Case 171: Facial Nerve Hemangioma

Abstract

HomeRadiologyVol. 260, No. 1 PreviousNext Diagnosis PleaseFree AccessCase 171Case 171: Facial Nerve HemangiomaSarah V. Mijangos, Daniel E. Meltzer Sarah V. Mijangos, Daniel E. Meltzer Author AffiliationsFrom the Department of Radiology, St. Luke’s-Roosevelt Hospital Center, 1000 10th Ave, Suite 4B-14, New York, NY 10013.Address correspondence to D.E.M. (e-mail: [email protected]).Sarah V. MijangosDaniel E. Meltzer Published Online:Jul 1 2011https://doi.org/10.1148/radiol.11092009MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked InEmail AbstractHistoryA 50-year-old man presented with left peripheral facial nerve paresis, which gradually progressed to complete paralysis over the course of several weeks. The patient underwent examination of the temporal bones with magnetic resonance (MR) imaging, which was followed by computed tomography (CT) (Aqulion 64; Toshiba Medical Systems, Togichi, Japan).Imaging FindingsMR imaging revealed an area of abnormal T2 hyperintensity (Fig 1) in the left geniculate ganglion. The lesion had intrinsic T1 signal that was isointense to brain parenchyma (Fig 2). Postgadolinium MR images showed intense enhancement of the lesion, with punctate areas of nonenhancement (Fig 3). These nonenhancing foci within the mass corresponded to the stippled foci of hypointensity visible on T1- and T2-weighted images. On axial CT images of the temporal bone, these punctate areas of hypointensity and nonenhancement corresponded to foci of bone density within an expansile lytic lesion involving the geniculate ganglion and labyrinthine segment of the facial nerve (Fig 4a). This intralesional osseous matrix had a honeycomb pattern, best demonstrated on the coronal CT image (Fig 4b).Figure 1a: (a, b) Axial T2-weighted 1.5-T MR images (repetition time msec/echo time msec, 4000/121; 3-mm section thickness) (a) at the level of the internal auditory canal and (b) immediately craniad to a. In a, there is abnormal globular signal hyperintensity in the region of the labyrinthine segment of the facial nerve and the inferior aspect of the left geniculate fossa (arrow), with stippled punctate foci of hypointense signal. In b, there is contiguous abnormal signal intensity in the more craniad aspect of the geniculate fossa (arrow).Figure 1a:Download as PowerPointOpen in Image Viewer Figure 1b: (a, b) Axial T2-weighted 1.5-T MR images (repetition time msec/echo time msec, 4000/121; 3-mm section thickness) (a) at the level of the internal auditory canal and (b) immediately craniad to a. In a, there is abnormal globular signal hyperintensity in the region of the labyrinthine segment of the facial nerve and the inferior aspect of the left geniculate fossa (arrow), with stippled punctate foci of hypointense signal. In b, there is contiguous abnormal signal intensity in the more craniad aspect of the geniculate fossa (arrow).Figure 1b:Download as PowerPointOpen in Image Viewer Figure 2: Axial T1-weighted 1.5-T MR image (550/10, 3-mm section thickness) at the same level as Figure 1b. The lesion in Figure 1b is isointense to brain parenchyma on this T1-weighted image (arrow). Note the stippled punctate foci of hypointense signal.Figure 2:Download as PowerPointOpen in Image Viewer Figure 3: Axial T1-weighted 1.5-T contrast material–enhanced (15 mL of intravenously injected gadopentetate dimeglumine, Magnevist; Bayer Healthcare Pharmaceuticals, Wayne, NJ) fat-saturated MR image (500/14, 3-mm section thickness) at the same level as Figure 1b shows intense enhancement of the lesion (arrow), with the exception of a few punctate areas of nonenhancement that correspond to the stippled hypointense foci in Figure 1b and to the more prominent ossific components visible in Figure 4a.Figure 3:Download as PowerPointOpen in Image Viewer Figure 4a: (a) Axial thin-section CT image of the left temporal bone at the level of the internal auditory canal (1-mm section thickness). There is a mildly expansile lytic lesion with fine internal high-attenuation spiculations in the region of the geniculate ganglion (long arrow). There is involvement of the labyrinthine portion of the facial nerve canal (short arrow) and proximal tympanic segment (*). (b) Coronal targeted reformatted CT image of the left temporal bone at the level of the cochlea (1-mm section thickness). The image is centered on the expansile lesion shown in a (arrow) and best demonstrates the honeycomb pattern of internal bone spicules within the geniculate fossa.Figure 4a:Download as PowerPointOpen in Image Viewer Figure 4b: (a) Axial thin-section CT image of the left temporal bone at the level of the internal auditory canal (1-mm section thickness). There is a mildly expansile lytic lesion with fine internal high-attenuation spiculations in the region of the geniculate ganglion (long arrow). There is involvement of the labyrinthine portion of the facial nerve canal (short arrow) and proximal tympanic segment (*). (b) Coronal targeted reformatted CT image of the left temporal bone at the level of the cochlea (1-mm section thickness). The image is centered on the expansile lesion shown in a (arrow) and best demonstrates the honeycomb pattern of internal bone spicules within the geniculate fossa.Figure 4b:Download as PowerPointOpen in Image Viewer DiscussionThis patient’s imaging features, especially the honeycomb pattern of ossific changes in the geniculate fossa, were virtually pathognomonic for ossifying hemangioma of the facial nerve.Facial nerve hemangioma of the temporal bone is a rare entity. These lesions have been reported to account for 0.7% of all intratemporal tumors (1,2). While there have been reports of this tumor in young children, the majority of patients present in the 3rd to 6th decade of life, with nearly equal distribution between men and women (3).Facial nerve hemangiomas are benign tumors, although their pathogenesis is not completely understood. The most common site of occurrence is the geniculate fossa, followed by the internal auditory canal; less frequently, hemangiomas occur in the posterior genu of the facial nerve (1). The distribution of tumor location has been shown to correlate with the density of capillary plexi surrounding the facial nerve, which is highest in the region of the geniculate ganglion (4).At surgery, facial nerve hemangiomas appear as blue highly vascular masses intimately associated with the facial nerve. Histopathologic evaluation reveals vascular channels lined with a layer of epithelium. Cavernous, capillary, or mixed types of hemangioma have been described and grouped according to the predominant size of the intralesional vascular spaces (5). When intratumoral bony spicules are identified, the lesion is commonly referred to as ossifying hemangioma (6). These ossific changes give ossifying hemangioma the characteristic honeycomb matrix seen at CT (7). Histologic evaluation of ossifying hemangioma reveals extensive bone remodeling with regions of osteoclastic absorption and new bone formation in the collagen-containing walls between the vascular channels rather than neoplastic bone formation by the tumor (8,9).Intratemporal capillary hemangiomas most often arise in the geniculate ganglion, and cavernous hemangiomas have a predilection for the internal auditory canal (2). Tumor size ranges from approximately 2 mm to 2 cm; however, these tumors are often symptomatic at an early stage of development while still small (usually <10 mm) (10). Symptoms depend on tumor location and result from tumor compression and/or invasion of adjacent structures. Patients with hemangiomas that originate in the geniculate fossa most often present with facial nerve paralysis that is slowly progressive over the course of several weeks (11). Patients with facial nerve dysfunction also may have a rapidly progressive or recurrent episodic course (8). Sensorineural hearing loss and pulsatile tinnitus may occur if there is erosion of the cochlear otic capsule (1). Other less common symptoms depend on the growth pattern of the tumor and the resulting involvement of neighboring structures.Patients with hemangioma of the internal auditory canal most often present with slowly progressive unilateral sensorineural hearing loss, which can eventually result in complete deafness (8). Facial nerve dysfunction is a less common symptom of tumors in this region. The clinical presentation of patients with hemagioma of the internal auditory canal is similar to that of patients with schwannoma at the same location, although hemangiomas cause more severe cranial nerve dysfunction than facial or vestibular nerve schwannomas of similar size (1). Patients with hemangioma of the mastoid segment of the facial nerve may have a variable presentation, and these lesions often cause symptoms characteristic of either of the aforementioned tumor types (8).While most patients with ossifying hemangioma of the geniculate ganglion present with facial nerve impairment, the progression of facial nerve impairment is frequently less rapid than is typical in patients with classic Bell palsy, and the symptoms may only partially resolve and then recur. In these clinical settings, imaging is an appropriate part of the diagnostic process (12). In our experience, the first imaging study to be performed is usually gadolinium-enhanced MR imaging, with use of thin-section sequences through the region of the internal auditory canals to evaluate the intracranial and temporal bone portions of the facial nerve. Intratemporal hemangiomas characteristically have variable signal intensity on T1-weighted images and increased signal intensity on T2-weighted images, with avid contrast enhancement. Low-signal-intensity foci may be seen on T1- and T2-weighted images, corresponding to the ossific matrix of the lesion. Once an enhancing mass has been detected in the geniculate fossa, thin-section CT enables exquisite visualization of associated bone changes. The margins of the lesion are irregular and amorphous, and there may be osseous spicules seen within the tumor matrix (7). While essentially pathognomonic, this typical honeycomb appearance of facial nerve hemangiomas at CT is present in only approximately 50% of cases (5). In the absence of this finding, differentiation from schwannoma or other more rare neoplasms, such as meningioma (3), may be difficult. A border of thin new bone may also be seen around the tumor, secondary to remodeling of the adjacent bone (8).The differential diagnosis based on the MR characteristics of the lesion include normal intratemporal facial nerve enhancement, Bell palsy, schwannoma of the facial nerve, and perineural spread of parotid malignancy along the facial nerve. Congenital cholesteatoma (epidermoid) of the middle ear may rarely produce facial nerve weakness, especially if there is congenital dehiscence of the facial nerve canal medial to the mass (13). This lesion may be visible at MR imaging but would not enhance with administration of a gadolinium-containing contrast agent.In an asymptomatic patient, normal enhancement of the geniculate ganglion or anterior tympanic or mastoid segments of the facial nerve can be seen on contrast material–enhanced T1-weighted images, which can sometimes be mistaken for abnormal enhancement. Normal enhancement is secondary to the extensive perineural vasculature of the facial nerve. Enhancement of the intracanalicular or intraparotid facial nerve is generally regarded as abnormal. Mild to moderate enhancement of the labyrinthine and mastoid segments may be normal, but intense enhancement of these portions of the facial nerve should raise suspicion of an underlying abnormality (14,15).Patients with Bell palsy experience acute onset (frequently in the course of 24 hours) of peripheral facial nerve paralysis, which may be partial or complete. Imaging characteristics include abnormal intense enhancement of the facial nerve (without thickening of the nerve) at postcontrast T1-weighted imaging, which can involve the entire course of the intratemporal facial nerve, including the labyrinthine segment or the portion of the nerve within the fundus of the internal auditory canal, distinguishing this pathologic enhancement from normal facial nerve enhancement. Bell palsy has been described in patients of all ages, with peak presentation occurring in the 5th decade of life (16).Patients with facial nerve schwannomas have a variable clinical presentation that depends on the pattern of growth. Symptoms may include hearing loss, facial nerve dysfunction, or both. Previous case series have shown that there is a wide range of age at presentation but that, on average, patients are late into the 4th decade of life at presentation (17,18). The most common location of a schwannoma is the geniculate ganglion (18). Schwannomas can also arise within the internal auditory canal; within the labyrinthine, tympanic, or mastoid portions of the facial nerve; or within the parotid gland. Contrast-enhanced T1-weighted MR imaging depicts an enhancing mass along the course of the facial nerve. Smooth expansion of the facial nerve canal is typically seen at thin-section CT. Facial nerve schwannomas tend to reach a relatively large size by the time they become symptomatic, whereas facial nerve hemangiomas may cause severe symptoms while they are still relatively small (5,11).Another important consideration in the differential diagnosis of abnormal facial nerve enhancement is perineural spread of parotid malignancy along the facial nerve. Patients may or may not have a known diagnosis of parotid malignancy and can present with facial nerve paralysis. Head and neck squamous cell cancer, adenoid cystic carcinoma of the parotid gland, and lymphoma are the most common malignancies known to track along the facial nerve (19). An enhancing invasive parotid mass may be seen on postcontrast T1-weighted images, with loss of normal fat signal in the stylomastoid foramen and abnormally avid enhancement of the facial nerve in a linear fashion, although the enhancement may be discontinuous (skip lesions).Complete surgical resection of facial nerve hemangiomas is the definitive treatment. It is crucial for the surgeon to consider whether the tumor can be separated from the facial nerve, thus sparing facial nerve disruption and further damage. Histologically, there remains some controversy in the literature about whether there is actual invasion of the facial nerve by the closely associated hemangioma or if perineural reaction alone is the cause of indistinct planes between the tumor and nerve (5,20–22). Preservation of facial nerve continuity is less frequently possible with geniculate ganglion tumors than with tumors involving the internal auditory canal because of intense perineural reaction, actual neural infiltration, or both (5). Tumor location, preoperative auditory function, and tumor size must be considered when planning the surgical approach. Although timing of surgical intervention remains somewhat controversial, most surgeons recommend early intervention to preserve facial function. In the best scenario, the tumor can be successfully dissected from the facial nerve with no disruption of neural integrity. In patients with intense perineural reaction or possible microinvasion by the tumor, surgical treatment would require resection of a portion of the facial nerve followed by cable grafting, often resulting in facial nerve function ranging from 50% to 75% (8).Used in combination, MR imaging and CT have high sensitivity for tumors of the facial nerve in general, and patients with facial nerve hemangioma may have additional osseous changes at CT that are virtually pathognomonic. Because of the local destructiveness of the tumor, early resection offers the best chance for recovery of facial nerve function (9,21). MR and CT are crucial in the prompt detection and diagnosis of facial nerve hemangioma (11).Congratulations to the 126 individuals and two resident groups that submitted the most likely diagnosis (facial nerve hemangioma) for Diagnosis Please, Case 171. The names and locations of the individuals and resident groups, as submitted, are as follows:Individual responsesOsamu Abe, MD, PhD, Itabashi-ku, Tokyo, JapanOliver M. Afchani, Sr, MD, Rapperswil, SwitzerlandGholamali Afshang, MD, Tinley Park, IllAlbert J. Alter, MD, PhD, Blanchardville, WisErsan Altun, MD, Istanbul, TurkeyGuis S. Astacio, MD, Rio de Janeiro, BrazilAsim K. Bag, MD, Birmingham, AlaKenneth F. Baliga, MD, Rockford, IllPanagiotis Baroutas, MD, Athens, GreecePhilippe P. Bertrand, MD, Zottegem, BelgiumDavid Besachio, DO, Santa Rita, GuamBradford A. Bottger, MD, Redding, ConnEric L. Bressler, MD, Minnetonka, MinnDaniel F. Broderick, MD, Jacksonville, FlaDouglas C. Brown, MD, Virginia Beach, VaMichael P. Buetow, MD, Okemos, MichIan A. Burgess, MD, North Sydney, New South Wales, AustraliaBalasundaram Chandra-Sekar, MD, Vestavia Hills, AlaShuji Chino, MD, Matsusaka, Mie, JapanCarla Conceição, MD, Lisboa, PortugalMauricio Ramos Corral, MD, El Paso, TexVictor Cuvinciuc, MD, Geneva, SwitzerlandApostolos V. Dalakidis, MD, Voula, GreeceMarc G. De Baets, MD, Collina d’Oro, Ticino, SwitzerlandPeter De Baets, MD, Damme, BelgiumJohannes F. De Villiers, MBChB, MMed, Gisborne, New ZealandThaworn Dendumrongsup, MD, Songkhla, ThailandCharles L. Diana, MD, Sandwich, MassBrendan T. Doherty, MD, Columbia, SCDionisios Drakopoulos, MD, Athens, Attiki, GreeceSeyed A. Emamian, MD, PhD, Rockville, MdChristian Escalona, MD, Mexico City, MexicoFrancis T. Flaherty, MD, Ridgefield, ConnAkira Fujikawa, MD, Setagaya, Tokyo, JapanGilles Genin, Annecy, FranceMark G. Goldshein, MD, Andover, MassTerrence M. Gross, MD, Winter Park, FlaSaurabh Guleria, MD, Milwaukee, WisPramod K. Gupta, MD, Plano, TexOsamu Hasegawa, MD, Koriyama, Fukushima, JapanRintaro Hashimoto, MD, Kobe, Hyogo, JapanD. C. Heasley, Jr, MD, Dallas, TexSodai Hoshiai, MD, Tsukuba, Ibarki, JapanAlberto C. Iaia, MD, Wilmington, DelNoriatsu Ichiba, MD, Otsu, Shiga, JapanSharada Jayagopal, MD, East Williston, NYMiral D. Jhaveri, MD, Chicago, IllKouhei Kamiya, Tokyo, JapanSabiha P. Karakas, MD, Cleveland, OhioSho Koyasu, MD, Kyoto, JapanPat S. Kwankaew, MD, Umphur Muang, Kanchanaburi, ThailandStefanos Lachanis, MD, Athens, GreeceMario A. Laguna, MD, Milwaukee, WisMichael Laucella, MD, Bay Shore, NYLaurent Letourneau-Guillon, MD, Toronto, Ontario, CanadaDonald R. Lewis Jr, MD, Huntington, WVLi-Chang Lien, MD, Orange, CalifDavid A. Lisle, MBBS, Brisbane, Queensland, AustraliaYohsuke Makino, Setagaya-ku, Tokyo, JapanYalachipura C. Manjunatha, MD, Chandigarh, IndiaSatoshi Matsushima, MD, Tokyo, JapanBarry C. McNulty, MD, Canton, OhioAlbert Mendelson, MD, Northbrook, IllAna L. Mendizabal, MD, Mexico City, MexicoManabu Minami, MD, PhD, Yokohama, JapanKenichi Mizuki, MD, Hamamatsu-shi, Shizuoka-Ken, JapanHiroyuki Morisaka, Kofu, Yamanashi, JapanThomas Moser, MD, Montreal, Quebec, CanadaKazuyoshi Nakamura, MD, Yokkaichi, Mie, JapanTammam N. Nehme, MD, Portage, MichTomokazu Nishiguchi, MD, Bordeaux Cedex, FranceHiroshi Nobusawa, MD, PhD, Ota, Tokyo, JapanMichael D. Orsi, MD, San Antonio, TexKlaus Orth, Aachen, GermanyVishal Panchal, San Diego, CalifIoannis E. Papachristos, MD, Agrinio, GreeceNarendrakumar P. Patel, MD, Newburgh, NYYeliz Pekcevik, Izmir, TurkeyMarco C. Pinho, MD, Boston, MassJoseph Platnick, MD, Staten Island, NYJohn M. Plotke, MD, Naperville, IllDavid J. Polinger-Hyman, MD, West Pittston, PaKalyan C. Poruri, MD, South Elgin, IllIlias Primetis, MD, Athens, GreeceKhashayar Rafat Zand, MD, Chestnut Hill, MassMuddassir Rashid, MBBS, Melbourne, Victoria, AustraliaMantosh S. Rattan, MD, Cincinnati, OhioMohamed-Ihab S. Reda, MD, Alexandria, EgyptVictor Regenbogen, MD, Pittsford, NYMatthew C. Rheinboldt, MD, Nashville, TennHugo Rodríguez Requena, MD, Madrid, SpainGlade E. Roper, MD, Sacramento, CalifRocky C. Saenz, DO, Farmington Hills, MichAkihiko Sakata, MD, Kyoto, JapanAkram Y. Saleh, BMedSc, Zarka, JordanRoberto Q. Santos, MD, Rio de Janeiro, BrazilSteven M. Schultz, MD, Fort Worth, TexStephen D. Scotti, MD, Charlottesville, VaAnthony J. Scuderi, MD, Johnstown, PaMatthew P. Shapiro, MD, Charlottesville, VaHideki Shima, MD, Tokyo, JapanTaro Shimono, MD, Osaka, JapanGary Sidhu, MD, Vancouver, British Columbia, CanadaDavid F. Sobel, MD, La Jolla, CalifGuner Sonmez, Istanbul, TurkeyJames D. Sprinkle, Jr, MD, Spotsylvania, VaNicholas J. Statkus, MD, Redding, Calif,Eijun Sueyoshi, MD, Nagasaki, JapanThomas P. Sullivan, MD, Seattle, WashHongliang Sun, MD, Beijing, ChinaPattama Tanaanantarak, Hatyai, Songkla, ThailandEliko Tanaka, MD, Yokohama, Kanagawa, JapanMateusz Terlecki, MD, Warszawa, PolandChristina M. Thuerl, MD, Seckach, GermanyEugene Tong, MD, Austin, TexMeric Tuzun, Ankara, TurkeyChristian von Falck, MD, Hannover, Lower Saxony, GermanyDavid I. Winger, MD, Lenox, MassBernard Wirth, MD, Lons, FranceToshihide Yamaoka, MD, Kyoto, JapanKurata Yasuhisa, MD, Kobe, Hyogo, JapanHajime Yokota, MD, Chiba, JapanStanko Yovichevich, MD, Marrickville, New South Wales, AustraliaJeffrey H. Zapolsky, MD, Oshkosh, WisHenry R. Zayas, MD, Stuart, FlaAhmed Zidan, MD, Barcelona, SpainResident group responsesPrince of Songkla University Radiology Residents, Songkla, ThailandNaval Medical Center Residents, San Diego, CalifAuthors stated no financial relationship to disclose.Part one of this case appeared 4 months previously and may contain larger images.References1 Mangham CA, Carberry JN, Brackmann DE. 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Crossref, Medline, Google ScholarArticle HistoryReceived November 7, 2009; revision requested December 11; revision received January 7, 2010; accepted January 18; final version accepted February 3.Published online: July 2011Published in print: July 2011 FiguresReferencesRelatedDetailsCited ByEuropean Journal of Radiology, Vol. 126Surgical Neurology International, Vol. 11Laryngoscope Investigative Otolaryngology, Vol. 4, No. 3Cranial Nerve Schwannomas: Diagnostic Imaging ApproachAaron D. Skolnik, Laurie A. Loevner, Deepak M. Sampathu, Jason G. Newman, John Y. Lee, Linda J. Bagley, Kim O. Learned, 19 August 2016 | RadioGraphics, Vol. 36, No. 5“Honeycomb” signLa Presse Médicale, Vol. 45, No. 5American Journal of Otolaryngology, Vol. 37, No. 2European Archives of Oto-Rhino-Laryngology, Vol. 272, No. 9Current Problems in Diagnostic Radiology, Vol. 44, No. 1A Case of Facial Nerve Cavernous Hemangioma in the Temporal BonePractica oto-rhino-laryngologica. 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