Cerebellopontine angle gangliogliomas: Report of two cases

Neurochirurgie 62 (2016) 266–270

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Clinical case

Cerebellopontine angle gangliogliomas: Report of two cases夽 S. Boissonneau a,∗ , L.-M. Terrier b , S. De La Rosa Morilla a , L. Troude a , J.P. Lavieille c , P.-H. Roche a a b c

Department of Neurosurgery, Assistance publique–Hôpitaux de Marseille, Aix-Marseille University, 13015 Marseille, France Department of Neurosurgery, CHRU de Tours, University Franc¸ois-Rabelais, 37000 Tours, France Department of ENT Surgery, Assistance publique–Hôpitaux de Marseille, Aix-Marseille University, 13015 Marseille, France

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Article history: Received 10 January 2016 Received in revised form 14 June 2016 Accepted 9 July 2016 Available online 19 October 2016 Keywords: Ganglioglioma Cerebellopontine angle Neuro-oncology Anaplastic ganglioglioma Brain tumor

a b s t r a c t Background. – Gangliogliomas are rare tumors of the central nervous system. We report two unusual cases of gangliogliomas located in the cerebellopontine angle (CPA). Population and methods. – The first patient was a 57-year-old woman, who presented with dizziness and harbored a non-enhanced heterogeneous mass located in the cisternal space of the CPA. A partial microsurgical removal was performed, and the pathological examination concluded a grade I ganglioglioma according to the WHO Classification. The postoperative course was uneventful without any adjuvant treatment and the 5-year imaging follow-up indicated a stable remnant tumor. The second patient was a 35-year-old male who presented with acute vertigo and imbalance associated with recent prominent headaches; MR imaging showed a large heterogeneous and post-contrast enhanced tumor mass located in the CPA cistern with a mass effect on the brain. An optimal subtotal surgical resection was performed. The pathologists concluded a WHO grade III ganglioglioma. In spite of adjuvant radiotherapy and chemotherapy, the evolution proved unfavorable and patient died from cancer complications within a 2-year period. In both cases, the precise origin of the tumor could not be clearly identified even if the major component was present in the cisternal space. Conclusion. – Gangliogliomas growing into the cisternal spaces are exceedingly rare particularly in the CPA. Due to its infiltrating behavior and major difficulties to identify the tumor margins, total resection is not routinely feasible. The histological grading is the most important predictor for oncological prognosis. © 2016 Elsevier Masson SAS. All rights reserved.

1. Introduction

1.1. Case no 1

Gangliogliomas are rare central nervous system (CNS) tumors and represent about 1 % of primitive cerebral tumors. Gangliogliomas are mostly encountered in children and young adults [1]. They can grow in any part of the CNS but most frequently from the temporal lobe where they usually induce seizures [2,3]. Some exceptional cases of optic chiasma or spinal cord gangliogliomas have also been described [4,5] as well as cisternal locations. In this report, we describe two cases of adult patients harboring a cerebellopontine angle (CPA) ganglioglioma of different grading and opposite prognoses.

A 57-year-old female patient, with no remarkable previous medical history, was referred to our department for a three-month history of dizziness and imbalance associated with decreased hearing of the left-right sides and tinnitus. The hearing was estimated to be grade B on the Gardner Robertson Hearing Scale. The clinical examination was remarkable for a left vestibular ataxia. There was no motor or sensory deficit and the cranial nerve examination was normal. The gadolinium-enhanced brain MRI and CT-scan showed a calcified tumor mass that occupied the left CPA, responsible for a significant mass effect on the middle cerebellar peduncle (Fig. 1). A microsurgical resection via a retrosigmoid approach was scheduled to relieve the compression of the brainstem and provide a biopsy specimen for histopathological examination. During surgery, the tumor occupied the cisternal space, and its texture was solid and poorly vascularized. Frozen section specimen suggested a glioma and the procedure was limited to surgical debulking in an attempt to minimize morbidity. At the end of the procedure, 60 % of the tumor volume was removed. The tumor had not obviously

夽 The patient or family has consented to the submission of the case report for submission to the journal. ∗ Corresponding author. Centre Hospitalo-Universitaire Nord, Assistance publique–Hôpitaux de Marseille, Service de Neurochirurgie du Pr-ROCHE, chemin des Bourrely, 13015 Marseille, France. E-mail address: [email protected] (S. Boissonneau). http://dx.doi.org/10.1016/j.neuchi.2016.07.001 0028-3770/© 2016 Elsevier Masson SAS. All rights reserved.

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Fig. 1. Preoperative MRI and TDM of MS G showing a left cerebellopontine lesion, enhanced by gadolinium injection.

developed from the brainstem but encased the acoustico-facial bundle and lower cranial nerves. The postoperative course was uneventful with no cranial nerve deficit. No complications occurred. The pathological diagnosis was consistent with a

calcified ganglioglioma grade I (WHO Classification), and confirmed by two pathologists. Following the multidisciplinary team meeting, a standard clinical and imaging follow-up was decided with no additional adjunctive radiation therapy. During the

Fig. 2. Radiological evolution after five years of a cerebellopontine angle ganglioglioma OMS rank I, from left to right: postoperative TDM, MRI at three months and five years.

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Fig. 3. Preoperative TDM of Mr. K. showing a voluminous tumor of the cerebellopontine angle.

early postoperative period the patient complained of vestibular ataxia that required physiotherapy and rehabilitation, tinnitus was still present, and hypoacousis had become worse. However, she returned to work and daily activities after 3 months and was sequentially followed-up with an MRI. At 5 years follow-up, the MRI showed a complete stability of the residual tumor with no new clinically-related symptoms (Fig. 2). 1.2. Case no 2 The second patient, a 35-year-old man with no previous medical history, complained of a 5-day history of intense vertigo, imbalance, headache and severe hypoacousis (Fig. 3). The brain MRI and CT-scan showed a large CPA calcified lesion on the right side, with mass effect on the fourth ventricle and brainstem (Fig. 4). Initially, a large biopsy by ENT surgeons was performed using a translabyrinthine approach and the pathological diagnosis was consistent with a chordoid meningioma. An early second

Fig. 5. MRI of Mr. K. after second surgery and adjuvant radiotherapy, showing tumoral residue.

surgical procedure was performed via a retrosigmoid approach. The major cisternal portion of the tumor (solid texture, hypervascularized) was resected but a tumor remnant was left at the level of the middle cerebellar peduncle adjacent that was clearly infiltrated, the tumor was very adhesive and there was no cleavage plane between the lesion and cranial nerves. No probe monitored intubation was used during the procedure. The pathological examination confirmed the diagnosis of an anaplastic ganglioglioma (Grade III WHO classification). Histological analyzes were reviewed by two different pathologists. In the early postoperative course, the patient experienced a right hemiparesis, a left hemi-hypoesthesia, and lower cranial nerve deficit on the right side. He underwent an aspiration pneumonia with respiratory distress that required emergency management including ventilation, antibiotic therapy and

Fig. 4. MRI of Mr. K. after initial surgery.

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Fig. 6. TEP-TDM showing the hyper-metabolism of tumoral residue, despite surgery and adjuvant radiotherapy.

a tracheotomy. After recovery of infection and a multidisciplinary team conference, adjunctive treatment that associated intravenous temozolomide (TMZ) (Temodal® ) and radiotherapy was started. Temodal® was begun three months after the second procedure, with radiotherapy. There was no tumoral evolution between the retrosigmoid procedure and the beginning of the chemoradiotherapy. Imaging follow-up showed an infiltrating tumor residue of the middle cerebellar peduncle, with hyper-metabolism on FDG PET-CT scan (Figs. 5 and 6). Eighteen months later, the patient experienced an acute obstructive hydrocephalus that required an urgent external ventricular drainage while the tumor mass remained stable. The analysis of the CSF showed an aseptic meningitis with high cellularity. In spite of an endoscopic ventriculocisternostomy, several episodes of drowsiness status due to recurrent obstructive hydrocephalus required further procedures of endoscopic fenestration. We decided not to proceed with a ventriculoperitoneal shunt due to the high cellularity of the CSF, and the important risk of a shunting malfunction. The patient died within 48 hours after admission due to hydrodynamic complications related to carcinomatous meningitis. 2. Discussion To date, there have been very few reports of gangliogliomas located in the CPA in the adult population [6,7]. However, although exceptional this disease cannot be completely ruled out when discussing the possible diagnosis and confronted with anatypical cisternal tumor mass occupying the CPA. In fact, no specific imaging features can be considered as highly suggestive of the diagnosis and CPA gliomas [7], exophytic papillomas, or medulloblastomas could be legitimately considered for discussion. In this study, we describe two cases of this exceptional entity in this location. In the first case, the patient is still alive and the postoperative course was uneventful despite a partial resection and no adjuvant treatment. In the second case, the resection was partial and additional oncological treatment was proposed due to the anaplastic grading of the tumor. Gangliogliomas consist of the association of glial and ganglionic mature cell proliferation. The clonal origins of these tissues are now established and, the majority of these tumors are classified as benign with a good prognosis. Few anaplastic forms exist and appear very challenging in particular for young adults. Prognosis of gangliogliomas correlates with histological grading and extent of resection [8–11] In some cases the preoperative diagnosis of a malignant glioma is proposed and may limit the extent of surgical resection resulting in a survival disadvantage for the patients [12]. Moreover, the radical surgical resection is not always safely achievable in particular in the setting of the highly complex neurovascular environment found within the CPA. The comparison of these two surgical cases leads us to establish a distinction between two different anatomical forms. The first case did not appear to infiltrate the brain parenchyma and appeared purely cisternal whereas the second displayed some brainstem infiltration. In both cases the size of the lesion was different, also

probably partially explaining the postoperative consequences that were much more difficult in the second case, outside the rank of the tumor. These case reports were aimed at informing neurosurgeons regarding the existence of this very rare entity among the CPA tumors, and underlines two completely different cases in terms of surgical and histological findings and postoperative behavior and prognosis. Currently, a ganglioglioma of the brainstem and cerebellopontine angle is more frequently described in the pediatric population [13–16], with few treatments, like targeted therapy (vemurafenib) was proposed, with interesting results in this patient population with the mutation BRAFV600E [17]. The association between BRAFV600E mutation and ganglioglioma seems to be more and more important for the prognosis and therapeutics [18]. 3. Conclusion Gangliogliomas of CPA are truly exceptional, but they still need to be considered as a potential diagnosis facing an atypical tumor mass occupying the CPA. Surprisingly, some of these tumors do not appear to be clearly connected to brain tissue. In spite of limited data from the literature and modest experience of our team, it appears that tumor biology seems to be more influential than the extent of resection in the prognosis. Disclosure of interest The authors declare that they have no competing interest. References [1] Song JY, Kim JH, Cho YH, Kim CJ, Lee EJ. Treatment and outcomes for gangliogliomas: a single-center review of 16 patients. Brain Tumor Res Treat 2014;2(2):49–55. [2] Bendersky D, Christiansen S, Ciraolo C, Tedesco N, García MC. Epilepsy due to a temporal ganglioglioma and its subsequent malignant transformation into a primitive neuroectodermal tumor. Surg Neurol Int 2012;3(1):79. [3] Giulioni M, Marucci G, Martinoni M, Marliani AF, Toni F, Bartiromo F, et al. Epilepsy associated tumors: review article. World J Clin Cases 2014;2(11):623–41. [4] Shuangshoti SEK. Ganglioglioma of the optic chiasm: case report and review of the literature. AJNR Am J Neuroradiol 2000;21(8):1486–9. [5] Gessi M, Dörner E, Dreschmann V, Antonelli M, Waha A, Giangaspero F, et al. Intramedullary gangliogliomas: histopathologic and molecular features of 25 cases. Hum Pathol 2016;49:107–13. [6] Cuthikonda B, Buckleair LJ, Goodman JC, Powell SZ, Rose JE. Ganglioglioma in the cerebellopontine angle of an adult without seizures. J La State Med Soc 2009;161(3):143–6. [7] Matsuzaki K, Uno M, Kageji T, Hirose T, Nagahiro S. Anaplastic ganglioglioma of the cerebellopontine angle. Case report. Neurol Med Chir (Tokyo) 2005;45(11):591–5. [8] Demierre B, Stichnoth FA, Hori A, Spoerri O. Intracerebral ganglioglioma. J Neurosurg 1986;65(2):177–82. [9] Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, et al. Central nervous system gangliogliomas. Part 2: clinical outcome. J Neurosurg 1993;79(6):867–73. [10] Luyken C, Blümcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J. Supratentorial gangliogliomas: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer 2004;101(1):146–55. [11] Zentner JHKW. Gangliogliomas: clinical, radiological and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry 1995;57(12):1497–502.

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