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Report 2013: Tumors of the pineal region
Neuroendoscopy and pineal tumors: A review of the literature and our considerations regarding its utility Neuroendoscopie et tumeurs pinéales : revue de la littérature et considérations sur son utilité C. Mottolese ∗ , A. Szathamari , P.A. Beuriat , B. Grassiot , E. Simon Neurological and neurosurgical hospital P.-Wertheimer, 59, boulevard Pinel, 69677 Bron, France
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Article history: Received 29 March 2013 Received in revised form 28 November 2013 Accepted 8 December 2013 Available online xxx
a b s t r a c t Endoscopy has entered into the armamentarium of pineal and pineal region tumor treatment. The technique permits not only to control hydrocephalus but also to obtain tissue samples for histological diagnosis. In this paper, we explain the utility of endoscopy for the treatment of pineal tumors and as well as report some personal considerations regarding this topic. © 2014 Elsevier Masson SAS. All rights reserved.
Keywords: Pineal tumors Hydrocephaly Endoscopy Biopsy Endoscopic third ventriculostomy
r é s u m é Mots clés : Tumeurs pinéale Hydrocéphalie Endoscopie Biopsie Ventriculocisternostomie endoscopique
L’endoscopie fait partie de l’arsenal thérapeutique des tumeurs de la glande et de la région pinéale. Avec cette technique, il est possible de contrôler l’hydrocéphalie et de pratiquer des biopsies pour le diagnostic histologique. Notre objectif est d’évaluer l’utilité de l’endoscopie dans le traitement des tumeurs de la glande et de la région pinéale. © 2014 Elsevier Masson SAS. Tous droits réservés.
1. Introduction Neuroendoscopic procedures in the treatment of pineal tumors have a great advantage in dealing with tissue sampling, gross morphological analysis of tumors, ventricular and cistern structures and the treatment of hydrocephalus. The neuroendoscopic treatment of cerebral tumors starts with the approach to the ventricular system to open the floor of the third ventricle and to explore the posterior part of the ventricle for a biopsy or for a treatment that is becoming increasingly popular [1–3] (Fig. 1).
∗ Corresponding author. E-mail address:
[email protected] (C. Mottolese).
Historically, Guyot was the first to report on the use of ventriculoscopy to treat a colloid cyst [4]. Fukushima reported the first modern description of the endoscopic technique introducing a ventriculofiberscope into the ventricular system [5]. The technical improvement with the introduction of new endoscopes with a better power of vision have imposed neuroendoscopy as a modern tool to treat pineal region tumors with a mini-invasive technique. This procedure must be used in patients with hydrocephalus because of the difficulties in approaching the ventricles without dilatation. The usefulness of endoscopy is based on the fact that intraventricular CSF serves as an excellent natural medium for light and image transmission. Patients with hydrocephalus are better candidates for this technique [6]. Approximately 90% of patients with pineal tumors have hydrocephalus at the time of presentation [7–9]. However, the absence of hydrocephalus does not
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Please cite this article in press as: Mottolese C, et al. Neuroendoscopy and pineal tumors: A review of the literature and our considerations regarding its utility. Neurochirurgie (2014), http://dx.doi.org/10.1016/j.neuchi.2013.12.008
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Figs. 1–2. Cystic pineal lesion on MRI T1 sequence before (1) and after (2) surgery with endoscopic fenestration. Lésion kystique pinéale en coupe sagittale en IRM, séquence T1, avant (1) et après (2) chirurgie par fenestration endoscopique.
contraindicate the use of endoscopy for the management or surgical biopsy of pineal tumors or pineal region tumors. Yamamoto et al. reported their experience with a flexible endoscope and Sooweidane and Luther reported a series of patients with biopsy without hydrocephalus [10,11]. Grunert et al. reported their first experience on patients with posterior third ventricle lesion stressing the difficulty in navigating through the posterior part of the ventricle [12]. In our experience, in the absence of ventricular dilatation, the endoscope insertion guided by neuronavigation decreases the rate of complication improving clinical results and making the approach to the lesion easier. Histological diagnosis is essential for the therapeutic program. The stereotaxic frame biopsy can be used to establish a diagnosis of pineal tumors even in the presence of venous hemorrhagic risk [13,14] whereas endoscopy offers multiple advantages such as: • possibility of preventing problems with deep ventricular structures by avoiding the deep venous system; • possibility of exploring the ventricular cavity rendering the staging of the disease more precise and therefore enabling the discovery of any metastasis; • possibility of obtaining a total removal in some cases by controlling the hemostasis in less vascularized lesions; • possibility of treating associated cysts or performing a septostomy or at the end treating hydrocephalus. (Figs. 1 and 2); • possibility to collect CSF for biochemical and cytological analysis and for research of tumor markers: the presence of alphafetoprotein and beta-human gonadotropin in serum and CSF are sufficient to diagnose a malignant germ cell tumor; • possibility to avoid highly vascularized part of the tumors choosing the zone to carry out the biopsy reducing the risk of hemorrhage. Definitively also if the presence of hydrocephalus seems associated with better results and a lower rate of complication, the absence of hydrocephalus does not contraindicate the use of endoscopy for the management or surgical biopsy of pineal tumors or pineal region tumors. As reported by Wong et al., endoscopy for pineal tumors can be selectively applied for endoscopic third ventriculostomy (ETV), endoscopic biopsy, and endoscopic assisted radical neurosurgical resection or cyst fenestration [15]. In some cases, with a bifocal localization, an endoscopic transnasal trans-sphenoidal approach can be useful to reach a diagnosis with the biopsy of an intraselar specimen. The advantage of endoscopic biopsy is that it provides histological evidence to make the diagnosis, permitting the planning of an appropriate treatment and avoiding opened surgery in cases of
germ cell tumors. Moreover, the histological diagnosis can confirm the direct approach for tumor removal for cases in which the radical removal could guarantee the cure. The histological type of pineal tumors that require a radical removal either after endoscopic inspection or after endoscopic biopsy, are those that have a benign evolution such as pinealocytomas or benign astrocytoma or pineal cysts. An important argument stressing the advantage of endoscopy is the significant reduction of costs related to a reduction in hospital stay as the accuracy of endoscopic biopsy has been well established [2,16,17]. For many years, endoscopy has been emerging as the preferred initial management for pineal lesions or pineal region tumors [7]. The theoretical risk of leptomeningeal dissemination related to the communication between the ventricular space and the leptomeningeal system has also been evoked in some studies reported in the literature [18]. This may be related to the manipulation of tumors that are debulked with or without a complete removal and primarily reported with metastatic lesions of the posterior part of the third ventricle with a rate of dissemination between 5 and 20% [18]. The possibility of underestimating metastasis is now less possible because in many protocols, mainly in pediatric pathology, the routine evaluation with cranio-medullary MRI increases the rate of a good staging of the disease. The use of multiple trajectories and a partial endoscopic removal seems to be implicated in the increased rate of metastatic dissemination. However, the study of Luther et al. does not appear to show an increased incidence of metastasis when an ETV is coupled with a pineal tumor biopsy (Luther, 2012). 2. Endoscopic third ventriculostomy (ETV) In ETV, for the treatment of hydrocephalus, the use of a flexible endoscope or rigid endoscope is discussed. At the beginning of our experience, we used a flexible endoscope that had the advantage of being thinner and therefore could facilitate the navigation in the ventricular system, particularly the inspection and the visualization of the posterior part of the third ventricle with the tumor. The problem with a flexible endoscope is the luminosity and the quality of the image that can be very disconcerting mainly in cases of bleeding because the hemorrhagic CSF does not facilitate a good vision, and lot of time is wasted before the hemorrhage is stopped. A biopsy with a rigid endoscope is preferable for luminosity and for the quality of pictures but greater care must be taken in order to avoid lesions to the venous system mainly at the level of the foramen of Monro. As regards this point, traction can facilitate bleeding of the cerebral internal or thalamo-striate vein. The size of histological samples is also important for the diagnosis, although
Please cite this article in press as: Mottolese C, et al. Neuroendoscopy and pineal tumors: A review of the literature and our considerations regarding its utility. Neurochirurgie (2014), http://dx.doi.org/10.1016/j.neuchi.2013.12.008
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Fig. 3. Peroperative endoscopic view of a pineal lesion after 180◦ rotation of the endoscope at the level of the 3rd ventricle. *: floor of the 3rd ventricle; §: cystic pineal lesion. Aspect peropératoire sous endoscopie d’une lésion cystique de la pinéale après une rotation de 180◦ dans le 3e ventricule. * : plancher du 3e ventricule ; § : lésion cystique de la pinéale.
an exophytic development of the lesion inside the third ventricle would facilitate larger samples. Also, a lesion remaining under the ependymal tissue would make the diagnosis more difficult. In the literature, the reports of biopsy success rates vary between 67 to 90% of cases [19–23] and the possibility to obtain multiple biopsy specimens from different zones of the tumors represent a real advantage of the endoscopic technique. With a flexible endoscope, Oppido et al. have reported a rate of histological diagnosis in 90% of intraventricular tumors [15,22,24–26]. The optimal position for the placement of a burr hole depends on the morphology of the ventricular system and on the shape the foramen of Monro. Some authors propose performing a biopsy or an ETV using a single trajectory as proposed by O’Brien et al. in 2006 [25]. To avoid the failure of histological diagnosis, it is recommended to carry out the burr hole permitting a penetration of the lateral ventricle more anterior, allowing the entrance of the third ventricle in a plane parallel to the choroid plane and to have a better visualization of the lesion, with the endoscope perpendicular to it. This creates a satisfactory trajectory to gain the Monro hole, the anterior part of the floor of the third ventricle and consequently the posterior part of the ventricle where the pineal tumors develop (Fig. 3). It is useful to remember that an important task is performing the ventriculostomy at the floor of the third ventricle to treat the hydrocephalus. Therefore, the ETV is performed before the biopsy to avoid problems in the event of bleeding. We use the endoscopic arm to avoid movements of the endoscope that can be responsible for major venous bleeding. The ETV is performed with a bipolar coagulator and it is enlarged with a Fogarty balloon catheter or with scissors to create a large communication and consequently a good flow form the ventricle to the cisternal system. Some authors have proposed the use of a laser to open the floor but as reported by Robinson and Cohen, it can be responsible for injury to the basilar artery that lies under the thin tuber cinereum [7]. We use the 30◦ lens endoscope that produce a larger plane of vision. The endoscope can facilitate the inspection
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of the third ventricle and in this way the discovery of metastatic localization can ameliorate the staging of the disease, permitting to adapt the complementary treatment. Once the stoma is completed and the flow is effective, the endoscope is pushed slowly posteriorly remembering to avoid the stretching of the venous system at level of the foramen of Monro in order to avoid any hemorrhage. The third ventriculostomy avoids the need of a shunt in 60% to 80% of patients with a long-term success rate. Yamini et al. reported an associated patency rate of 85% in 33 patients with a triventricular hydrocephaly induced by a compression of the aqueduct [8]. Once the endoscope is inserted posteriorly, we turn it 180◦ to access the lesion with a more direct vision. As soon as the lesion is viewed, staged biopsies are performed and rapidly send to the pathologist, for tumor cell culture and microscopic analysis. For small lesions without a major vascular component, endoscopic removal can be achieved using the endoscopic ultrasonic aspirator or bipolar forceps for coagulation. The main problem, in our experience, is to avoid lesions to the venous system, particularly the vein of Galen or to the cerebral internal veins. In cases of pineal tumor biopsies, the increased release of protein can induce CSF absorption disorders with a consequent hydrocephalus that may need a shunt as reported by Yamini et al. [8]. In cases of bifocal germ cell tumors the endoscopic approach has been proposed via an endonasal route by some authors but never performed in our experience. In these cases, the biopsy has always been performed after the ETV. In some cases with acute loss of vision, a direct approach can be indicated. We were confronted to this situation in two cases and the rapid decompression of the optic nerves and optic chiasm favored a quick recovery of vision, before any complementary treatment.
3. Biopsy and histological results The evolution of endoscopic technique and the possibility to control the hemorrhage with bipolar coagulator forceps has contributed to the successful evolution of the endoscopic biopsy for pineal tumors associated with hydrocephaly. The only exception would be in cases of highly vascularized tumors and in this situation, it is recommended to not carry-out a biopsy [1,9,27,28]. The endoscopic treatment of hydrocephalus is now the recommended option because it has been recognized as being superior to the external ventricular drain as it reduces the rate of infectious complications and avoids the risks of an internal device. As previously reported by Pople et al., the possibility of histological errors does not disqualify the method of primary neuroendoscopic ventriculostomy for biopsy [21,29]. The histological diagnosis for pineal tumors, as for other intraventricular lesions, depends on the quality and size of the tissue samples. Consequently, the endoscopic technique for biopsy can be associated with a percentage of errors because the specimen can be too small therefore not permitting a diagnosis (Figs. 4 and 5). The accuracy of endoscopic histological diagnosis varies between 61% to 100% [7–9,15,24,25,29,30]. The endoscopic biopsy is very useful in cases of primary germ cell intraventricular tumors because in these cases a direct approach for complete removal is not necessary. In the series of Wong et al., an histological diagnosis was possible in 84% of cases and when the diagnosis was not possible the specimens showed a clot or a simple gliosis [15]. For Pople et al., the rate of success was of 94%, 32 of 34 cases, whereas for Depreitere, the rate of success diagnosis was 69% [21,28]. For Morgensten et al., the diagnostic efficacy was 80% without complications [9]. The reasons for non-diagnosis can be related to insufficient tissue sampling, the difficulty in exposition of the target or the
Please cite this article in press as: Mottolese C, et al. Neuroendoscopy and pineal tumors: A review of the literature and our considerations regarding its utility. Neurochirurgie (2014), http://dx.doi.org/10.1016/j.neuchi.2013.12.008
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Figs. 4–5. Intraoperative aspect of endoscopic biopsy before (4) and after sampling with the view of a fragment of the tumoral tissue (5). Aspect peropératoire d’une biopsie avant (4) et après prélèvement avec visualisation du fragment tumoral(5).
hemorrhage [21]. The rigid endoscope permits the use of larger instruments and subsequently the possibility of obtaining a greater amount of biopsy specimens without increased morbidity or mortality [30]. It has to be remembered that differences exists between the histological answer obtained at moment of the biopsy and the definitive histological diagnosis when a direct approach is performed [28]. The rate of hemorrhagic complications varies between 12 to 20% [8,15]. In our experience, we were never forced to stop the surgical procedure for an hemorrhagic complication but in 20% of cases, the diagnosis was impossible because the samples of tissue were judged too small by pathologists. The possibility of dissemination of tumoral cells during the endoscopic procedure or at the level of the endoscopic channel have been reported stressing the potential risk of dissemination after the biopsy followed by an ETV [18,23]. We never observed this situation and we think that this can be related to a technical error, i.e. the opening of biopsy forceps could theoretically be responsible for a dissemination of pathological tissue. Oi and Matsumoto stressed this possibility but they were never confronted with the problem of tumor dissemination [20] as for Shono et al. in 8 patients [31]. Luther reported two out of 22 patients with tumors classified as being at high risk of dissemination [18]. The incidence of dissemination after an endoscopic biopsy was of 6.8% according the national Japanese experience [32]. The risk of metastasis during the surgical endoscopic procedure stresses the importance of a complete radiological preoperative investigation with a cerebral and spinal MRI that must be completed before any surgical decision is made in order to have the exact staging of the disease both in children and adults. If an endoscopic removal has been programmed with an intraventricular approach, the main problem is represented by the size of the tumor and its vascularization. For Gaab and Schroder, the ideal size for removal is of 2 cm or less, while for Oi and Matsumoto, the tumor can be larger because they generally become symptomatic when their size is superior to 2 cm, producing an obstruction of the Sylvius aqueduct [20,26,30]. The vascular structure of tumors is of great importance. In our opinion, even with the possibility to use bipolar forceps, hemostasis can be very difficult to carry out. 4. Microsurgical and endoscopic assisted resection The possibility of using an assisted endoscopic technique during microsurgical resection of tumors has previously been reported [11,16,33]. During the microsurgical procedure, the use of an endoscope will permit the visualization of tumor specimens in the blind operative corners that can be removed either with the help of the endoscope or with the microscope. Normally at the end of the microsurgical removal of a pineal tumor, the
exploration of blind corners have to be the final task before the definitive closure but this strategy is not specific to surgery of pineal tumors. The endoscopic inspection of the operative field increases the rate of radical removal that is always an important prognostic factor for the cure of patients with a cerebral tumor. The complete endoscopic intraventricular removal of pineal tumors is possible as reported by Gaab and Oi but in our experience, this can be carried-out in particular cases of small size and poorly vascularized tumors. Pure endoscopic resection of pineal tumors through the arachnoidal space has also been proposed using a suboccipital or interhemispheric approach as recently reported in the literature [16] or by a supracerebellar infratentorial route [34]. This approach can be reserved for particular cases. However, generally we prefer microsurgical resection assisted by endoscopy.
5. Conclusion The review of literature shows that the endoscopic technique is very useful for treatment of hydrocephalus as well as pineal region lesions, and to establish a diagnosis with a biopsy before deciding the specific treatment strategy to adopt for each lesion. The possibility of removing pineal lesions with a basic endoscopic technique can be reserved for particular small lesions that can be approached via a transventricular root. The indication of endoscopic technique through the arachnoid space can be reserved for the same small non-highly vascularized lesions. However, in fact, the risk and the control of hemorrhagic complications seems more severe than that imposed by a microsurgical approach. The possibility of microsurgical removal assisted by the endoscopic technique is currently a well-established surgical program to increase the rate of total removal for pineal tumors. This technique has been shown to be also useful for other ventricular or extra-cerebral lesions.
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Please cite this article in press as: Mottolese C, et al. Neuroendoscopy and pineal tumors: A review of the literature and our considerations regarding its utility. Neurochirurgie (2014), http://dx.doi.org/10.1016/j.neuchi.2013.12.008