Solid Cerebellar Hemangioblastoma with Peritumoral Edema: 5-Years Follow Up

pISSN 2384-1095 iMRI 2015;19:248-251 http://dx.doi.org/10.13104/imri.2015.19.4.248 eISSN 2384-1109

Solid Cerebellar Hemangioblastoma with Peritumoral Edema: 5-Years Follow up

Kyoung Jin Hwang, Soo Jin Song, Key-Chung Park, Sung Sang Yoon, Tae-Beom Ahn Department of Neurology, School of Medicine, Kyung Hee University, Seoul, Korea

Hemangioblastomas are angioblastic tumors of the central nervous system. Cerebellar hemangioblastomas are traditionally classified into two morphologic types-cystic and solid. Cystic hemangioblastomas are associated with peritumoral edema, but solid Case Report hemangioblastomas are not. We report a case of solid cerebellar hemangioblastoma with massive peritumoral edema. An 83-year-old female visited our hospital due to a sudden headache. Five years ago, she had been admitted to our hospital with similar headache and diagnosed with cerebellar hemangioblastoma. Follow-up brain Received: November 5, 2015 Revised: December 9, 2015 MRI 5 years later showed an increased size of a homogeneous enhancing mass with Accepted: December 11, 2015 aggravated peritumoral edema in the left lower cerebellar hemisphere. Cerebral angiography showed a highly vascularized mass in the cerebellum, which was Correspondence to: compatible with a solid-type hemangioblastoma. Tae-Beom Ahn, M.D. Department of Neurology, Kyung Keywords: Hemangioblastoma; Peritumoral edema; Solid Hee University Medical Center, #1 Hoegi-dong, Dongdaemun-gu, Seoul 130-702, Korea. Tel. +82-2-958-8499 Fax. +82-2-958-8490 INTRODUCTION Email: [email protected] Hemangioblastomas (HBs) are benign vascular neoplasms that originate in the central nervous system (CNS) and comprise about 1.5-2.5% of intracranial tumors. They are usually in the cerebellum (76%) and uncommon in the cerebral hemispheres (9%), spinal cord (7%), and brainstem (5%) (1). A single tumor may be sporadic, but multiple

This is an Open Access article distributed tumors are almost always associated with Von Hippel-Lindau disease, an autosomal under the terms of the Creative Commons dominant disorder characterized by various lesions, including renal cell carcinoma, Attribution Non-Commercial License (http://creativecommons.org/licenses/ pheochromocytoma and visceral cysts. by-nc/3.0/) which permits unrestricted Cerebellar HBs are traditionally classified into two morphologic types: cystic and solid non-commercial use, distribution, and (1). Cystic-HBs are associated with peritumoral edema (2), but solid HBs are not. reproduction in any medium, provided the original work is properly cited. Here, we report a case of solid cerebellar HB with massive peritumoral edema.

CASE REPORT Copyright © 2015 Korean Society of Magnetic Resonance in An 83-year-old female visited our hospital due to a sudden headache. The headache Medicine (KSMRM) was dull, continuous, and concentrated in the left temporo-occipital area. She did not complain of nausea. Five years ago, she had been admitted to our hospital with a similar headache. Brain

248 www.i-mri.org http://dx.doi.org/10.13104/imri.2015.19.4.248 magnetic resonance imaging (MRI) (Figs. 1, 2) at the first treatment, her symptoms improved. admission showed a homogeneous enhancing mass (ovoid shape, 7.2 × 5.9 mm in size) with surrounding edema in the left lower cerebellar hemisphere. However, she refused DISCUSSION management and did not attend the hospital thereafter. The patient had no history of hypertension or diabetes This report describes a patient with solid cerebellar HB mellitus, and no relevant family history was found. Her with massive peritumoral edema. Although peritumoral vital signs were within the normal ranges. A neurological edema of HB is associated with cystic lesions of HB (2), it examination was normal. Her blood tests were normal. is interesting that the solid-type HB may also contribute to Follow-up brain MRI (Figs. 1, 2) 5 years later showed an formation of peritumoral edema. increased size of the homogeneous enhancing mass (9.0 Cerebellar HBs are divided into four subtypes according × 7.4 mm) with aggravated peritumoral edema in the left to histological (3) and MRI (4) findings: simple cyst without lower cerebellar hemisphere. Cerebral angiography (Fig. 3) a macroscopic nodule (5% of posterior fossa HBs), cyst showed a highly vascularized mass in the cerebellum, which with a mural nodule (60%), solid tumors (26%), and solid was compatible with a solid-type HB. tumors with small internal cysts (9%). In this case, the Chest and abdominal computed tomography were normal. cerebellar HB was solid. However, histologically, there is no The patient underwent gamma knife surgery. After the distinct differentiation between the mainly cystic and solid

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d e f Fig. 1. T1 weighted postcontrast MR images. Brain MRI at the time of first admission (a-c) shows a homogeneous enhancing mass (~7.2 × 5.9 mm in size) in the left lower cerebellum. Follow-up brain MRI (d-f) 5 years later showed an increased size of the homogeneous enhancing mass (~9.0 × 7.4 mm) in the left lower cerebellar hemisphere. www.i-mri.org 249 Solid Hemangioblastoma with Peritumoral Edema | Kyoung Jin Hwang, et al.

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Fig. 2. Fluid-attenuated inversion recovery MR images. Brain MRI at the time of the first admission (a-d) shows edematous changes in the left lower cerebellum. Follow-up brain MRI (e- h) 5 years later shows aggravated peritumoral edema in the left lower cerebellar hemisphere.

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tumor subtypes (5). Both subtypes present with a biphasic symptom of cerebellar HB is headache. Vomiting, ataxia, differentiation composed of large vacuolated stromal cells vertigo, diplopia and nystagmus are also common and a dense capillary network. symptoms. The majority of symptomatic tumors are Generally, HB tends to enlarge extremely slowly. In associated with cysts (6), and the progressive growth of the particular, solid lesions remain stable in size and therefore cystic component of the tumor results in mass effects. asymptomatic for many years (1). The most common Peritumoral edema is a precursor to formation of HB-

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which depends on increased tumor vascular permeability, extravasation of fluid may contribute to formation of both edema and cyst. Indeed, chemotherapeutic agents that directly or indirectly decrease vascular permeability, may reduce both edema and cyst formation (8). This case suggests that solid cerebellar HB might also be accompanied by peritumoral edema; clinicians should be aware of these imaging characteristics of solid HB.

REFERENCES 1. Hussein MR. Central nervous system capillary haemangi- oblastoma: the pathologist's viewpoint. Int J Exp Pathol 2007;88:311-324 2. Lonser RR, Vortmeyer AO, Butman JA, et al. Edema is a Fig. 3. Cerebral angiography shows a hypervascular mass precursor to central nervous system peritumoral cyst (arrow) supplied by the left posterior inferior cerebellar formation. Ann Neurol 2005;58:392-399 artery. 3. Richard S, Martin S, David P, Decq P. Von Hippel-Lindau disease and central nervous system hemangioblastoma. Progress in genetics and clinical management. associated cysts, which could progress into peritumoral Neurochirurgie 1998;44:258-266 cysts in the cerebellum (2). Peritumoral cyst formation in 4. Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton CNS is initiated by increased vascular permeability of the TH. Posterior fossa hemangioblastomas: MR imaging. tumor. As the plasma ultrafiltrate enters the interstitial Radiology 1989;171:463-468 space of the tumor, interstitial pressure increases and 5. Rachinger J, Buslei R, Prell J, Strauss C. Solid haemangi- plasma extravasates with convective distribution into the oblastomas of the CNS: a review of 17 consecutive cases. surrounding tissue. If the delivery of plasma from the tumor Neurosurg Rev 2009;32:37-47; discussion 47-48 exceeds the capacity to absorb the extravasated fluid of the 6. Slater A, Moore NR, Huson SM. The natural history of surrounding tissue, edema and subsequent cyst formation cerebellar hemangioblastomas in von Hippel-Lindau occur (2). disease. AJNR Am J Neuroradiol 2003;24:1570-1574 However, this case showed a solid HB with peritumoral 7. Nie Q, Guo P, Shen L, Li X, Qiu Y. Early-stage hemangi- edema and aggravated edema at the 5-year follow up. oblastoma presenting as a small lesion with significant Although similar imaging cases that showed peritumoral edema in the cerebellum. J Craniofac Surg 2015;26:e119- edema in solid HB have been reported (5, 7), they did 121 not emphasize its importance. By cerebral angiography, 8. Girmens JF, Erginay A, Massin P, Scigalla P, Gaudric solid lesions often demonstrate a complex arteriovenous A, Richard S. Treatment of von Hippel-Lindau retinal malformation with a clear vascular blush, multiple feeding hemangioblastoma by the vascular endothelial growth vessels and draining veins (5). It could be hypothesized that factor receptor inhibitor SU5416 is more effective for peritumoral edema of solid HB has a similar mechanism to associated macular edema than for hemangioblastomas. Am J Ophthalmol 2003;136:194-196 that of peritumoral cysts. Based on the pathogenesis of HB,