diagnosis and treatment of cavernous hemangioma of the … · diagnosis and treatment of cavernous...

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J Neurosurg Volume 124 • March 2016 639 CLINICAL ARTICLE J Neurosurg 124:639–646, 2016 C AVERNOUS hemangiomas are rare vascular malfor- mations of the central nervous system 26 that seldom occur in the internal auditory canal (IAC); only 50 cases have been reported. 1–5,7–25,29–40 Cavernous heman- giomas of the IAC are thought to arise from the capillary plexus of the epineurium surrounding Scarpa’s ganglion, 5 causing symptoms of intraneural vascular infiltration such as sensorineural hearing loss, tinnitus, dizziness, and facial nerve symptoms. Neuroimaging may show a small tumor in the IAC. Although the clinical and radiological features of cavernous hemangiomas of the IAC can make it difficult to distinguish them from schwannomas arising from cra- nial nerves VII or VIII, preoperative recognition of IAC tu- mors is possible if the specific radiological findings and the relatively severe symptoms of cranial nerve dysfunction are considered in relation to the small tumor size. Early surgical intervention may increase the chance of improved facial nerve function. We describe the symptomatology, ABBREVIATIONS CPA = cerebellopontine angle; HB = House-Brackmann; IAC = internal auditory canal; VS = vestibular schwannoma. SUBMITTED December 8, 2014. ACCEPTED March 27, 2015. INCLUDE WHEN CITING Published online September 25, 2015; DOI: 10.3171/2015.3.JNS142785. *Drs. Zhu, Huang, and Li contributed equally to this article. Diagnosis and treatment of cavernous hemangioma of the internal auditory canal *Wei Dong Zhu, MD, 1–3 Qi Huang, MD, 1–3 Xi Ye Li, MD, 1–3 Hong Sai Chen, MD, PhD, 1–3 Zhao Yan Wang, MD, PhD, 1–3 and Hao Wu, MD, PhD 1–3 1 Department of Otolaryngology–Head and Neck Surgery, Xinhua Hospital; 2 Ear Institute; and 3 Shanghai Key Laboratory of Translational Medicine on Ear and Nose Diseases, Shanghai Jiaotong University, School of Medicine, Shanghai, People’s Republic of China OBJECTIVE Cavernous hemangioma of the internal auditory canal (IAC) is an extremely rare type of tumor, and only 50 cases have been reported in the literature prior to this study. The aim in this study was to describe the symptomatol- ogy, radiological features, and surgical outcomes for patients with cavernous hemangioma of the IAC and to discuss the diagnostic criteria and treatment strategy for the disease. METHODS The study included 6 patients with cavernous hemangioma of the IAC. All patients presented with senso- rineural hearing loss and tinnitus, and 2 also suffered from vertigo. Five patients reported a history of facial symptoms with hemispasm or palsy: 3 had progressive facial weakness, 1 had a hemispasm, and 1 had a history of recovery from sudden facial paresis. All patients underwent CT and MRI to rule out intracanalicular vestibular schwannomas and facial nerve neuromas. Five patients had their tumors surgically removed, while 1 patient, who did not have facial problems, was followed up with a wait-and-scan approach. RESULTS All patients had a presurgical diagnosis of cavernous hemangioma of the IAC, which was confirmed patho- logically in the 5 patients who underwent surgical removal of the tumor. The translabyrinthine approach was used to remove the tumor in 4 patients, while the middle cranial fossa approach was used in the 1 patient who still had functional hearing. Tumors adhered to cranial nerves VII and/or VIII and were difficult to dissect from nerve sheaths during sur- geries. Complete hearing loss occurred in all 5 patients. In 3 patients, the facial nerve could not be separated from the tumor, and primary end-to-end anastomosis was performed. Intact facial nerve preservation was achieved in 2 patients. Patients were followed up for at least 1 year after treatment, and MRI showed no evidence of tumor regrowth. All patients experienced some level of recovery in facial nerve function. CONCLUSIONS Cavernous hemangioma of the IAC can be diagnosed preoperatively through analysis of clinical fea- tures and neuroimaging. Early surgical intervention may preserve the functional integrity of the facial nerve and provide a better outcome after nerve reconstruction. However, preservation of functional hearing may not be achieved, even with the retrosigmoid or middle cranial fossa approaches. The translabyrinthine approach seems to be the most appropriate approach overall, as the facial nerve can be easily located and reconstructed. http://thejns.org/doi/abs/10.3171/2015.3.JNS142785 KEY WORDS cavernous hemangioma; internal auditory canal; hearing loss; facial nerve; skull base ©AANS, 2016 Unauthenticated | Downloaded 04/16/21 08:34 PM UTC

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Page 1: Diagnosis and treatment of cavernous hemangioma of the … · diagnosis and treatment of cavernous hemangioma of the iac Follow-up data were available in all patients, and the mean

J Neurosurg  Volume 124 • March 2016 639

cliNical articleJ Neurosurg 124:639–646, 2016

Cavernous hemangiomas are rare vascular malfor-mations of the central nervous system26 that seldom occur in the internal auditory canal (IAC); only 50

cases have been reported.1–5,7–25,29–40 Cavernous heman-giomas of the IAC are thought to arise from the capillary plexus of the epineurium surrounding Scarpa’s ganglion,5 causing symptoms of intraneural vascular infiltration such as sensorineural hearing loss, tinnitus, dizziness, and facial nerve symptoms. Neuroimaging may show a small tumor

in the IAC. Although the clinical and radiological features of cavernous hemangiomas of the IAC can make it difficult to distinguish them from schwannomas arising from cra-nial nerves VII or VIII, preoperative recognition of IAC tu-mors is possible if the specific radiological findings and the relatively severe symptoms of cranial nerve dysfunction are considered in relation to the small tumor size. Early surgical intervention may increase the chance of improved facial nerve function. We describe the symptomatology,

abbreviatioNs CPA = cerebellopontine angle; HB = House-Brackmann; IAC = internal auditory canal; VS = vestibular schwannoma. submitted December 8, 2014.  accepted March 27, 2015.iNclude wheN citiNg Published online September 25, 2015; DOI: 10.3171/2015.3.JNS142785.*Drs. Zhu, Huang, and Li contributed equally to this article.

Diagnosis and treatment of cavernous hemangioma of the internal auditory canal*wei dong Zhu, md,1–3 Qi huang, md,1–3 Xi Ye li, md,1–3 hong sai chen, md, phd,1–3 Zhao Yan wang, md, phd,1–3 and hao wu, md, phd1–3

1Department of Otolaryngology–Head and Neck Surgery, Xinhua Hospital; 2Ear Institute; and 3Shanghai Key Laboratory of Translational Medicine on Ear and Nose Diseases, Shanghai Jiaotong University, School of Medicine, Shanghai, People’s Republic of China

obJective Cavernous hemangioma of the internal auditory canal (IAC) is an extremely rare type of tumor, and only 50 cases have been reported in the literature prior to this study. The aim in this study was to describe the symptomatol-ogy, radiological features, and surgical outcomes for patients with cavernous hemangioma of the IAC and to discuss the diagnostic criteria and treatment strategy for the disease.methods The study included 6 patients with cavernous hemangioma of the IAC. All patients presented with senso-rineural hearing loss and tinnitus, and 2 also suffered from vertigo. Five patients reported a history of facial symptoms with hemispasm or palsy: 3 had progressive facial weakness, 1 had a hemispasm, and 1 had a history of recovery from sudden facial paresis. All patients underwent CT and MRI to rule out intracanalicular vestibular schwannomas and facial nerve neuromas. Five patients had their tumors surgically removed, while 1 patient, who did not have facial problems, was followed up with a wait-and-scan approach.results All patients had a presurgical diagnosis of cavernous hemangioma of the IAC, which was confirmed patho-logically in the 5 patients who underwent surgical removal of the tumor. The translabyrinthine approach was used to remove the tumor in 4 patients, while the middle cranial fossa approach was used in the 1 patient who still had functional hearing. Tumors adhered to cranial nerves VII and/or VIII and were difficult to dissect from nerve sheaths during sur-geries. Complete hearing loss occurred in all 5 patients. In 3 patients, the facial nerve could not be separated from the tumor, and primary end-to-end anastomosis was performed. Intact facial nerve preservation was achieved in 2 patients. Patients were followed up for at least 1 year after treatment, and MRI showed no evidence of tumor regrowth. All patients experienced some level of recovery in facial nerve function.coNclusioNs Cavernous hemangioma of the IAC can be diagnosed preoperatively through analysis of clinical fea-tures and neuroimaging. Early surgical intervention may preserve the functional integrity of the facial nerve and provide a better outcome after nerve reconstruction. However, preservation of functional hearing may not be achieved, even with the retrosigmoid or middle cranial fossa approaches. The translabyrinthine approach seems to be the most appropriate approach overall, as the facial nerve can be easily located and reconstructed. http://thejns.org/doi/abs/10.3171/2015.3.JNS142785KeY words cavernous hemangioma; internal auditory canal; hearing loss; facial nerve; skull base

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w. d. Zhu et al.

radiological features, and surgical outcomes of 6 patients with cavernous hemangioma of the IAC with special em-phasis on the diagnostic criteria and treatment strategy.

methodspatient data

Ethics approval for this study was given by the Ethi-cal Committee of Xinhua Hospital, and informed consent was obtained from the participants. Six patients with cav-ernous hemangioma of the IAC were treated in the De-partment of Otolaryngology–Head and Neck Surgery of the Xinhua Hospital between 2006 and 2013 in Shanghai. These patients consisted of 2 women and 4 men, with ages ranging from 23 to 40 years (mean 31.7 years).

clinical FeaturesAll patients presented with initial symptoms of pro-

gressive sensorineural hearing loss and tinnitus. Vertigo was reported by 2 patients. Five patients experienced fa-cial symptoms with a hemispasm or palsy: 3 presented with progressive facial weakness, 1 had a hemispasm, and 1 presented with a history of recovery from sudden facial paresis. Table 1 summarizes the preoperative symptoms.

radiological evaluationAll patients underwent high-resolution CT and gado-

linium-enhanced MRI. Irregular bony erosion leading to IAC enlargement and calcification were the typical signs of cavernous hemangioma on the high-resolution CT scan. MRI demonstrated a high contrast-enhancing tumor in-volving the outer one-third of the IAC.

surgical approachOne young patient without facial problems was treated

with a wait-and-scan approach. The remaining 5 patients underwent surgical removal of the tumor. The translaby-rinthine approach was performed in 4 patients who had no useful hearing (designated as Class C and D using the

American Academy of Otolaryngology–Head and Neck Surgery 1995 hearing scale). The middle fossa approach was performed in 1 patient who had a Class B preopera-tive hearing level.

Follow-upAll patients were followed up for at least 1 year after

treatment and underwent annual MRI. Facial nerve func-tion in each patient was also recorded.

resultsWith regard to localization, the lesion was usually con-

fined to the IAC, with extension to the fundus in 4 patients and into the cerebellopontine angle (CPA) in 1 patient. Radiological findings included irregular bony erosion of the IAC and typical intralesional calcification on CT scans and heterogeneous tumor enhancement with gadolinium on MR images (Fig. 1). Complete tumor removal was achieved in a single-stage procedure in all 5 patients who underwent surgical intervention. In 2 patients, the lesion could be dissected away from the cochlear/facial nerve complex. In 3 patients, the facial nerve was compressed tightly by the tumor, and sacrifice of the facial nerve was inevitable. In each of these 3 patients, the facial nerve was repaired by end-to-end anastomosis (Fig. 2). There were no postoperative complications. Histological examina-tions confirmed the diagnosis of a cavernous hemangioma infiltrating the nerves.

Postoperatively, facial nerve paresis was noted in all 5 patients; improvement was subsequently observed in all of them (House-Brackmann [HB] Grade II in 1 patient, Grade III in 2 patients, and Grade IV in 2 patients), includ-ing the 3 patients who had undergone facial nerve recon-struction. All patients were completely deaf after surgery, including the 1 patient in whom the middle fossa approach had been used. Although the continuity of the cochlear nerve was maintained in that 1 patient, it could not be functionally preserved because of its extreme adherence to the tumor (Table 1).

table 1. summary of clinical data in 6 patients with cavernous hemangioma of the iac

Case No.

Age (yrs), Sex Main Symptoms

Duration of Hearing 

Problems (yrs)Surgical  Approach

FNF (HB Grade)

Intraop FindingsPostop ResultsPreop Postop FU

1 40, M SNHL, tinnitus, recovery from sudden facial paralysis

2 Translabyrinthine I VI II Lesion adherent to CN VII; FN reconstruction

FW, deaf

2 27, M SNHL, tinnitus, vertigo, FW 2 Translabyrinthine III V III Lesion adherent to CN VII & VIII; FN anatomically intact

FW, deaf

3 31, M SNHL, tinnitus, vertigo, FW 1 Middle fossa IV IV IV Lesion compressing CN VII & VIII; FN anatomically intact

FW, deaf

4 37, F SNHL, tinnitus, recurrent facial paralysis

1 Translabyrinthine IV VI IV Lesion infiltrating CN VII & VIII; FN reconstruction

FW, deaf

5 32, F SNHL, tinnitus, hemispasm 3 Translabyrinthine I VI III Lesion infiltrating CN VII & VIII; FN reconstruction

FW, deaf

6 23, M SNHL, tinnitus 1 Wait & scan I NA I NA NA

CN = cranial nerve; FN = facial nerve; FNF = facial nerve function; FU = follow-up (1 year after surgery or diagnosis); FW = facial weakness; HB = House-Brackmann; NA = not available; SNHL = sensorineural hearing loss.

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diagnosis and treatment of cavernous hemangioma of the iac

Follow-up data were available in all patients, and the mean follow-up period was 31.3 months (range 12–96 months). In all 5 patients, no lesion recurrence was ob-served on MR images obtained during the postoperative follow-up period. One patient, who was undergoing a wait-and-scan approach, showed no enlargement of the lesion and no report of facial problems. Follow-up examination is still pending in this patient.

discussionPrevious authors have studied many of the common tu-

mors of the IAC, including vestibular schwannomas (VSs), meningiomas, primary cholesteatomas, and facial nerve neuromas. However, hemangiomas rarely occur in the IAC. These tumors can adhere to the facial, acoustic, or intermediate nerves. Cavernous hemangiomas of the IAC are extremely rare lesions, with only 50 cases reported in the literature thus far, most of which consisted of case re-ports. Furthermore, previously reported patients with IAC hemangiomas were often preoperatively misdiagnosed with intracanalicular VS or facial neuroma. In some re-ports, although cavernous hemangioma was highly sus-pected, the diagnosis could not be confirmed without a pathological result.

Cavernous hemangioma of the IAC originates from the capillary bed of the epineurium surrounding Scarpa’s ganglion and can either compress or infiltrate the nerve.21 Depending on the tumor location and the nerve of origin, these lesions can cause severe and progressive sensori-neural hearing loss and facial nerve symptoms such as hemispasm or palsy, even when the tumors are relatively small. Furthermore, the development of retrocochlear signs and symptoms seems to occur more rapidly in cav-ernous hemangioma. In our series, the duration of hearing problems ranged from 1 to 3 years (Table 1), which was probably due to the intraneural infiltrating growth manner of the lesion. Patients with cavernous hemangioma of the IAC are younger than those with VSs. In the literature and in our series, age was reported in a total of 48 patients,

ranging in age from 21 to 66 years with a median age of 38 years.

The most common clinical feature of cavernous hem-angioma is hearing loss, which occurs in almost every pa-tient. In the literature and in our series, preoperative hear-ing level was reported in a total of 54 patients (Tables 1 and 2). Hearing loss occurred in 53 patients (98.1%). Hear-ing loss is attributed to nervous infiltration. Facial nerve problems, including sudden and transient facial paralysis, recurrent facial paralysis, progressive facial paralysis, and facial spasm, were the second most common features, which occurred in 51.8% (28 of 54) of the patients previ-ously reported.

Review of radiological findings in these previously re-ported patients reveals irregular bony erosion of the IAC and typical intralesional calcification on CT scans.3,17,30 The infiltrating, intraneural growth manner of the tumor leads to early and irregular bony erosion, which is consis-tent with most previously reported cases. In some patients, CT imaging should be studied carefully by comparing the thin layer and bilateral area of the lesion. On MR images, the typical indicator of cavernous hemangioma of the IAC is heterogeneous enhancement with gadolinium, which al-lows for differentiation from schwannomas.4,20,31

The clinical and radiological features of cavernous hemangiomas of the IAC make it difficult to distinguish the lesion from the more commonly occurring VSs and facial neuromas.6 However, in cavernous hemangiomas of the facial/vestibulocochlear nerve complex, facial weak-ness and acoustic deficit are usually severe even when the lesion size is small. The signs and symptoms of retroco-chlear hearing loss seem to occur more rapidly in cavern-ous hemangiomas. Furthermore, the diagnosis can be con-

Fig. 1. Radiological findings of cavernous hemangiomas of the IAC. Typical irregular bony erosion of the IAC and intralesional calcification appear on the CT scan, and heterogeneous enhancement is shown on the MR image.  a: Axial CT scan.  b: Coronal CT scan.  c: Axial T2-weighted MR image showing irregular isointense mass located in the IAC.  d: Axial T1-weighted MR image showing heterogeneous gado-linium enhancement of cavernous hemangioma in the IAC.

Fig. 2. Images obtained in a 40-year-old man with right cavernous hemangioma of the IAC who underwent translabyrinthine tumor removal and facial nerve reconstruction with end-to-end anastomosis.  a: In-traoperative photograph depicts the hemorrhagic tumor and the facial nerve surrounded by tumor.  b: Intraoperative photograph depicts the intraneural infiltration of tumor. The facial nerve could not be preserved in this patient.  c: Facial nerve reconstruction with end-to-end anas-tomosis.  d: Pathological findings show cavernous hemangioma with extension into neural fibers. H & E, original magnification ×40. Figure is available in color online only.

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table 2. literature review of cavernous hemangiomas of the iac*

Case No. Authors & Year

Age (yrs), Sex

Lesion  Location

Main  Symptoms

Surgical  Approach Intraop Findings Postop Results

1 Sundaresan et al., 1976

23, M IAC  HL, FW Retrosigmoid Lesion attached to CN VIII Improvement of FW

2 50, M IAC  HL, FW Retrosigmoid Lesion attached to interme-diate nerve

Improvement of FW, HL remained

3 Brackmann et al., 1980

NA IAC NA NA NA NA

4 NA IAC NA NA NA NA5 Mangham et al., 

198129, M IAC HL Translabyrinthine CN VII involved, facial-

facial anastomosisTotal HL, facial 

paralysis6 44, F IAC HL Translabyrinthine CN VII involved, tumor 

left around intact facial nerve

Total HL, no FW

7 Pappas et al., 1989

26, M IAC HL, tinnitus Translabyrinthine Lesion adherent to CN VII Delayed total FW, recovering

8 31, F IAC, CPA HL, tinnitus, FW Translabyrinthine Lesion adherent to CN VII FW, improved9 29, M IAC, CPA HL Translabyrinthine Facial-facial anastomosis FW, improved10 39, M IAC  HL Translabyrinthine NA Normal FNF11 56, M IAC HL, tinnitus Translabyrinthine Lesion adherent to CN VII Delayed postop FW, 

improved12 44, M IAC HL, tinnitus Translabyrinthine Lesion adherent to CN VII Delayed postop FW, 

improved13 66, F IAC HL, FW Translabyrinthine NA Normal FNF14 Madden & Siri-

manna, 199036, F IAC HL, FW, HFS Translabyrinthine Facial-facial anastomosis 1 yr postop no im-

provement in FW15 Matias-Guiu et al., 

199024, F IAC HL, tinnitus NA Lesion attached to co-

chlear nerveDeaf on affected site

16 Bordi et al., 1991 29, M IAC HL Retrosigmoid Lesion completely displac-ing & sectioning CN VII

Facial nerve recon-struction 2 wks postop, deaf

17 Cremers et al., 1991

39, M IAC HL, tinnitus, FW Transotic Facial nerve anatomically intact

Considerable FW

18 Jacobson & Reams, 1991

41, F IAC Unsteadiness Middle fossa Lesion compressing facial nerve

Preservation of hearing, full facial nerve recovery

19 Atlas et al., 1992 38, F IAC HL Translabyrinthine NA NA20 Fujino et al., 1993 58, M IAC HL, tinnitus,  

vertigoRetrosigmoid Lesion adherent to facial & 

cochlear nerveAuditory deficit, mild 

FW21 Saleh et al., 1993 44, M IAC HL, FW Translabyrinthine Lesion surrounding CN VII FW22 Babu et al., 1994 36, M IAC HL, vertigo Retrosigmoid Lesion originating in CN VII FNF normal, HL 

improved23 Dufour et al., 1994 NA, M IAC HL, HFS NA NA NA24 NA, M IAC HL NA NA NA25 NA, M IAC HL, HFS NA NA NA26 NA, M IAC HL NA NA NA27 NA, M IAC HL, HFS NA NA NA28 Fukuda et al., 

199534, M IAC Vertigo, head-

ache, HLMiddle fossa Lesion near CN VII Normal FNF, HL 

same29 Greiner-Perth et 

al., 199732, M IAC Dizziness, tin-

nitus, HLRetrosigmoid Lesion intimately con-

nected to CN VII & VIIINeurological deficits 

subsided30 Kohan et al., 1997 NA IAC HL, tinnitus, FW Translabyrinthine Subtotal tumor resection No CN deficit

(continued)

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diagnosis and treatment of cavernous hemangioma of the iac

firmed through the presence of irregular bony erosion and intralesional calcification on CT scan, by heterogeneous enhancement of the tumor on MR images, and through associated clinical features. Table 3 summarizes the dif-ferential diagnosis of the IAC tumor.

The vascularized tumor grows in an intraneural infil-

trating manner that can result in nerve dysfunction at an early stage even though the tumor size is small. This phe-nomenon can be explained by the fact that the blood sup-ply to the nerve trunk is accordingly reduced as the tumor grows, creating a desperate need for blood. Early-stage tumor removal increases the likelihood of preserving the

table 2. literature review of cavernous hemangiomas of the iac* (continued)

Case No. Authors & Year

Age (yrs), Sex

Lesion  Location

Main  Symptoms

Surgical  Approach Intraop Findings Postop Results

31 Omojola et al., 1997

45, M IAC HL, tinnitus, HFS, FW

Translabyrinthine Facial nerve separated from tumor

FW

32 Roche et al., 1997 34, F IAC HL Translabyrinthine Facial nerve anterosuperior to tumor

FW

33 62, F IAC, CPA HL, tinnitus Translabyrinthine Lesion adherent to CN VII FW, hypoglossal-fa-cial anastomosis

34 Sasaki et al., 1999 39, F IAC Tinnitus, HL Retrosigmoid Lesion compressing CN VIII & fanned CN VII, 70% tumor removal

Hearing preserved, FW resolved

35 Gjuric et al., 2000 43, F IAC Tinnitus, aural fullness, head-ache, HL

Middle fossa Tumor arising from inferior vestibular nerve

Hearing, FNF preserved

36 Sepehrnia et al., 2000

53, M IAC HL, tinnitus, FW Retrosigmoid Lesion adherent to CN VII Mild FW, HL increased

37 Shaida et al., 2000 30, F IAC HL, FW, tinnitus Translabyrinthine Lesion compressing CN VII FW improved38 Alobid et al., 2002 61, M IAC Tinnitus, HL, FW Retrosigmoid Lesion compressing CN VII FNF unchanged39 Aquilina et al., 

200429, F IAC, multiple 

infratentori-al locations

HL, FW Translabyrinthine Lesion adherent to CN VII & VIII

FW unchanged

40 Barrera et al., 2004

21, M IAC HL, tinnitus Translabyrinthine Lesion adherent to superior vestibular nerve

Uneventful

41 Masazuku et al., 2005

21, M IAC HL, FW Retrosigmoid Lesion adherent to CN VII & VIII

FW improved

42 Samii et al., 2006 23, F IAC, CPA HL, FW Suboccipital ret-rosigmoid

Lesion adherent to CN VII & VIII

FW improved, deaf

43 28, M IAC HL, tinnitus Suboccipital ret-rosigmoid

Lesion adherent to co-chlear nerve

FW, deaf

44 29, M IAC, CPA HL, tinnitus Suboccipital ret-rosigmoid

Lesion adherent to CN VIII FW, deaf

45 40, M IAC HL, HFS Suboccipital ret-rosigmoid

Lesion adherent to co-chlear nerve

FNF unchanged, deaf

46 42, M IAC HL, tinnitus Suboccipital ret-rosigmoid

Lesion adherent to CN VIII FNF unchanged, deaf

47 53, M IAC HL, FW Suboccipital ret-rosigmoid

Lesion adherent to CN VII & VIII, facial nerve reconstruction

FW, deaf

48 53, F IAC HL, tinnitus, HFS Suboccipital ret-rosigmoid

Lesion adherent to CN VII & VIII, facial nerve reconstruction

FW, deaf

49 Lenarz et al., 2007 51, M IAC HL, FW, tinnitus Translabyrinthine Lesion adherent to CN VII & VIII, facial nerve reconstruction

FW, deaf

50 Safronova et al., 2009

40, M IAC HL, FW Suboccipital ret-rosigmoid

Lesion adherent to CN VII & VIII

FW improved

HFS = hemifacial spasm; HL = hearing loss.*  Cavernous hemangiomas at other sites, including the temporal bone, middle ear, cerebellopontine angle, or geniculate ganglion, were excluded. 

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integrity of the facial nerve and may improve surgical out-comes. In the literature, a wait-and-scan approach is not used for these tumors; early surgical removal is the first choice of treatment. The natural history of the lesion in-dicates that treatment for cavernous hemangiomas of the IAC should be more aggressive than treatment for intra-canalicular VSs and facial neuromas.21,36 Patients with VS of the IAC can be followed with annual MRI and audio-metric examination. Similarly, patients with facial neuro-ma should not undergo surgery until facial palsy appears because the facial nerve cannot be preserved during sur-gery. However, for patients with cavernous hemangiomas, early surgical intervention should be performed, which may improve the chances of preserving the functional in-tegrity of the facial nerve and provide better results after nerve reconstruction. In the literature and in our series, nerve infiltration status was determined during surgery in 46 out of 56 patients. Facial nerve infiltration occurred in 36 of these patients (78.3%), and functional facial in-tegrity was preserved in 23 patients (50%). Although it is tempting to excise these benign lesions in view of their relatively slow-growing nature, a period of observation seems a point of consideration. However, when consider-ing the intraneural infiltrating growth manner, surgical in-tervention seems inevitable, and the chances of preserving facial nerve function are higher with smaller lesions than with larger ones. Complete surgical removal of the lesion, while avoiding complications such as bleeding into sur-rounding structures, is the goal of treatment. With regard to the timing of surgery, it has been suggested that sur-gery should be performed after an initial diagnosis. How-ever, in some newly diagnosed patients who do not have facial problems, a wait-and-scan approach can be used with strict follow-up. If the tumor enlarges or any facial problems develop, surgical removal of the tumor should be performed as soon as possible. The facial problems that indicate infiltration of the facial nerve include facial paresis, facial spasm, mild facial weakness, and transient facial palsy.

The surgical approaches for treatment of IAC tumors are retrosigmoid, middle cranial fossa, or translabyrin-thine. The retrosigmoid approach has often been advo-cated by neurosurgeons since they are more familiar with that approach. However, in patients with a significant hear-ing deficit, the translabyrinthine approach seems to be the best approach. With this approach, cerebellar retraction is avoided and the facial nerve can be easily located. Fur-thermore, it is easy to perform facial nerve reconstruction using either primary end-to-end anastomosis or a sural

nerve graft. In patients with residual levels of functional hearing, the middle cranial fossa approach may be better than the retrosigmoid approach, since the tumor often in-volves the fundus of the IAC. However, both the middle cranial fossa and the retrosigmoid approaches rarely pre-serve functional hearing because of intraneural infiltration of the tumor, even though the continuity of the cochlear nerve is preserved. Results using the various surgical ap-proaches have been reported for 47 patients. The translab-yrinthine approach was most common and was used in 25 of 47 patients (53.2%); 18 patients (38.3%) were treated us-ing the retrosigmoid approach, and 4 patients (8.5%) were treated using the middle cranial fossa approach. However, only 3 patients experienced serviceable hearing preserva-tion. Therefore, we advocate use of the translabyrinthine approach for the majority of patients in order to achieve the best postoperative facial nerve function. The treatment strategy for cavernous hemangiomas is summarized in Fig. 3.

table 3. differential diagnosis criteria for iac tumors

Lesion TypeClinical Feature Radiological Findings Treatment 

StrategyHearing Loss Facial Problem Location CT MRI

Cavernous hemangioma  Most common & severe

Common Outer 1/3 of IAC

Irregular bony erosion & calcification

Heterogeneous enhancement 

Surgery

Vestibular schwannoma Common Rare Outer 1/3 of IAC

None or smooth bony ero-sion, no calcification

Homogeneous enhancement 

Often wait & scan

Facial neuroma Less common Common Often involves geniculum

None or smooth bony ero-sion, no calcification

Homogeneous enhancement

Wait until FNF HB III

Fig. 3. Treatment strategies for cavernous hemangioma of the IAC. FN = facial nerve; MF = middle fossa approach; RS = retrosigmoid ap-proach; TL = translabyrinthine approach.

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diagnosis and treatment of cavernous hemangioma of the iac

The severity of compression and/or infiltration of neu-ral structures determines patient prognosis and functional outcome of surgery. The final surgical outcome with regard to preserving facial nerve function is highly dependent on early intervention. Tumor removal in the early stages seems to increase the likelihood of preserving nerve func-tion. However, in cases of intraneural infiltration, separa-tion of the tumor from the nerve is not possible and resec-tion of the infiltrated portion of the facial nerve with nerve reconstruction becomes necessary.21

conclusionsThe presence of a small, heterogeneous contrast-en-

hancing tumor with irregular bony erosion and intralesion-al calcification in the IAC accompanied by severe senso-rineural hearing loss and facial palsy should be identified as cavernous hemangioma. Early recognition and surgical intervention may improve the chances of preserving the functional integrity of the facial nerve and provide better results after nerve reconstruction. The translabyrinthine approach is appropriate for these tumors, although the ret-rosigmoid or middle cranial fossa approach can also be performed in some patients with useful preoperative hear-ing. However, serviceable hearing preservation is almost impossible to achieve, even if cochlear nerve integrity is maintained.

acknowledgmentsWe thank these patients for their generous cooperation in this

study. This study was supported by the National Natural Science Foundation of China (Nos. 81371086 and 81200742).

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disclosureThe authors report no conflict of interest concerning the materi-als or methods used in this study or the findings specified in this paper.

author contributionsConception and design: Wang, Wu. Acquisition of data: Zhu, Huang. Analysis and interpretation of data: Zhu. Drafting the article: Zhu. Critically revising the article: Huang, Wu. Reviewed submitted version of manuscript: Li. Statistical analysis: Li, Chen. Administrative/technical/material support: Chen. Study supervision: Wang, Wu.

correspondenceZhao Yan Wang, Xinhua Hospital, Shanghai Jiaotong University, School of Medicine, Department of Otolaryngology–Head and Neck Surgery, 1665 Kongjiang Rd., Shanghai 200092, People’s Republic of China. email: [email protected].

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