suggestive, in
approach to cavernous sinus hemangiomas. Neurosurgery
select cases Tc-99m tagged RBC nuclear medicine studies can be 2007;60(03):483–488, discussion 488–489
helpful in securing the diagnosis. Management of these lesions 13 Bansal S, Suri A, Singh M, et al. Cavernous sinus hemangioma: a
is directed by clinical presentation and tumor size, as aggressive fourteen year single institution experience. J Clin Neurosci 2014;
surgical resection can have significant morbidity and is not 21(06):968–974
14 Tang X, Wu H, Wang B, et al. A new classification and clinical
indicated in patients with no significant cranial neuropathy.
results of Gamma Knife radiosurgery for cavernous sinus heman-
Radiation therapy plays a critical role in the management of giomas: a report of 53 cases. Acta Neurochir (Wien) 2015;157
these tumors, with excellent long-term control rates. (06):961–969, discussion 969

Journal of Neurological Surgery Reports Vol. 79 No. R2/2018

You might also like

pFad - Phonifier reborn

Pfad - The Proxy pFad of © 2024 Garber Painting. All rights reserved.

Note: This service is not intended for secure transactions such as banking, social media, email, or purchasing. Use at your own risk. We assume no liability whatsoever for broken pages.


Alternative Proxies:

Alternative Proxy

pFad Proxy

pFad v3 Proxy

pFad v4 Proxy