Neurochirurgie 61 (2015) 50–53
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ScienceDirect www.sciencedirect.com
Short clinical case
Suggestive value of predilection site and imaging features of pediatric brainstem ganglioglioma including a case report Gangliogliome du tronc cérébral chez l’enfant : valeur prédictive du site et des caractéristiques de l’imagerie, à partir d’un cas X. Anqi a , L. Zhenlin b , H. Xin c , Y. Chao a,∗ a
Department of Neurosurgery, West China Hospital, Sichuan University, 37, Guo Xue Xiang Street, Chengdu, Sichuan, 610041, PR China Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041, PR China c Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, PR China b
a r t i c l e
i n f o
Article history: Received 13 May 2014 Received in revised form 16 October 2014 Accepted 2 November 2014 Available online 7 February 2015 Keywords: Brainstem Ganglioglioma MR imaging Pediatric neurosurgery
a b s t r a c t Brainstem ganglioglioma is rarely reported. Due to its low incidence and atypical site, a brainstem ganglioglioma could easily be misdiagnosed as occurs with other pathological neoplasms radiologically. Here, we report an 8-year-old girl with a brainstem tumor confirmed as a ganglioglioma based on postoperative pathology results. We suggest that when a tumor located in the lower brainstem with benign radiological characteristics occurs in a child with a long-term history, the possibility of brainstem ganglioglioma should be considered in the preoperative diagnosis in addition to other low-grade neoplasms. Early stage diagnosis of brainstem ganglioglioma based on the clinical and imaging features is valuable for clinicians in order to perform effective treatment and achieve a good prognosis. © 2015 Elsevier Masson SAS. All rights reserved.
r é s u m é Mots clés : Tronc cérébral Gangliogliome Imagerie par résonance magnétique Neurochirurgie pédiatrique
Peu de cas de gangliogliomes sont rapportés dans la littérature. Du fait de la faible incidence et du siège atypique des gangliogliomes du tronc cérébral, leur diagnostic radiologique peut être difficile, comme c’est le cas pour d’autres tumeurs. Nous rapportons le cas d’une enfant de 8 ans ayant une tumeur du tronc cérébral pour laquelle les analyses anatomopathologiques postopératoires ont permis d’établir le diagnostic de gangliogliomes. Quand une tumeur située dans la partie basse du tronc cérébral présente des caractères radiologiques de bénignité, chez un enfant ayant des signes fonctionnels depuis une longue durée, le diagnostic de gangliogliome ou de tumeur de bas grade doit être évoqué. Faire le diagnostic de gangliogliome à un stage précoce permet d’entreprendre un traitement efficace et d’assurer un bon pronostic. © 2015 Elsevier Masson SAS. Tous droits réservés.
Ganglioglioma is a type of low-grade tumor with low incidence. Generally, it occurs supratentorially, while rarely encountered in the brainstem [1]. According to the literature, the majority of brainstem ganglioglioma cases were found in childhood [1–3]. Due to its unusual location and low incidence, it is very difficult to make a diagnosis before surgery, and about half of the cases were confirmed on autopsy. Because of its benign characteristics
and good prognosis, making the probable diagnosis of brainstem ganglioglioma appropriately based on preoperative clinical and imaging findings is very helpful for neurosurgeons to perform proper treatment. Here, we report the case of an 8-year-old girl with a brainstem ganglioglioma, and explore the value of the imaging findings for preoperative diagnosis. 1. Case report
∗ Corresponding author. E-mail address: shion [email protected] (Y. Chao). http://dx.doi.org/10.1016/j.neuchi.2014.11.007 0028-3770/© 2015 Elsevier Masson SAS. All rights reserved.
An 8-year-old girl, with 1-month history of hoarseness and dysphagia, was admitted to our hospital. The neurological
X. Anqi et al. / Neurochirurgie 61 (2015) 50–53
51
Fig. 1. The preoperative MR imaging of an 8-year-old girl with brainstem ganglioglioma. The MRI showed an irregular cystic-solid tumor located in the dorsal medulla oblongata and lower pons, pressing and protruding into the 4th ventricle, with heterogeneous iso-/hypointense on T1 weighted imaging (A) and non-uniform hyperintense on T2 weighted imaging (B). On the contrast-enhanced T1 weighted imaging, partial solid component of the tumor revealed vivid enhancement. C. The details of tumoral extension into left lateral foramen of 4th ventricle are clearly shown. IRM chez une enfant de 8 ans, présentant un gangliogliome du tronc cérébral. Présence d’une tumeur solide kystique, irrégulière, située dans le bulbe rachidien et la partie basse du pont, protuse dans le 4e ventricule, avec un hypo-isosignal en imagerie pondérée en T1 (A) et un hypersignal non uniforme en T2 (B). L’imagerie pondérée en T1 avec rehaussement montre le composant solide de la tumeur. L’extension tumorale au niveau du foramen latéral gauche du 4e ventricule est clairement mise en évidence par les images pondérées en T2 avec rehaussement.
examinations showed that she had difficulty in feeding, with extreme malnutrition and atrophy of the limb muscles. The preoperative MRI showed a solid-cystic mass, about 3.5 × 4.0 cm, located in medulla oblongata and pons, protruding into the 4th ventricle, with heterogeneous iso/hypointensity on T1 weighted imaging (T1WI) and iso/hyperintensity on T2 weighted imaging (T2WI). The contrast-enhanced T1WI showed a irregular mass of the dorsal brainstem with vivid enhanced solid tumoral components, invading median and lateral foramen of 4th ventricle, with localized involvement of the left cerebellopontine angle (Fig. 1). The partial resection through a posterior fossa median approach was performed. On macroscopic examination, the lesion was whitish-yellow with a hard texture, located in the medulla oblongata and pons with the compression of the bottom of 4th ventricle. The boundary of the tumor was undefined. During the operation, the exophytic portions of the tumors outside brainstem, including solid tumoral components invading the median and left lateral foramen, was removed.
The postoperative pathological examination (Fig. 2) revealed intratumoral and multi-cellular components composed of ganglion and glial cells. Immunohistochemical examination showed the tumor tissues were positive for Ki67, S-100, p53 and GFAP. Thus, the pathological diagnosis of ganglioglioma was confirmed. On the 4th day after surgery, CT scan revealed that the mass effect was alleviated with partial resection of the tumor, and 4th ventricle form was restored (Fig. 3). After the operation, the patient’s symptoms gradually alleviated, and she was discharged on the 21st day after surgery. 2. Discussion Gangliogliomas, composed by ganglion and glial cells, are a type of slow-growing benign neoplasms, with an incidence of less than 2% of the primary intracranial tumors with predilection age of the 1st 3 decades of life [1–3]. Generally, gangliogliomas occur in the supratentorial region with seizure as the first symptom, typically
Fig. 2. A. Photomicrograph of the tumor shows the coexistence of ganglion and glial cells (hematoxylin–eosin staining, × 200). B. Ki-67 labeling involves a few scattered glial components (the value less than 5%). C. GFAP is positive in neoplastic glial element of gangliogliomas and mitotic figures are rare. A. Microphotographie de la tumeur montrant la coexistence d’un ganglion et de cellules gliales (hématoxyline-éosine, × 200). B. Le marquage Ki-67 montre quelques composantes gliales dispersées (valeur inférieure à 5 %). C. Le GFAP est positif au niveau des éléments gliaux néoplasiques des gangliogliomes, et les mitoses sont rares.
52
X. Anqi et al. / Neurochirurgie 61 (2015) 50–53
Fig. 3. The postoperative CT imaging at the 4th day after surgery. The CT revealed that the mass effect was alleviated with partial resection of the tumor, and the 4th ventricle form was restored. Tomodensitométrie postopératoire (4e jour après la chirurgie). Elle révèle la diminution de l’effet de masse par la résection partielle de la tumeur, et la restauration du 4e ventricule dans sa forme initiale.
in the temporal and frontal lobes. Under rare conditions, they could appear at the 3rd ventricle, thalamus or brainstem, and the symptoms could include progressive cranial nerve symptoms, ataxia, or sudden respiratory and cardiac arrest [3]. Cases of brainstem gangliogliomas are very rare, and to our knowledge half of them were confirmed by autopsies [1,3]. Due to its low incidence, few brainstem gangliogliomas have been diagnosed by neuroradiologists and neurosurgeons preoperatively. Because of the benign nature of the tumor but important location, it is necessary for clinicians to be aware of the imaging and clinical manifestations of brainstem ganglioglioma. Gangliogliomas can be solid or cystic-solid, and similar to the other low-grade gliomas on imaging. On CT scan the solid part appears as iso/hypo-densities, often with calcifications as hyperdensity. On MRI, the ganglioglioma appears as hypointensity on T1WI, while on T2WI, it is often displayed as hyperintensity, and higher signals in the liquid parts if there are cysts. The peritumoral edema is infrequently seen. After gadolinium injection, the solid components always show a vivid enhancement. As regards the cystic-solid ganglioglioma, it can display enhanced intracapsular mural nodules. The brainstem ganglioglioma could have various appearances and lack of specificities on the imaging. Thus, it is very difficult to distinguish them radiologically. According to the literature, we found that brainstem gangliogliomas are commonly located in the medulla oblongata or the pontobulbar region as in our case, but rarely in the mesencephalic region [3]. We believe that the tendency of its predilection site may be a helpful suggestion in diagnosing brainstem ganglioglioma. The median and lateral apertures of the 4th ventricle are close to the medulla oblongata level, which means the 4th ventricular tumor from lower brainstem might involve the subarachnoid space of posterior cranial fossa via these foramens. Displaying the extension of the tumor into the apertures of 4th ventricle and posterior fossa subarachnoid on imaging is very important in guiding the operation, especially where the emphasis is to resect the exophytic portions of the tumor outside the brainstem. In our case, the cerebellopontine angle region was involved by the tumor via the lateral
foramens of the 4th ventricle [3]. Based on the preoperative MRI, the neurosurgeons explored this region and carefully resected the tumor tissues. On imaging, the differentiation of brainstem ganglioglioma from other benign brainstem neoplasms is difficult, especially when the tumor is the cystic-nodular type with the similar imaging appearance to a pilocytic astrocytoma and hemangioblastoma. Reviewing the literature, the majority of the brainstem gangliogliomas occurred in children, while hemangioblastomas are more frequent in adults [1,3,4]. This difference of predilection age might be helpful in suggesting brainstem ganglioglioma in children. The pilocytic astrocytoma also has a tendency to occur in children, which makes it more difficult to differentiate it with a ganglioglioma. However, the latter with predilection site of the lower brainstem should be observed in preoperative diagnosis. In our opinion, when this type of mass occurs in a child, especially the sac-nodular type, located in the lower brainstem with a long-term history and benign tumor characteristics on imaging, the possibility of brainstem ganglioglioma should be included in the differential diagnosis. As regards ganglioglioma treatment, extensive radical resection showed a good prognosis in the supratentorial region [1]. According to Lang et al., for patients with supratentorial ganglioglioma who accepted total resection, the 10-year survival rate reached 85% [3,5]. As for the brainstem ganglioglioma, due to its close proximity to the vital center, currently the subtotal or partial excision is the main surgical choice as opposed to total resection, for the main purpose of removing the protruding tumoral tissues from brainstem, particularly the enhanced solid tumoral portion on MRI. Concerning partial resection of the tumor, prognosis is relatively good according to the literature, with a 5-year survival rate of 78% [5]. The postoperative radiotherapeutic effect of ganglioglioma still remains controversial as regards its insensitivity to radiation, radiant adverse effects in children, and potential malignant tendency. However, some authors also believe that radiotherapy has a certain effect in controlling residual tumor after subtotal resection. As reported in some series, chemotherapy has limited value in ganglioglioma treatment, and may cause serious complications such as blood disorders [3,6]. Currently, chemotherapy or radiotherapy is chosen in cases of tumor progression or recurrence as an adjuvant therapy. 3. Conclusion The brainstem ganglioglioma is a rare benign tumor. Based on a review of the literature and our particular case, we consider that the medulla oblongata and lower part of pons might be predilection sites. The brainstem ganglioglioma is difficult to differentiate from other low-grade tumors based on the imaging alone. Therefore, we suggest that when a tumor located in the lower brainstem with benign radiological characteristics occurs in a child with a longterm history, the possibility of brainstem ganglioglioma should be considered in the preoperative diagnosis along with other lowgrade neoplasms. Partial resection is still the ideal therapy for brainstem ganglioglioma with main purpose of removing the tumor portion protruding from brainstem. The brainstem ganglioglioma might invade the cerebellopontine angle region via the lateral foramens of the 4th ventricle, and therefore being aware of the extending area on imaging is very valuable for neurosurgeons in order to create an effective surgical program. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
X. Anqi et al. / Neurochirurgie 61 (2015) 50–53
References [1] Lagares A, Gómez PA, Lobato RD, Ricoy JR, Ramos A, de la Lama A. Ganglioglioma of the brainstem: report of three cases and review of the literature. Surg Neurol 2001;56(5):315–22. [2] Mpairamidis E, Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N. Brainstem ganglioglioma. J Child Neurol 2008;23(12):1481–3. [3] Zhang S, Wang X, Liu X, Ju Y, Hui X. Brainstem gangliogliomas: a retrospective series. J Neurosurg 2013;118(4):884–8.
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[4] Kanno H, Kuratsu J, Nishikawa R, Mishima K, Natsume A, Wakabayashi T, et al. Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease. Acta Neurochir (Wien) 2013;155(1):1–7. [5] Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Ab- bott IR, et al. Central nervous system gangliogliomas. Part 2: clinical outcome. J Neurosurg 1993;79: 867–73. [6] Baussard B, Di Rocco F, Garnett MR, Boddaert N, Lellouch-Tubiana A, Grill J, et al. Pediatric infratentorial gangliogliomas: a retrospective series. J Neurosurg 2007;107(4 suppl.):286–91.
Disponible en ligne sur
ScienceDirect www.sciencedirect.com
Short clinical case
Suggestive value of predilection site and imaging features of pediatric brainstem ganglioglioma including a case report Gangliogliome du tronc cérébral chez l’enfant : valeur prédictive du site et des caractéristiques de l’imagerie, à partir d’un cas X. Anqi a , L. Zhenlin b , H. Xin c , Y. Chao a,∗ a
Department of Neurosurgery, West China Hospital, Sichuan University, 37, Guo Xue Xiang Street, Chengdu, Sichuan, 610041, PR China Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041, PR China c Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, PR China b
a r t i c l e
i n f o
Article history: Received 13 May 2014 Received in revised form 16 October 2014 Accepted 2 November 2014 Available online 7 February 2015 Keywords: Brainstem Ganglioglioma MR imaging Pediatric neurosurgery
a b s t r a c t Brainstem ganglioglioma is rarely reported. Due to its low incidence and atypical site, a brainstem ganglioglioma could easily be misdiagnosed as occurs with other pathological neoplasms radiologically. Here, we report an 8-year-old girl with a brainstem tumor confirmed as a ganglioglioma based on postoperative pathology results. We suggest that when a tumor located in the lower brainstem with benign radiological characteristics occurs in a child with a long-term history, the possibility of brainstem ganglioglioma should be considered in the preoperative diagnosis in addition to other low-grade neoplasms. Early stage diagnosis of brainstem ganglioglioma based on the clinical and imaging features is valuable for clinicians in order to perform effective treatment and achieve a good prognosis. © 2015 Elsevier Masson SAS. All rights reserved.
r é s u m é Mots clés : Tronc cérébral Gangliogliome Imagerie par résonance magnétique Neurochirurgie pédiatrique
Peu de cas de gangliogliomes sont rapportés dans la littérature. Du fait de la faible incidence et du siège atypique des gangliogliomes du tronc cérébral, leur diagnostic radiologique peut être difficile, comme c’est le cas pour d’autres tumeurs. Nous rapportons le cas d’une enfant de 8 ans ayant une tumeur du tronc cérébral pour laquelle les analyses anatomopathologiques postopératoires ont permis d’établir le diagnostic de gangliogliomes. Quand une tumeur située dans la partie basse du tronc cérébral présente des caractères radiologiques de bénignité, chez un enfant ayant des signes fonctionnels depuis une longue durée, le diagnostic de gangliogliome ou de tumeur de bas grade doit être évoqué. Faire le diagnostic de gangliogliome à un stage précoce permet d’entreprendre un traitement efficace et d’assurer un bon pronostic. © 2015 Elsevier Masson SAS. Tous droits réservés.
Ganglioglioma is a type of low-grade tumor with low incidence. Generally, it occurs supratentorially, while rarely encountered in the brainstem [1]. According to the literature, the majority of brainstem ganglioglioma cases were found in childhood [1–3]. Due to its unusual location and low incidence, it is very difficult to make a diagnosis before surgery, and about half of the cases were confirmed on autopsy. Because of its benign characteristics
and good prognosis, making the probable diagnosis of brainstem ganglioglioma appropriately based on preoperative clinical and imaging findings is very helpful for neurosurgeons to perform proper treatment. Here, we report the case of an 8-year-old girl with a brainstem ganglioglioma, and explore the value of the imaging findings for preoperative diagnosis. 1. Case report
∗ Corresponding author. E-mail address: shion [email protected] (Y. Chao). http://dx.doi.org/10.1016/j.neuchi.2014.11.007 0028-3770/© 2015 Elsevier Masson SAS. All rights reserved.
An 8-year-old girl, with 1-month history of hoarseness and dysphagia, was admitted to our hospital. The neurological
X. Anqi et al. / Neurochirurgie 61 (2015) 50–53
51
Fig. 1. The preoperative MR imaging of an 8-year-old girl with brainstem ganglioglioma. The MRI showed an irregular cystic-solid tumor located in the dorsal medulla oblongata and lower pons, pressing and protruding into the 4th ventricle, with heterogeneous iso-/hypointense on T1 weighted imaging (A) and non-uniform hyperintense on T2 weighted imaging (B). On the contrast-enhanced T1 weighted imaging, partial solid component of the tumor revealed vivid enhancement. C. The details of tumoral extension into left lateral foramen of 4th ventricle are clearly shown. IRM chez une enfant de 8 ans, présentant un gangliogliome du tronc cérébral. Présence d’une tumeur solide kystique, irrégulière, située dans le bulbe rachidien et la partie basse du pont, protuse dans le 4e ventricule, avec un hypo-isosignal en imagerie pondérée en T1 (A) et un hypersignal non uniforme en T2 (B). L’imagerie pondérée en T1 avec rehaussement montre le composant solide de la tumeur. L’extension tumorale au niveau du foramen latéral gauche du 4e ventricule est clairement mise en évidence par les images pondérées en T2 avec rehaussement.
examinations showed that she had difficulty in feeding, with extreme malnutrition and atrophy of the limb muscles. The preoperative MRI showed a solid-cystic mass, about 3.5 × 4.0 cm, located in medulla oblongata and pons, protruding into the 4th ventricle, with heterogeneous iso/hypointensity on T1 weighted imaging (T1WI) and iso/hyperintensity on T2 weighted imaging (T2WI). The contrast-enhanced T1WI showed a irregular mass of the dorsal brainstem with vivid enhanced solid tumoral components, invading median and lateral foramen of 4th ventricle, with localized involvement of the left cerebellopontine angle (Fig. 1). The partial resection through a posterior fossa median approach was performed. On macroscopic examination, the lesion was whitish-yellow with a hard texture, located in the medulla oblongata and pons with the compression of the bottom of 4th ventricle. The boundary of the tumor was undefined. During the operation, the exophytic portions of the tumors outside brainstem, including solid tumoral components invading the median and left lateral foramen, was removed.
The postoperative pathological examination (Fig. 2) revealed intratumoral and multi-cellular components composed of ganglion and glial cells. Immunohistochemical examination showed the tumor tissues were positive for Ki67, S-100, p53 and GFAP. Thus, the pathological diagnosis of ganglioglioma was confirmed. On the 4th day after surgery, CT scan revealed that the mass effect was alleviated with partial resection of the tumor, and 4th ventricle form was restored (Fig. 3). After the operation, the patient’s symptoms gradually alleviated, and she was discharged on the 21st day after surgery. 2. Discussion Gangliogliomas, composed by ganglion and glial cells, are a type of slow-growing benign neoplasms, with an incidence of less than 2% of the primary intracranial tumors with predilection age of the 1st 3 decades of life [1–3]. Generally, gangliogliomas occur in the supratentorial region with seizure as the first symptom, typically
Fig. 2. A. Photomicrograph of the tumor shows the coexistence of ganglion and glial cells (hematoxylin–eosin staining, × 200). B. Ki-67 labeling involves a few scattered glial components (the value less than 5%). C. GFAP is positive in neoplastic glial element of gangliogliomas and mitotic figures are rare. A. Microphotographie de la tumeur montrant la coexistence d’un ganglion et de cellules gliales (hématoxyline-éosine, × 200). B. Le marquage Ki-67 montre quelques composantes gliales dispersées (valeur inférieure à 5 %). C. Le GFAP est positif au niveau des éléments gliaux néoplasiques des gangliogliomes, et les mitoses sont rares.
52
X. Anqi et al. / Neurochirurgie 61 (2015) 50–53
Fig. 3. The postoperative CT imaging at the 4th day after surgery. The CT revealed that the mass effect was alleviated with partial resection of the tumor, and the 4th ventricle form was restored. Tomodensitométrie postopératoire (4e jour après la chirurgie). Elle révèle la diminution de l’effet de masse par la résection partielle de la tumeur, et la restauration du 4e ventricule dans sa forme initiale.
in the temporal and frontal lobes. Under rare conditions, they could appear at the 3rd ventricle, thalamus or brainstem, and the symptoms could include progressive cranial nerve symptoms, ataxia, or sudden respiratory and cardiac arrest [3]. Cases of brainstem gangliogliomas are very rare, and to our knowledge half of them were confirmed by autopsies [1,3]. Due to its low incidence, few brainstem gangliogliomas have been diagnosed by neuroradiologists and neurosurgeons preoperatively. Because of the benign nature of the tumor but important location, it is necessary for clinicians to be aware of the imaging and clinical manifestations of brainstem ganglioglioma. Gangliogliomas can be solid or cystic-solid, and similar to the other low-grade gliomas on imaging. On CT scan the solid part appears as iso/hypo-densities, often with calcifications as hyperdensity. On MRI, the ganglioglioma appears as hypointensity on T1WI, while on T2WI, it is often displayed as hyperintensity, and higher signals in the liquid parts if there are cysts. The peritumoral edema is infrequently seen. After gadolinium injection, the solid components always show a vivid enhancement. As regards the cystic-solid ganglioglioma, it can display enhanced intracapsular mural nodules. The brainstem ganglioglioma could have various appearances and lack of specificities on the imaging. Thus, it is very difficult to distinguish them radiologically. According to the literature, we found that brainstem gangliogliomas are commonly located in the medulla oblongata or the pontobulbar region as in our case, but rarely in the mesencephalic region [3]. We believe that the tendency of its predilection site may be a helpful suggestion in diagnosing brainstem ganglioglioma. The median and lateral apertures of the 4th ventricle are close to the medulla oblongata level, which means the 4th ventricular tumor from lower brainstem might involve the subarachnoid space of posterior cranial fossa via these foramens. Displaying the extension of the tumor into the apertures of 4th ventricle and posterior fossa subarachnoid on imaging is very important in guiding the operation, especially where the emphasis is to resect the exophytic portions of the tumor outside the brainstem. In our case, the cerebellopontine angle region was involved by the tumor via the lateral
foramens of the 4th ventricle [3]. Based on the preoperative MRI, the neurosurgeons explored this region and carefully resected the tumor tissues. On imaging, the differentiation of brainstem ganglioglioma from other benign brainstem neoplasms is difficult, especially when the tumor is the cystic-nodular type with the similar imaging appearance to a pilocytic astrocytoma and hemangioblastoma. Reviewing the literature, the majority of the brainstem gangliogliomas occurred in children, while hemangioblastomas are more frequent in adults [1,3,4]. This difference of predilection age might be helpful in suggesting brainstem ganglioglioma in children. The pilocytic astrocytoma also has a tendency to occur in children, which makes it more difficult to differentiate it with a ganglioglioma. However, the latter with predilection site of the lower brainstem should be observed in preoperative diagnosis. In our opinion, when this type of mass occurs in a child, especially the sac-nodular type, located in the lower brainstem with a long-term history and benign tumor characteristics on imaging, the possibility of brainstem ganglioglioma should be included in the differential diagnosis. As regards ganglioglioma treatment, extensive radical resection showed a good prognosis in the supratentorial region [1]. According to Lang et al., for patients with supratentorial ganglioglioma who accepted total resection, the 10-year survival rate reached 85% [3,5]. As for the brainstem ganglioglioma, due to its close proximity to the vital center, currently the subtotal or partial excision is the main surgical choice as opposed to total resection, for the main purpose of removing the protruding tumoral tissues from brainstem, particularly the enhanced solid tumoral portion on MRI. Concerning partial resection of the tumor, prognosis is relatively good according to the literature, with a 5-year survival rate of 78% [5]. The postoperative radiotherapeutic effect of ganglioglioma still remains controversial as regards its insensitivity to radiation, radiant adverse effects in children, and potential malignant tendency. However, some authors also believe that radiotherapy has a certain effect in controlling residual tumor after subtotal resection. As reported in some series, chemotherapy has limited value in ganglioglioma treatment, and may cause serious complications such as blood disorders [3,6]. Currently, chemotherapy or radiotherapy is chosen in cases of tumor progression or recurrence as an adjuvant therapy. 3. Conclusion The brainstem ganglioglioma is a rare benign tumor. Based on a review of the literature and our particular case, we consider that the medulla oblongata and lower part of pons might be predilection sites. The brainstem ganglioglioma is difficult to differentiate from other low-grade tumors based on the imaging alone. Therefore, we suggest that when a tumor located in the lower brainstem with benign radiological characteristics occurs in a child with a longterm history, the possibility of brainstem ganglioglioma should be considered in the preoperative diagnosis along with other lowgrade neoplasms. Partial resection is still the ideal therapy for brainstem ganglioglioma with main purpose of removing the tumor portion protruding from brainstem. The brainstem ganglioglioma might invade the cerebellopontine angle region via the lateral foramens of the 4th ventricle, and therefore being aware of the extending area on imaging is very valuable for neurosurgeons in order to create an effective surgical program. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.
X. Anqi et al. / Neurochirurgie 61 (2015) 50–53
References [1] Lagares A, Gómez PA, Lobato RD, Ricoy JR, Ramos A, de la Lama A. Ganglioglioma of the brainstem: report of three cases and review of the literature. Surg Neurol 2001;56(5):315–22. [2] Mpairamidis E, Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N. Brainstem ganglioglioma. J Child Neurol 2008;23(12):1481–3. [3] Zhang S, Wang X, Liu X, Ju Y, Hui X. Brainstem gangliogliomas: a retrospective series. J Neurosurg 2013;118(4):884–8.
53
[4] Kanno H, Kuratsu J, Nishikawa R, Mishima K, Natsume A, Wakabayashi T, et al. Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease. Acta Neurochir (Wien) 2013;155(1):1–7. [5] Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Ab- bott IR, et al. Central nervous system gangliogliomas. Part 2: clinical outcome. J Neurosurg 1993;79: 867–73. [6] Baussard B, Di Rocco F, Garnett MR, Boddaert N, Lellouch-Tubiana A, Grill J, et al. Pediatric infratentorial gangliogliomas: a retrospective series. J Neurosurg 2007;107(4 suppl.):286–91.
