Neurol Sci DOI 10.1007/s10072-015-2216-z
LETTER TO THE EDITOR
Solid hemangioblastoma of vestibular nerve mimicking vestibular schwannoma Gordan Grahovac1
Received: 16 March 2015 / Accepted: 13 April 2015 Ó Springer-Verlag Italia 2015
Keywords Hemangioblastoma Magnetic resonance imaging Von Hippel–Lindau disease Vestibular schwannoma Dear Sir, We report a case of solid hemangioblastoma originating from the vestibulocochlear nerve that was misdiagnosed with vestibular schwannoma. Most common cerebellopontine angle (CPA) neoplasms are vestibular schwannoma and accounting more of 90 % of neoplasms in CPA [1]. Central nervous system hemangioblastoma (HMBs) are a benign hypervascular neoplasm that constitutes 1–2.5 % of all intracranial tumors in the posterior fossa [2]. Hemangioblastomas commonly have a classical cystic appearance with a vascular mural nodule [2]. A 75-year-old woman has been treated for hemangioblastoma of the left cerebellar hemisphere in 1986 and in 1989. The contrast-enhanced computed tomography (CT) revealed cyst with mural nodule of the left cerebellar hemisphere. In 2013 she was hospitalized because of sudden right deafness and cerebellar ataxia. CT and magnetic resonance imaging (MRI) of the brain demonstrated a 2.9 9 2.6-cm solid tumor in the right cerebellopontine angle that was vividly enhancing and filling the inner auditory channel that was most consistent with vestibular schwannoma (Fig. 1). The patient underwent resection of the lesion via a right suboccipital retrosigmoid approach. Highly vascular solid & Gordan Grahovac [email protected] 1
Department of Neurosurgery, Clinical Hospital Dubrava, Av. Gojka Suska 6, 10000 Zagreb, Croatia
mass was revealed and removed in piecemeal fashion. The procedure was complicated with profuse bleeding from the tumor. The bleeding was controlled by bipolar coagulation, Surgicel (Ethicon, USA) and Surgiflo with thrombin (Ethicon, USA). The postoperative course was uneventful. Pathologic evaluation revealed a highly vascular neoplasm composed of mildly atypical stromal cells with hyperchromatic nuclei and variable amounts of cytoplasm (Fig. 2). Large number of cells displayed copious amounts of foamy, lipidized cytoplasm. A final diagnosis of hemangioblastoma was made. The patient clinical workup included detailed clinical history, ophthalmoscopy examination, MRI of the brain and spine and CT of the abdomen, and plasma chromogranin A for Von Hippel–Lindau (VHL) syndrome was negative. Hemangioblastoma are benign highly vascular lesions that most commonly arise in the cerebellum. These tumors can remain asymptomatic for long time due to slow growth. 25 % of the HMBs occur in patients with autosomal-dominant disease called VHL disease [3]. The HMBs of the cerebellum have classical appearance, reddish to brown mural nodule with single or multiple cysts. In cases when the HMBs occurs in spinal cord or cranial and peripheral nerves, they can have solid MRI appearance as presented in our case. Hemangioblastoma seldom occur within CPA [4–7]. Majority of CPA hemangioblastoma originates from cerebellar tissue. Only in three cases the origin of the HMBs was from vestibulocochlear nerve, as was in our case [4, 6]. In the case by Ferroli et al. [5] the tumor was only visualized during surgical removal of cerebellar HMBs in the patient with known VHL disease. Hemangioblastoma and schwannoma of the CPA are characterized by iso- to hypointensity on T1-weighted
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Neurol Sci
Fig. 1 Preoperative MRI. Axial (a), T2-weighted (b), T2-weighted fast spin echo (FSI) (c), T2-weighted flair shows inhomogeneous tumor matrix with numerous serpentine flow voids in and around the
tumor, and d T1-weighted image with contrast gadolinium enhancement showing well homogeneous enhanced neoplasm of the right cerebellopontine angle with mass filling internal auditory meatus
Fig. 2 Photomicrographs of the neoplasm. a A highly vascular neoplasm consisting of capillaries and small neoplastic cells (hematoxylin–eosin, original magnification 9100). b Stromal cells with hyperchromatic nuclei and copious laden-cytoplasm (hematoxylin–
eosin, original magnification 9200). c High power view showing numerous capillaries and tumor cells (hematoxylin–eosin, original magnification 9200)
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Neurol Sci
images, and strong gadolinium enhancement. On T2weighted images both lesions show high signal intensity. The HMBs can have high-flow vessels and dilated feeding arteries within or on the surface of the lesion that is best seen on T2-weighted and T2-weighted fluid attenuated inversion recovery sequence such was in our case. Treatment of the patients with HMBs can sometimes be demanding because of the locations and vascularization of the lesion and the fact that patient with VHL disease can develop multiple lesions over time [2]. Safe removal requires circumferential exposure by finding the plane between the tumor and the brain tissue with subsequent de-vascularization. In some cases piecemeal resection of the tumor must be utilized such was our case, but profuse bleeding can complicate removal. The angiography can give us insight into vascularization pattern and embolization can reduce the risk for significant intraoperative bleeding. Hemangioblastoma of the cranial nerve still harbor diagnostic challenge and neurosurgical pitfalls, and they should be considered special clinical entity when compared with other hemangioblastoma. Because of their location and vascularization they pose significant neurosurgical treating challenge. Conflict of interest of interest.
The authors declare that they have no conflict
Ethical standard All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
References 1. Machinis TG, Fountas KN, Dimopoulos V, Robinson JS (2005) History of acoustic neurinoma surgery. Neurosurg Focus 18(4):e9 2. Resche F, Moisan JP, Mantoura J, de Kersaint-Gilly A, Andre MJ, Perrin-Resche I, Menegalli-Boggelli D, Lajat Y, Richard S (1993) Haemangioblastoma, haemangioblastomatosis, and von Hippel– Lindau disease. Adv Tech Stand Neurosurg 20:197–304 3. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH (2003) The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 98(1):82–94. doi:10.3171/jns.2003.98.1.0082 4. Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A (2010) Hemangioblastoma of the cerebellopontine angle. Arch Otolaryngol Head Neck Surg 136(7):734–738. doi:10.1001/ archoto.2010.98 5. Ferroli P, Giombini S, Franzini A, Broggi G (2003) Cranial nerve hemangioblastoma. J Neurosurg 98(4):934–935 author reply 935 6. Resto VA, Lustig LR (2000) Hemangioblastoma of the internal acoustic canal in a patient with von Hippel–Lindau disease: a case report and review of the literature. Arch Otolaryngol Head Neck Surg 126(1):85–88 7. Brackmann DE, Bartels LJ (1980) Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg 88(5):555–559
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LETTER TO THE EDITOR
Solid hemangioblastoma of vestibular nerve mimicking vestibular schwannoma Gordan Grahovac1
Received: 16 March 2015 / Accepted: 13 April 2015 Ó Springer-Verlag Italia 2015
Keywords Hemangioblastoma Magnetic resonance imaging Von Hippel–Lindau disease Vestibular schwannoma Dear Sir, We report a case of solid hemangioblastoma originating from the vestibulocochlear nerve that was misdiagnosed with vestibular schwannoma. Most common cerebellopontine angle (CPA) neoplasms are vestibular schwannoma and accounting more of 90 % of neoplasms in CPA [1]. Central nervous system hemangioblastoma (HMBs) are a benign hypervascular neoplasm that constitutes 1–2.5 % of all intracranial tumors in the posterior fossa [2]. Hemangioblastomas commonly have a classical cystic appearance with a vascular mural nodule [2]. A 75-year-old woman has been treated for hemangioblastoma of the left cerebellar hemisphere in 1986 and in 1989. The contrast-enhanced computed tomography (CT) revealed cyst with mural nodule of the left cerebellar hemisphere. In 2013 she was hospitalized because of sudden right deafness and cerebellar ataxia. CT and magnetic resonance imaging (MRI) of the brain demonstrated a 2.9 9 2.6-cm solid tumor in the right cerebellopontine angle that was vividly enhancing and filling the inner auditory channel that was most consistent with vestibular schwannoma (Fig. 1). The patient underwent resection of the lesion via a right suboccipital retrosigmoid approach. Highly vascular solid & Gordan Grahovac [email protected] 1
Department of Neurosurgery, Clinical Hospital Dubrava, Av. Gojka Suska 6, 10000 Zagreb, Croatia
mass was revealed and removed in piecemeal fashion. The procedure was complicated with profuse bleeding from the tumor. The bleeding was controlled by bipolar coagulation, Surgicel (Ethicon, USA) and Surgiflo with thrombin (Ethicon, USA). The postoperative course was uneventful. Pathologic evaluation revealed a highly vascular neoplasm composed of mildly atypical stromal cells with hyperchromatic nuclei and variable amounts of cytoplasm (Fig. 2). Large number of cells displayed copious amounts of foamy, lipidized cytoplasm. A final diagnosis of hemangioblastoma was made. The patient clinical workup included detailed clinical history, ophthalmoscopy examination, MRI of the brain and spine and CT of the abdomen, and plasma chromogranin A for Von Hippel–Lindau (VHL) syndrome was negative. Hemangioblastoma are benign highly vascular lesions that most commonly arise in the cerebellum. These tumors can remain asymptomatic for long time due to slow growth. 25 % of the HMBs occur in patients with autosomal-dominant disease called VHL disease [3]. The HMBs of the cerebellum have classical appearance, reddish to brown mural nodule with single or multiple cysts. In cases when the HMBs occurs in spinal cord or cranial and peripheral nerves, they can have solid MRI appearance as presented in our case. Hemangioblastoma seldom occur within CPA [4–7]. Majority of CPA hemangioblastoma originates from cerebellar tissue. Only in three cases the origin of the HMBs was from vestibulocochlear nerve, as was in our case [4, 6]. In the case by Ferroli et al. [5] the tumor was only visualized during surgical removal of cerebellar HMBs in the patient with known VHL disease. Hemangioblastoma and schwannoma of the CPA are characterized by iso- to hypointensity on T1-weighted
123
Neurol Sci
Fig. 1 Preoperative MRI. Axial (a), T2-weighted (b), T2-weighted fast spin echo (FSI) (c), T2-weighted flair shows inhomogeneous tumor matrix with numerous serpentine flow voids in and around the
tumor, and d T1-weighted image with contrast gadolinium enhancement showing well homogeneous enhanced neoplasm of the right cerebellopontine angle with mass filling internal auditory meatus
Fig. 2 Photomicrographs of the neoplasm. a A highly vascular neoplasm consisting of capillaries and small neoplastic cells (hematoxylin–eosin, original magnification 9100). b Stromal cells with hyperchromatic nuclei and copious laden-cytoplasm (hematoxylin–
eosin, original magnification 9200). c High power view showing numerous capillaries and tumor cells (hematoxylin–eosin, original magnification 9200)
123
Neurol Sci
images, and strong gadolinium enhancement. On T2weighted images both lesions show high signal intensity. The HMBs can have high-flow vessels and dilated feeding arteries within or on the surface of the lesion that is best seen on T2-weighted and T2-weighted fluid attenuated inversion recovery sequence such was in our case. Treatment of the patients with HMBs can sometimes be demanding because of the locations and vascularization of the lesion and the fact that patient with VHL disease can develop multiple lesions over time [2]. Safe removal requires circumferential exposure by finding the plane between the tumor and the brain tissue with subsequent de-vascularization. In some cases piecemeal resection of the tumor must be utilized such was our case, but profuse bleeding can complicate removal. The angiography can give us insight into vascularization pattern and embolization can reduce the risk for significant intraoperative bleeding. Hemangioblastoma of the cranial nerve still harbor diagnostic challenge and neurosurgical pitfalls, and they should be considered special clinical entity when compared with other hemangioblastoma. Because of their location and vascularization they pose significant neurosurgical treating challenge. Conflict of interest of interest.
The authors declare that they have no conflict
Ethical standard All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
References 1. Machinis TG, Fountas KN, Dimopoulos V, Robinson JS (2005) History of acoustic neurinoma surgery. Neurosurg Focus 18(4):e9 2. Resche F, Moisan JP, Mantoura J, de Kersaint-Gilly A, Andre MJ, Perrin-Resche I, Menegalli-Boggelli D, Lajat Y, Richard S (1993) Haemangioblastoma, haemangioblastomatosis, and von Hippel– Lindau disease. Adv Tech Stand Neurosurg 20:197–304 3. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH (2003) The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 98(1):82–94. doi:10.3171/jns.2003.98.1.0082 4. Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A (2010) Hemangioblastoma of the cerebellopontine angle. Arch Otolaryngol Head Neck Surg 136(7):734–738. doi:10.1001/ archoto.2010.98 5. Ferroli P, Giombini S, Franzini A, Broggi G (2003) Cranial nerve hemangioblastoma. J Neurosurg 98(4):934–935 author reply 935 6. Resto VA, Lustig LR (2000) Hemangioblastoma of the internal acoustic canal in a patient with von Hippel–Lindau disease: a case report and review of the literature. Arch Otolaryngol Head Neck Surg 126(1):85–88 7. Brackmann DE, Bartels LJ (1980) Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg 88(5):555–559
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