Open Access

Cavernous hemangioma of the third ventricle: a case report and review of the literature

  • Moon-Soo Han1,
  • Kyung-Sub Moon1Email author,
  • Kyung-Hwa Lee2,
  • Seul-Kee Kim3 and
  • Shin Jung1
World Journal of Surgical Oncology201412:237

https://doi.org/10.1186/1477-7819-12-237

Received: 28 May 2014

Accepted: 20 July 2014

Published: 29 July 2014

Abstract

Background

Although cavernous hemangiomas (CHs) can be found anywhere in the central nervous system, CHs of the third ventricle have been reported in only 29 patients (including our case). In the current case report, we discuss the clinical characteristics and surgical outcome of CHs of the third ventricle.

Case presentation

A 64-year-old female was admitted to our emergency room with a sudden decreased level of consciousness. Brain imaging studies demonstrated a multi-lobulated hemorrhagic mass in the third ventricle. The lesion was removed via the transcallosal-interforniceal approach and pathologically diagnosed as CH. Postoperatively, the patient had a transient neurological deficit due to hypothalamic injury and recovered to the normal status at 2 months after the operation. In the review of 29 cases, the mean age of the patients was 40 years with a slight female preponderance (female/male, 17/12). The majority of the patients complained of a mass effect with signs of increased intracranial pressure; only one case was asymptomatic. Gross total resection was achieved in 81% of the cases. Around 80% of the patients were asymptomatic or improved from the initial symptoms. Mortality rate was 6.9% and the most common complication was hydrocephalus.

Conclusions

As demonstrated in the review of the previous reports, the outcome is favorable after surgical excision for CH of the third ventricle. Hence, surgical excision appears to be the treatment of choice for CH located in the third ventricle, which tends to grow rapidly resulting in a mass effect.

Keywords

Cavernous hemangioma Complication Outcome Surgery Third ventricle

Background

Cavernous hemangiomas (cavernomas, cavernous angiomas, cavernous malformations; CH) are vascular hamartomas that are reported to be found at any location in the central nervous system (CNS). Due to the increased use of computerized tomography (CT) scan and magnetic resonance imaging (MRI), more CHs have been diagnosed in recent years. However, intraventricular location of CHs is uncommon, and the incidence of intraventricular CHs has been reported to be only about 2.5 to 10.8% of all intracranial CHs[1, 2]. The most frequent location of intraventricular CHs is the lateral ventricle and involvement of the third ventricle is quite rare. Based on the review of Medline database (PubMed, http://www.ncbi.nlm.nih.gov/PubMed), only 29 cases (including our case) of CH of the third ventricle have been reported[320].

We present the case of a patient who had a CH in the third ventricle that was resected through the transcallosal interforniceal approach. In addition, we also review the previously reported cases and discuss their clinical characteristics and surgical outcomes.

Case presentation

A 64-year-old female was admitted to our emergency room with a sudden decreased level of consciousness. Except for an intermittent and mild degree headache, there was no specific history of head trauma and medical illness. On neurological examination, she showed a drowsy mentality with Glasgow Coma Scale score of 14/15 and the right homonymous hemianopsia. She did not have motor/sensory and cranial nerve deficits, and cerebellar signs. There were no abnormal laboratory findings. Non-contrasted CT scan showed a heterogeneously hyperattenuated hemorrhagic mass within the third ventricle compressing the hypothalamus, without definitive hydrocephalus (Figure 1). Brain MRI revealed a 40 × 30 × 28 mm sized multi-lobulated mass with a recent hemorrhage in the third ventricle, which extended to the foramen of Monro and hypothalamus. There was no definite contrast enhancement (Figure 2).

Right-side interhemispheric, transcallosal interforniceal approach was used for removal of the lesion. At surgery, the lesion was found to be a red colored, multi-lobulated mass, which had numerous vascular channels and multi-staged hemorrhage. Although there were severe adhesions between the base of the lesion and the basilar arterial system, gross total removal of the lesion was possible due to the presence of the discrete sticky hemosiderin rim, which allowed differentiation of the lesion from the surrounded normal parenchyma (Figure 3). To prevent hypothalamic injury, the resection of hemosiderin-stained tissue was restricted to the minimum.

Histopathological examination of the lesion revealed a CH composed of large, irregularly dilated, blood-filled vascular channels lined by flat endothelium (Figure 4). Postoperatively, the patient developed transient diabetes insipidus, somnolence, and general weakness due to hypothalamic injury, but these symptoms gradually disappeared with conservative treatment. Finally, she recovered to the normal status at 2 months after the operation.
Figure 1

Axial non-contrast CT images show a large and heterogeneously hyperattenuated hemorrhagic mass in the suprasellar area, with dilatation of the anterior part of the third ventricle (arrow).

Figure 2

Preoperative MRI images. Sagittal T1-weighted (A) and post-contrast T1-weighted (B) images show a non-enhancing hemorrhagic mass in the anterior third ventricle and hypothalamic area. Axial T1-weighted (C), T2-weighted (D), and susceptibility weighted (E) images demonstrate a typical cavernous malformation with heterogeneous signal intensity and hemosiderin rim indicating mixed acute or subacute stage hemorrhage.

Figure 3

Intraoperative photographs through transcallosal interforniceal approach. (A) After dissection of the corpus callosum, the interforniceal plane (asterisk) was observed between the bilateral septum pallucidum; (B) After entering the third ventricle via the interforniceal approach, a red-colored and multi-lobulated cavernous hemangioma with numerous vascular channels and different-staged hemorrhage was encountered; (C and D) Removal of the lesion revealed the patency of the Sylvian aqueduct (asterisk) over the massa intermedia and the basilar system in the base of the lesion.

Figure 4

Pathologic findings of the cavernous hemangioma. The microphotograph displayed a blood-filled lesion composed of irregularly dilated channels and a slightly fibrotic capsule surrounding the lesion (hematoxylin and eosin, original magnification × 100).

Discussion

CHs are vascular hamartomas which are reported to be found anywhere in the CNS. However, intraventricular CHs are rare and their incidence was reported to be only about 2.5 to 10.8% of all cerebral cavernous malformations[1, 2]. CHs may be diagnosed based on symptoms of acute hemorrhage, seizures, or progressive neurologic deficits. Chadduck et al.[21] reported that there was no difference between the clinical symptoms and signs of intraventricular CHs and parenchymal CHs. However, because of the rarity, the natural history and clinical features of CHs located in the third ventricle have not been fully investigated and there are no definite recommendations for its management.

Overall, 29 patients with a well-described CH in the third ventricle have been reported in the literature, including our case[320] (Table 1), with a slight female preponderance (female/male ratio, 58/42%). The median age of the patients was 40 years (range, 8–64 years) and 6 patients were of the pediatric age group (21%). The most frequent initial clinical symptoms included a mass effect, with signs of increased intracranial pressure (headache, nausea, vomiting, visual disturbance, memory impairment and signs of hypothalamic dysfunction) in 23 patients (79%). Intraventricular hemorrhage from the lesion occurred in 3 cases (10%) and seizures in 2 cases (7%); only one patient was asymptomatic. This higher incidence of mass effect symptoms may be because of the direct compression of the surrounding structures, due to CH growth. Katayama et al.[5] stated that intraventricular CHs tend to grow rapidly resulting in giant malformation, because of low mechanical resistance caused by lack of the surrounding brain tissue and repeated hemorrhage in the CH. In the literature, the mean size of the lesions was reported to be 23 mm (range, 12–40 mm). Although intralesional bleeding can frequently occur when CHs grow within the ventricle, bleeding from a CH into the ventricular system is rare as per the previous reports[10].
Table 1

Summarized surgically resected cavernous hemangioma of the third ventricle

Authors

Publication year

Age (year)

Sex

Symptom

Size (cm)

Approach

Extent of resection

Outcome

Postoperative complication

Vaquero et al.[3]

1980

18

F

Diplopia

TC

GTR

Improved

 

Pozzati et al.[4]

1980

31

F

Headache, vomiting

TV

GTR

Improved

 

Lavyne et al.[5]

1983

48

F

Headache, memory impairment

1.5

TC + TV + SC

PR

Not improved

HDC, IVH

Amagasa et al.[6]

1984

40

M

Homonymous hemianopsia, endocrine function deficit

IH + TLT

GTR

Improved

 

Harbaugh et al.[7]

1984

44

F

Headache, vomiting, IVH

2

TC + TV

GTR

Improved

HDC

Yamasaki et al.[8]

1986

9

M

Headache

2.5

GTR

Improved

 
  

15

F

Lower temporal quadrantopsia

1.5

PR

No symptom

 
  

36

M

Headache, vomiting, mental change

2.5

PR

Improved

 

Voci et al.[9]

1989

19

F

IVH

TC

GTR

Improved

 

Ogawa et al.[10]

1990

16

M

Headache, nausea

2

IH + TLT

GTR

No symptom

 
  

40

M

Homonymous hemianopsia, endocrine function deficit

2

IH + TLT

GTR

Improved

 

Katayama et al.[11]

1994

9

F

Seizure

IH + TLT

PR

Death

 
  

50

F

Improved

 
  

45

F

IVH

Not improved

Vegetative state

  

49

M

Visual field defect, endocrine function deficit

2

Improved

 
  

47

F

Memory impairment

3

SC + TVI

GTR

Improved

Transient DI, Recurrence

Sinson et al.[12]

1995

43

F

Headache, memory impairment

3

IH + TC + IF

GTR

Death

 
  

36

F

Memory impairment, weight gain

3

IH + TC + IF

GTR

Not improved

HDC

  

52

F

Headache, nausea

3.5

TCo

GTR

Improved

 
  

32

F

Headache, vomiting, diplopia

2

IFT + SCbll

GTR

Improved

 

Reyns et al.[13]

1999

42

M

Seizure

2.5

TCo + TVI

PR

Improved

Recurrence

Crivell et al.[14]

2002

38

M

Memory impairment, gait disturbance, headache, vomiting

TCo + TVI

GTR

Improved

 

Wang et al.[15]

2003

62

F

Gait disturbance

TCo + TV

GTR

Not improved

ICH on thalamus, CNS infection

Milenkovic et al.[16]

2005

56

M

Headache, memory impairment, bizarre behavior

TC + TV + TF

GTR

Improved

 

Darwish et al.[17]

2005

47

F

No symptom

1.5

TC + TV + TF

GTR

No symptom

HDC

Longatti et al.[18]

2006

35

M

Headache, vomiting, neck pain

1.2

TV

GTR

Improved

 

Zakaria et al.[19]

2006

8

M

Headache, vomiting, gait disturbance

TC

GTR

Improved

 

Kivelev et al.[20]

2010

52

M

Headache, vomiting

TC + IF(?)

GTR

Improved

 

Present study

2012

64

F

Mental change, homonymous hemianopsia

4

TC + IF

GTR

Improved

Transient DI & hypothalamic injury symptoms*

*; totally resolved at 2 months after the operation.

–, not available; DI, Diabetes insipidus; F, Female; GTR, Gross total resection; HDC, Hydrocephalus; ICH, Intracerebral hemorrhage; IF, Interforniceal; IH, Interhemisphric; IFT, Infratentorial; IVH, Intraventricular hemorrhage; M, Male; PR, partial resection; SC, Subchoroidal; SCbll, Supracerebellar; TC, Transcallosal; TCo, Transcortical; TF, Transforaminal; TLT, Translamina terminalis; TV, Transventricular; TVI, transvelum interpositum.

The radiological findings of the intraventricular CHs do not differ from those of the intraparenchymal type[10]. Generally, on CT scans, the CH is suggested by the presence of a high density area, absence of perilesional edema, and mild or no contrast enhancement because of blood pool effects, calcification, and recent hemorrhage[22]. On MRI images, the CHs usually have mixed signal intensities. High signal intensities correlate with the presence of methemoglobin and low signal intensities correlate with calcifications and fibrosis within the lesion on T1- and T2-weighted images. A peripheral rim of low signal intensity correlates with the paramagnetic effect of hemosiderin[23].

A conservative treatment is appropriate for an asymptomatic CH located in the supratentorial parenchyma. However, CHs located in the third ventricle, surrounded by vital structures, are especially dangerous. It has also been documented that these lesions show a rapid growth[5], resulting in significant morbidity. For these reasons, the third ventricular CH needs to be treated more aggressively. As shown in Table 1, 80% of the patients were asymptomatic or improved from their initial symptoms after the surgical procedure. The most frequent post-operative complication was a hydrocephalus, observed in four patients. Postoperative mortality was 6.9% (2/29). The important point to be noted, as illustrated by our case, is that large-sized lesions frequently involve the hypothalamus[6, 10, 11]. Therefore, careful dissection of the lesion should be performed to prevent damage to the hypothalamus. To reduce this complication, minimizing the resection of hemosiderin-stained tissue and preservation of associated developmental venous anomalies are the key points, as in surgery for CHs located in the brain stem or cranial nerves[24, 25]. Furthermore, during the operation for CHs buried in the parenchyma with a critical neurological function, initial dissection and removal of the lesion should be attempted on the short trajectory after observation of the surface changes caused by the hemorrhage[24, 26]. Considering these principles, transcallosal-interforniceal approach can provide a direct, short corridor to the third ventricle with wide exposure of the lesion.

Conclusions

Surgical excision appears to be the treatment of choice for CHs located in the third ventricle, which tend to grow rapidly and cause a mass effect. Using the short corridor to the third ventricle, obtaining wide exposure of the lesion, and minimizing resection of the surrounding hemosiderin-stained tissue can lead to a favorable surgical outcome, as demonstrated in the previous reports, including this report.

Consent

Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Abbreviations

CH: 

Cavernous hemangioma

CNS: 

Central nervous system

CT: 

Computed tomography

MRI: 

Magnetic resonance imaging.

Declarations

Authors’ Affiliations

(1)
Department of Nueurosurgery, Chonnam National University Research Institute of Medical Science, Chonnam National University Hwasun Hospital & Medical School
(2)
Department of Pathology, Chonnam National University Research Institute of Medical Sciences, Chonnam National University Hwasun Hospital & Medical School
(3)
Department of Radiology, Chonnam National University Research Institute of Medical Sciences, Chonnam National University Hwasun Hospital & Medical School

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Copyright

© Han et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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