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Table of Contents
ORIGINAL ARTICLE
Year : 2019  |  Volume : 2  |  Issue : 1  |  Page : 10-15

Clinical management and prognostic outcome of intracranial ventricular tumors: A study of 134 cases


1 Department of Neurosurgery, University of Hyderabad, Hyderabad, Telangana, India
2 Department of Biotechnology and Bioinformatics, School of Life Sciences, University of Hyderabad, Hyderabad, Telangana, India
3 Department of Pathology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India

Date of Web Publication9-Sep-2019

Correspondence Address:
Phanithi Prakash Babu
doupt CRST_17_19F-23/ 71, Department of Biotechnology and Bioinformatics, School of Life Sciences, University of Hyderabad, Hyderabad - 500 046, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CRST.CRST_19_19

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  Abstract 


Introduction: Ventricular tumors are known to have a relatively rare occurrence and are underlined by complex pathophysiology.
Methods: We retrospectively analyzed the clinicopathological details of 134 patients with intracranial ventricular lesions over a 5-year period. The patients were selected on the basis of diagnosed lesions by magnetic resonance imaging and further histopathology study. We have assessed the clinicopathological details from the database.
Results: The median age of patients was 30 years (range: 1–73 years). In 47% of the cases, the lesions were located in the anterior third ventricle; glioma (n = 43) and colloidal cysts (n = 41) were the most common lesions. Presenting symptoms included seizures and features of hydrocephalus in 38.8% of patients. Open microsurgical (81.6%) and endoscopic (18.4%) surgery were performed. An open microsurgical approach was the main surgical treatment with operative mortality of 0.86% and permanent morbidity of 6.7%. Sixteen of the 27 patients in the endoscopic group underwent endoscopic third ventriculostomy (ETV) with biopsy. Survival was affected by the type of lesion. The progression-free survival was highest in the patients with neurocytoma and lowest in the patients with glioma (P = 0.01). Twenty-three patients received adjuvant radiation therapy. The decision regarding radiation therapy was made on the basis of individual lesion, histopathological type, and proliferative index. The mean overall survival was 34 months.
Conclusions: Management of intraventricular tumors requires multimodality treatment including open microscopic surgery, neuroendoscopy, and radiation therapy as per the tumor pathology and hydrocephalus status. Simultaneous endoscopic biopsy with ETV can avoid major open surgery and mitigate the possible postsurgical complications.

Keywords: Cysts, glioma, open microsurgery, third ventricle, ventricular tumor


How to cite this article:
Kriankumar C K, Deshpande RP, K. Chandrasekhar Y B, Rao I S, Panigrahi M, Babu PP. Clinical management and prognostic outcome of intracranial ventricular tumors: A study of 134 cases. Cancer Res Stat Treat 2019;2:10-5

How to cite this URL:
Kriankumar C K, Deshpande RP, K. Chandrasekhar Y B, Rao I S, Panigrahi M, Babu PP. Clinical management and prognostic outcome of intracranial ventricular tumors: A study of 134 cases. Cancer Res Stat Treat [serial online] 2019 [cited 2019 Nov 21];2:10-5. Available from: http://www.crstonline.com/text.asp?2019/2/1/10/266443




  Introduction Top


Ventricular tumors usually have a complex pathology and heterogeneous outcomes both in pediatric patients and in adults. Tumors located anatomically close to the ventricular system are relatively rare. They are phenotypically slow growing, making the symptoms appear gradually and thus complicating the therapeutic course. Primary ventricular tumors originate in the ventricular walls and then extend to the ventricular system, whereas the periventricular tumors originate in the anatomical location adjacent to the ventricular system. Patients usually present with symptoms such as headache, vomiting, seizures, and motor weakness. The symptoms are generally nonspecific as they arise from the increased intracranial pressure.

Ventricular tumors are generally benign. These tumors are known to arise from a variety of cells and often cause obstruction to the movement of cerebrospinal fluid (CSF) in the brain. Thus, they differ from other tumors on account of their anatomic location, the pathology of migration, and symptoms, which arise due to malfunction or obstruction of the regions in which they arise.[1],[2],[3] The most common ventricular lesions include ependymal lesions, glioma, meningioma, colloid cysts, and choroid plexus papilloma.[4],[5] In the pediatric population, the tumors of the lateral ventricle include giant cell astrocytoma, ependymoma, and choroid plexus papilloma.[6]

Surgical approaches, mainly transcallosal or transcortical, are the standard of care. The choice of surgical resection depends on the type, size, and location of the lesions; size of the ventricles; and the anatomic proximity with the adjacent regions. The type of surgery affects clinical morbidity and mortality.[7]

In the present study, we describe the clinicopathological profile of 134 cases with ventricular lesions. Further, we elaborate the type and suitability of surgical approaches based on the prognostic outcomes and comment on the surgical complications and the remedial measures undertaken.


  Methods Top


Patient population

The database of patients was maintained (2013–2017) at the Krishna Institute of Medical Sciences in the Department of Neurosurgery, Telangana State, India. This is a tertiary care center, and patients are often referred for neurosurgical service. The patients diagnosed by magnetic resonance imaging (MRI) and further histopathological staining were included in the study, using the search term 'ventricular lesions', in the database. Each patient was assigned a unique number, and the database was completely anonymized. The clinicopathological details including age, sex, presenting symptoms, and type of treatment were noted. The location of the tumor was initially determined by MRI. The type of surgical resection was determined by the neurosurgeon, and the decision was based on the type of lesion, location of lesion, MRI characteristics, hydrocephalus status, and the health of the patients. Informed consent was obtained from participants for the performance of surgery and for inclusion in the study. The study was approved by the institutional ethical committee board, renewed registration No. KFRC/2017/62-04.

Tumor management, histopathology, and follow-up

Based on the initial diagnosis by MRI, the surgery was performed. Tumor location was categorized into three subtypes: lateral ventricle, anterior third ventricle, and posterior third ventricle. The type of surgery was broadly divided into two groups: open microsurgical or endoscopic surgery. Depending on the extent of resection, the type of surgical removal was classified as gross total, near-total, or partial resection. The resected tissue was subjected to further histopathological examination. The MIB-1 index was calculated to evaluate the degree of proliferation. Patients were monitored for complications including seizures, hydrocephalus, intraventricular hemorrhage, and memory impairment. The complications were managed by ventriculoperitoneal shunt, reexploration, or temporary CSF diversion. After surgery, patients were monitored for recurrence and immediate postoperative complications; initially at 3 months and 6 months and then every year. The monitoring was done at each assigned visit by clinical evaluation and MRI.

Statistical analysis

The statistical analysis was performed using Sigma Plot software (San Jose, CA, USA) and Graph Pad Prism (Graph Pad software San Diago, CA). Clinical data were presented in absolute numbers and percentages. Progression-free survival (PFS) was defined as follow-up period for which no disease symptoms were observed. Overall survival (OS) was defined as total available follow-up period for each patient. The follow-up was started once the patients were diagnosed with the lesions by MRI till the date of event. Patients who could not be contacted for over 6 months were considered lost to follow-up.

Kaplan–Meier method was employed to calculate the PFS and OS in the total cohort. The difference in survival between the various groups of patients categorized by the histologic subtypes and the grade of the tumor was evaluated by the log-rank test. The difference between the survival patterns among groups as represented by the type of lesions was analyzed by one-way ANOVA. P <0.05 was considered statistically significant.


  Results Top


Clinicopathological features

The clinicopathological features of the patients diagnosed with ventricular lesions are described in [Table 1]. The median age was 30 years, range 1–73 years. The initial diagnosis and location of the lesion were based on the MRI and further confirmed by histopathology [Table 2]. Headache was the most common presenting symptom in 58% of patients. The most common lesions were glial tumors in 43 (32.1%) cases followed by the colloidal cysts in 41 (31.3%) cases and craniopharyngioma in 6 (4.5%) cases, whereas ependymal cysts and meningioma were observed in 5 (3.7%) cases each. Glioma and the colloidal cysts were mostly observed in the adult population, whereas craniopharyngioma, germ cell tumors, and choroid plexus neoplasms were noted in the pediatric population [Table 2].
Table 1: Clinical profile of ventricular lesions

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Table 2: Pathological profile of intraventricular tumors

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Surgical management

Surgical approaches were either open microsurgical or endoscopic and were dependent on the anatomical location of the tumor. In total, 116 cases were operated by the open microsurgical approach, whereas 27 cases were by endoscopic procedure; some patients underwent more than one surgery [Table 3]. In most of the cases where the lesions were located at the lateral ventricle and anterior and posterior third ventricle, the open microsurgical approach was practiced. The types of surgical excisions were gross total, near-total, subtotal, or biopsy [Table 4]. Gross total resection was performed for the lesions that were located at all the anatomic locations (lateral ventricle and anterior and posterior third ventricle). When the lesion was at posterior third ventricle, near-total resection was performed most commonly followed by the gross total resection (46.7%). [Figure 3] shows the pre- and postoperative image of an oligodendroglial tumor in the ventricle.
Table 3: Tumor location and surgical strategy used

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Table 4: Extent of excision of the ventricular lesions and the associated anatomical location

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Figure 3: Representative magnetic resonance imaging image (a) with a visible tumor. (b) Postoperative image taken after 3 months. The patient was subjected to complete tumor excision. (c) Histopathological characterization of the surgically resected tumor as oligodendroglioma

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Hospital stay

The average and median postoperative stay in the hospital were 5.8 and 5 days, respectively (ranging from 2 to 17 days).

Postoperative complications

Complications were more commonly seen with the open microsurgical approach than with the endoscopic approach. Postoperatively, 9 (6.7%) patients had intraventricular hemorrhage, which resolved with conservative management. Two patients had seizures, hydrocephalus, meningitis, transient paranoid syndrome, and intracerebral hematoma subsequent to ventricular puncture in one patient. Among the total cases, 11 (9.3%) open microscopic and 2 (7.6%) patients who underwent the endoscopic approach developed seizures. Four (15.3%) of the endoscopic cases developed hydrocephalus and intracranial hemorrhage postoperatively. In 26 (71%) cases with open microsurgical complications, no additional treatment was required, while 5 (10%) patients were operated by temporary CSF diversion, 4 (8.1%) had ventriculoperitoneal shunt done, and 1 (2%) required reoperation [Table 5]. In the 41 patients with colloidal cysts, only 2 (4.9%) patients developed significant postsurgical deficit.
Table 5: Surgical complications and the remedial measures in the surgical approaches

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On follow-up, 6 (11.2%) patients required ventriculoperitoneal shunt. We did not observe any mortality or permanent morbidity.

Adjuvant radiotherapy

Patients were postsurgically treated with radiotherapy in high-grade astrocytoma and progressive lesions. Thirty-seven percent of patients were treated with adjuvant radiotherapy. The adjuvant radiotherapy was given by external beam radiation with 59.4 Gy dose in 33 fractions.

Prognosis and survival outcomes

At a mean follow-up of 34.6 months, 103 patients are alive and disease-free, 14 patients have recurred, 7 patients are alive with recurrent disease, 5 patients have died, and 5 patients are lost to follow-up [Figure 1] and [Figure 2]. There were no mortalities reported in the eleven patients with central neurocytoma, in the seven patients with pilocytic astrocytoma, and in the nine patients with pineal parenchymal tumors. The maximum number of deaths occurred in the patients with high-grade astrocytoma, followed by the oligodendroglial tumors [Figure 1]. Mortality was noted in astrocytoma cases (23%), but not in neurocytoma cases. Recurrent tumor was noted in three astrocytoma patients and was treated by radiotherapy. Among the nine pineal parenchymal tumors, five were subjected to postoperative radiotherapy, and there was no mortality reported in this group.
Figure 1: Overall survival of the patients with the ventricular lesions. There was a statistically significant difference among the groups. Log-rank (Mantel-Cox) test was used to estimate the significance (P = 0.01)

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Figure 2: Progression-free survival in patients with the ventricular tumors (P < 0.05)

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The median PFS was 33.5 months and median OS was 34 months.


  Discussion Top


In the present study, we report the pathology, management, and prognostic outcomes of 134 patients with ventricular tumors. The patients were initially diagnosed by MRI imaging and were confirmed by histopathological diagnosis. The median age was 30 years, range 1–73 years, and tumors occurred with near equal frequency in males and females. Headache and vomiting were the most commonly reported symptoms. The most common pathologies were glioma and colloidal cysts, and the most common anatomical location was the anterior third ventricle. Surgical management was done by open microsurgical and endoscopic operations. Tumor pathology and hydrocephalus status were the determining factors for surgical approaches. Open microsurgical approach was most suitable for the ventricular lesions with significantly less morbidity.

In the literature, ventricular tumors have been noted to have a complex pathophysiology, can be benign or malignant, and respond differently to therapeutic approaches.[8] The presenting symptoms with ventricular lesions are caused by ventricular dilatation and intracranial hypertension.[9] The surgical resection of the lesions is often fraught with significant complications and postoperative morbidity.[10]

In our study, we found more adults with ventricular tumors, followed by pediatric cases (25%). An earlier study reported the incidence in the pediatric population to be around 15%.[11] The tumors were distributed equally among men and women, with majority located at the anterior third ventricle. In 47% cases, the tumors were located in the third ventricle and 26% of cases at the lateral ventricle. The most common location at the third ventricle has also been reported in the earlier literature.[12],[13] Headache, vomiting, and visual disturbances were the most common presenting symptoms and are supported by the previous observations.[10] Glial tumors were observed most commonly (32.1%) followed by the colloidal cysts (31.3%); this is similar to what has already been reported in the literature.[10] An open microscopic or endoscopic approach was generally used for the surgical resection. The endoscopic approach was used to maintain proper CSF circulation, circumventing the requirement for a permanent shunt. Open microsurgical approach was most commonly practiced. In the literature, there is a scarcity of information on the clinical management and prognostic details of ventricular tumors. However, it was noted that when the tumor was located in the lateral position, the interhemispheric transcallosal approach is most suitable.[9] A nationwide study conducted in Japan demonstrated that neuroendoscopic approaches for hydrocephalus-associated ventricular tumors significantly enhanced the quality of postsurgical living in patients.[12] There are few studies from India that document the histological and radiological characterization and management of the ventricular tumors in children and report to be benefited by the microsurgical technique.[14],[15]

To our knowledge, this is the first study in India documenting the pathological pattern in a large number of patients with meticulous open microsurgical and endoscopic approaches. The prognostic and survival outcomes validate the choice of surgical approach. Furthermore, the group of patients we studied is wide ranging. As ours is a tertiary care referral center, the numbers may not be completely representative of the spectrum of ventricular tumors elsewhere.


  Conclusions Top


Overall, our results show that intraventricular tumor surgery requires multimodality treatment, including open microscopic surgery, neuroendoscopy, and radiation therapy as per the tumor pathology and hydrocephalus status. The open microscopic approach is a safe and a definitive procedure that results in significant postoperative success as measured by less complications and morbidity. Endoscopic third ventriculostomy with biopsy can avoid permanent shunt in 88.8% of the patients with hydrocephalus.

Acknowledgment

The authors thank financial assistance from the Department of Science and Technology (DST-India) (Grant no: SB/EMEQ-257/2013, SR/CSRI/196/2016), the Department of Biotechnology (DBT-India) (Grant No. BT/PR18168/MED/29/1064/2016, BT/PR13111/MED/29/149/2009), and the Indian Council of Medical Research (ICMR-India) (30/2/2010-01710/SIC/PI/N/162) for lab funding DST CSRI- (SR/CSRI/196/2016) DBT- (DBT/04/0401/2017/01181). RDP is thankful to the Department of Biotechnology (DBT-India) (Award no: DBT JRF/2011-12/95) for student fellowship.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Chernov MF, Kamikawa S, Yamane F, Ishihara S, Kubo O, Hori T. Neurofiberscopic biopsy of tumors of the pineal region and posterior third ventricle: Indications, technique, complications, and results. Neurosurgery 2006;59:267-77.  Back to cited text no. 8
    
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Hassaneen W, Suki D, Salaskar AL, Levine NB, DeMonte F, Lang FF, et al. Immediate morbidity and mortality associated with transcallosal resection of tumors of the third ventricle. J Clin Neurosci 2010;17:830-6.  Back to cited text no. 10
    
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Krähenbühl AK, Baldauf J, Guhl S, Gaab MR, Schroeder HW. Endoscopic biopsy for intra – And paraventricular tumors: Rates of complications, mortality, and tumor cell dissemination. J Neurol Surg A Cent Eur Neurosurg 2016;77:93-101.  Back to cited text no. 13
    
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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]



 

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