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Intraventricular Craniopharyngiomas: Surgical Management and Outcome Analyses in 24 Cases
Key words Attachment grade - Extent of resection - Intraventricular craniopharyngiomas -
Abbreviations and Acronyms BMI: Body mass index CP: Craniopharyngioma GTR: Gross total removal IVC: Intraventricular craniopharyngioma MRI: Magnetic resonance imaging STR: Subtotal removal VF: Ventricular floor From the 1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; 2 Max-Planck-Institute of Experimental Medicine, Göttingen, Germany; and 3Department of Neuropathology, Beijing Neurosurgical Institute, Beijing, China To whom correspondence should be addressed: Song Lin, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2014) 82, 6:1209-1215. http://dx.doi.org/10.1016/j.wneu.2014.06.015 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2014 Elsevier Inc. All rights reserved.
- OBJECTIVE:
Pure intraventricular craniopharyngioma is a rare subtype of craniopharyngioma that attaches frequently to the hypothalamus. The main challenge in tumor removal is protection of hypothalamic structure. The aim of our study was to set up a feasible risk-evaluation approach to help the surgeons make an individual treatment plan.
- METHODS:
We reviewed retrospectively 24 patients with pure intraventricular craniopharyngioma who underwent surgical therapy. Third ventricular deformation and thalamic attachment of the tumor were assessed by preoperative magnetic resonance imaging and intraoperative inspection. Correlations between the outcome, extent of removal, and different attachment grades were analyzed.
- RESULTS:
Preoperative magnetic resonance imaging grade had a significant correlation with functional outcome. Attachment grade was significantly correlated with endocrine, functional, and radiologic outcome. In the gross total removal (GTR) group, significantly more newly developed endocrinologic deficits were observed compared with patients who underwent subtotal removal (STR). All 6 GTR cases with the greatest attachment grade (grade 2) acquired newly developed endocrine insufficiency.
- CONCLUSIONS:
Attachment and deformation grade evaluated based on our criteria are significantly correlated with postoperative outcome. GTR of tightly attached tumor is associated with worse endocrinologic, functional, and radiologic outcomes compared with STR. Hence, we suggest that individual surgical plans should be made according to the grade of tumor attachment and hypothalamic deformation. STR should used in tumors with a high grade to achieve a good long-term outcome and avoid severe postoperative sequelae.
INTRODUCTION Craniopharyngioma (CP) accounts for 2%e 4% of the intracranial neoplasms. Yasargil et al. (24) devided CPs into 6 subtypes: 1) purely intrasellar-infradiaphragmatic; 2) intra- and suprasellar, infra- and supradiaphragmatic; 3) supradiaphragmatic parachiasmatic, extraventricular; 4) intra- and extraventricular; 5) paraventricular; and 6) pure intraventricular craiopharyngioma (IVC). Pure IVC is strictly defined as a CP exclusively located in the third ventricle that does not penetrate the third ventricular floor (VF). The presence of an intact pituitary stalk and third VF differentiate pure IVC distinctly from CP in other locations. However, only 0.5%e11% of CPs are pure IVC. Because of its rarity, a systemic approach to this tumor is still needed (1-3).
METHODS Patient Population Between January 2007 and April 2012, 830 patients with CP underwent surgery in the neurosurgery department of Beijing Tiantan Hospital; among these, 24 patients presented with pure IVC. The postoperative follow-up was conducted in the first month, every 3 months during the first year, and annually thereafter. The mean follow-up period was 42 months (range, 11e58 months). Radiologic Assessment Exclusive intraventricular location was assured by the presence of a patent
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suprasellar cistern, a normal pituitary stalk, and the absence of sellar abnormality in magnetic resonance imaging (MRI) findings as described previously (2). Maximal diameters in the vertical, horizontal, and sagittal axis were determined as Dx, Dy, and Dz. Tumor volume was estimated approximately by the formula (Dx Dy Dz)/2. Gross total removal (GTR) was defined when the residual tumor was not visible on postoperative MRI scans or its volume was smaller than 5% of the preoperative volume. Postoperative radiologic examinations were performed within 1 week. Degree of Hypothalamus Deformation. Preoperative MRI was graded as follows: grade
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0, completely identifiable third ventricular walls without bulging of lamina terminalis; grade 1, (partially) identifiable third walls with bulging of lamina terminalis; and grade 2, severe deformation, or third ventricle and lamina terminalis were not identifiable (Figure 1). Degree of Postoperative Hypothalamic Damage. The degree of postoperative hypothalamic damage was defined with the help of a neuroradiologist, who graded the damage according to postoperative MRI
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findings as follows (Figure 1): grade 0, no hypothalamic damage (intact third VF); grade 1, slight hypothalamic damage (identifiable but not intact third VF); and grade 2, severe hypothalamic damage (third VF not identifiable). Attachment Grading Intraoperatively, the extent of tumor attachment to hypothalamus was graded based on surgeons’ inspection and confirmed by review of surgical video record after the surgery: grade 0, no visible
attachment to third ventricular wall or floor, and the tumor border was well defined; grade 1, tumor presented a pedicle or mild attachment to the anterior third VF and presented an otherwise well-dissectible boundary; and grade 2, tumor presented widely and with close attachment to third VF or/and wall. Clinical Evaluation Presenting symptoms, psychological, ophthalmologic, and endocrinologic status of the patients were analyzed preoperatively
Figure 1. Pre- and postoperative magnetic resonance imaging (MRI) grading: Left Preoperative MRI: Assessment of hypothalamic deformation. grade 0, third ventricular structures clear identifiable, without the lamina terminalis bulging; grade 1, Lamina terminalis bulging, identifiable third ventricular structure; Grade 2, severe deformation of third ventricle lamina terminalis not identifiable. Right Postoperative MRI: Grade 0, no hypothalamic damage (intact third ventricular floor); grade 1 slight hypothalamic damage (identifiable but not intact third ventricular floor); grade 2 severe hypothalamic damage (third ventricular floor not identifiable).
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and by each follow-up. Functional outcome was evaluated according to Fahlbusch et al. (5) into 5 categories: good (independent and without impairment); moderate (independent and with a certain degree of impairment); fair (partially dependent with functional disability); poor (totally dependency); or death at the time point of follow-up. For each patient, body mass index (BMI) was calculated and recorded. For adults, patients with a BMI between 25 and 29.9 were considered as overweight and patients with BMI >30 as obese. Endocrinologic Evaluation For evaluation of anterior pituitary function, basal plasma levels of cortisol, growth hormone, thyroid-stimulating hormone, triiodothyroxine (T3), thyroxine (T4), luteinizing hormone, follicle-stimulating hormone, prolactin, testosterone, estradiol, and progesterone were measured. Panhypopituitarism was defined when the secretion of all anterior pituitary hormones (except for prolactin) was deficient. Posterior pituitary function was assessed by monitoring fluid intake and urine output. Serum electrolyte balance was monitored postoperatively until it had reached the normal range. Treatment Strategy No patient received radiotherapy before the initial surgery. The operations were performed by senior surgeons in our institute. Four different transcranial approaches were applied according to the location of lesions in the third ventricle. The frontal transcallosal approach was adopted in 15 cases in which the lesions extended to the upper half of the third ventricle. After splitting the corpus callosum from the midline, we incised the septum pellucidum and the fornix to approach the tumors in 11 cases. In the other 4 cases, one of the lateral ventricles expended medially over the midline, so we entered the lateral ventricle and exposed the tumors through dilated foramen of Monro. The frontal transcortical-transventricular approach was applied in 2 cases in which one frontal horn of the lateral ventricle was significantly enlarged. The tumors were resected through the dilated foramen of Monro. In the other 7 cases, tumors were situated in anterior and lower half of the third ventricle and created a bulge the
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lamina terminalis. With the pterional approach or subfrontal approach, we incised the lamina terminalis to access the third ventricle. Three of these 7 tumors were solid, 2 were purely cystic, and the other 2 had both solid and cystic components. After evacuation of the cyst contents, cyst wall was separated from the third ventricle in the cystic tumors. External drainage was placed in the third ventricle postoperatively for 24e48 hours to prevent potential hydrocephalus, intraventricular hemorrhage, and intracranial hypertension. Statistics Statistical analyses were performed with the Statistical Package for Social Sciences (version 17.0, SPSS, Chicago, Illinois, USA). The Exact Pearson c2 test, Pearson correlation analysis, or 2-tailed Fisher exact test was applied to analyze the data. Differences were considered statistically significant at the P < 0.05 level.
Table 1. Characteristics of Patients Characteristic
No. (%)
Number of cases
24
Age, years Median
40.2
Range
15e61
M/F ratio
10.5:1
Presenting complaints on admission Headache
16 (66.7)
Ophthalmologic deficits
10 (41.7)
Sexual dysfunction or amenorrhoea 8 (33.3) Mental disturbances or drowsiness
8 (33.3)
Diabetes insipidus
5 (20.8)
Intracranial hypertension
3 (12.5)
Preoperative MRI grade Grade 0
2 (8.3)
Grade 1
12 (50)
Grade 2
10 (41.7)
Intraoperative attachment grade
RESULTS Clinical Features Clinical features of the patients are summarized in Table 1.
Grade 0
5 (20.8)
Grade 1
10 (41.7)
Grade 2
9 (37.5)
F, female; M, male; MRI, magnetic resonance imaging.
Radiologic Findings The diameter of the tumors ranged from 3.0 to 4.0 cm (mean, 3.64 cm). Rounded or elliptic pure solid tumor was found in 20 cases; 2 purely cystic and 2 basal solid/ upper cystic tumors were found. The solid tumors and the wall of cystic tumors demonstrated contrast enhancement in gadolinium-diethylene triamine pentaacetic acid MRI. Lateral ventricle dilation presented in 12 cases (50.0%). Total calcification was found in 11 cases (45.8%), 7 (29.2%) in the tumor mass, and 4 (16.7%) on the capsule. Tumor volume in this cohort ranged from 9 cm3 to 44.1 cm3 (mean, 19.7 cm3). Preoperative MRI Grading In 2 (8.3%) cases, no bulging of the lamina terminalis was observed (grade 0); in 12 (59%) cases, grade 1 was noted on preoperative MRI. In the other 10 (41.6%) cases, the third ventricle was deformed, and third ventricular border was not identifiable (grade 2).
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Degree of Attachment On the basis of the criteria described previously, 5 cases (20.8%) were evaluated as attachment grade 0, 10 cases (41.7%) as attachment grade 1, and 9 (37.5%) cases as attachment grade 2.
Histopathologic Findings All tumor specimens were reviewed by 2 neuropathologists from the pathology department. According to previous studies, the papillary CP was located predominantly intraventricularly whereas the majority of adamantinomatous type more frequently had a suprasellar or parasellar location (21). In children, most craniopharyngimoas were the adamantinomatous type, whereas in adults, both types were observed with nearly equal frequency (23). In our series, 41.7% papillary type and 58.3% adamantinomotous type were found in the 24 cases. The histopathologic pattern of the
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tumor did not show significant association with attachment grade.
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Surgical Outcomes Total removal was the intended for all the cases; however, only GTR was achieved in 19 (79.2%) of 24 cases. Subtotal removal (STR) was achieved in 5 cases (20.8%) because of a wide and close attachment to the hypothalamus (3/5), inadequate view of the tumor (1/5), and not dissectible fine capsule remnant (1/5). Mortality and Morbidity Mortality. No patient died within the first 3 months after surgery. At the date of last follow-up, 5 patients died. One patient died of severe endocrine dysregulation at 6 months. The other 4 patients experienced recurrence and died after conservative therapy or radiotherapy at 44 months, 12 months, 12 months, and 43 months, respectively. Morbidity. Two (8.3%) patients presented with complications in the first month after surgery (the early postoperative period). One patient developed mental disturbances and memory dysfunction. A seizure occurred in another patient and was controlled effectively afterward. Third ventricular drainage was performed in 15 cases. Hydrocephalus was completely resolved in all cases, and extubation was performed in 24e48 hours. Recurrence At the time point of last follow-up, 4 cases from the GTR group (4/19) and 2 from the STR (2/5) group experienced recurrence. The recurrence rates of GTR group and STR group did not differ significantly. Postoperative MRI Grading Patients with widely dispersed and strongly attached tumors (attachment grade 2) had significantly more severe damage than patients with tumors of attachment grade 0 and attachment grade 1 (P ¼ 0.026) (Table 1). Clinical Outcome Visual ability was improved in 7 cases, remained unchanged in 3 cases, and no deterioration was observed within 1 month after the surgery. Mean weight at the first follow-up was 24.2 kg (3.1 kg). Obesity and overweight were observed in 8.3% (2/
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Treatment of Residual and Recurrent Tumor In the 6 cases with recurrence, one patient underwent a second operation with good functional outcome. One patient with cystic tumor recurrence received stereotactic interstitial irradiation. One patient received radiosurgery, which failed to reduce the size of the tumor. The other 3 patients received conservative therapy because of severe hypothalamic dysregulation and poor health conditions. At last follow-up, the latter 4 patients died.
24) and in 54.2% (13/24) of the patients, respectively. Two patients experienced emaciation. Newly developed psychological disturbances were documented in 3 patients. Three months after surgery, the functional outcome of patients was evaluated and served as follow-up baseline. Both preoperative MRI grade and attachment grade were correlated significantly with functional outcome (Tables 2 and 3).
Endocrinologic Outcome Considering all endocrine functions, significant deterioration was observed after surgery (P < 0.001, McNemar test; Figure 2). The rate of patients who underwent GTR and developed diabetes insipidus was significantly greater than that of the STR group (P value of 0.047). Comparing the different attachment grades, we found that the rate of newly developed panhypopituitarism in the grade 2 group was significantly greater compared with the attachment grade 0. The deterioration rate of corticotroph function in the attachment grade 2 and grade 1 groups were significantly greater compared with the grade 0 group. Significantly more cases in grade 2 group developed diabetes insipidus and needed endocrine-replacement medication than in grade 0 and grade 1 groups (Table 3 and Figure 3). Remarkably, among all the 9 cases with attachment grade 2 tumors, only the 6 cases with GTR developed panhypopituitarism and corticotroph insufficiency.
DISCUSSION Preoperative and Intraoperative Evaluation The association of prognosis when tumor is attached to the third ventricular wall has been reported previously (14); however, because of the obscuring effect of the border between third VF and tumor capsule, the attachment of the tumor to the hypothalamus could not be evaluated preoperatively (16). Controversy still exists regarding the correlation between hypothalamic involvement visible in MRI findings and outcome (14). IVCs that seemed to be closely attached to hypothalamic walls on preoperative MRI often were found to have an intact capsule separating the tumor mass from third ventricular wall (15). In our study, the extent of tumor attachment was graded based on surgeons’ inspection and confirmed by review
Table 2. Association Preoperative MRI Grades with Functional Outcome Preoperative MRI Grade Characteristics
0
1
2
Total
Total
2
12
10
24
Good
2 (100)
8 (66.7)
0 (0)
10 (41.6)
Moderate
0 (0)
4 (33.3)
5 (50)
9 (37.5)
Fair
0 (0)
0 (0)
3 (30)
3 (1.3)
Poor
0 (0)
0 (0)
2 (20)
2 (8.3)
Mortatlities
0 (0)
0 (0)
0 (0)
0 (0)
Functional outcome, n (%)
P Value
0.014
MRI, magnetic resonance imaging.
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Table 3. Outcomes Associated with Attachment Grades Intraoperative Attachment Grade Characteristics Total Tumor volume, mean SD, cm
3
0
1
2
Total
P Value
5
10
9
24
20.6 10
15 5.3
24.3 10.6
19.7 9.3
0.053
Gross total removal, n (%)
5 (100)
8 (80)
6 (66.7)
19 (79.2)
0.386
Recurrence, n (%)
1 (16.7)
1 (16.7)
4 (66.7)
6 (25)
0.235
0
4 (80)
4 (40)
0 (0)
8 (33.3)
1
1 (20)
5 (50)
6 (66.7)
2
0 (0)
1 (10)
3 (33.3)
Panhypopituitarism
0 (0)
2 (20)
6 (66.7)
8 (33.3)
0.02
Corticotroph insufficiency
0 (0)
7 (10)
6 (66.7)
13 (54.2)
0.03
Diabetes insipidus
2 (40)
6 (60)
7 (100)
15 (62.5)
0.006
1 (20)
2 (20)
8 (88.9)
11 (45.8)
0.003
Postoperative MRI grade, n (%)
0.026
12 (50) 4 (16.7)
New endocrine deficits, n (%)
Endocrine replacement, n (%) Functional outcome, n (%)
0.004
Good
3 (60)
6 (60)
0 (0)
Moderate
2 (40)
4 (40)
4 (44.4)
9 (37.5)
Fair
0 (0)
0 (0)
3 (33.3)
0 (0)
Poor
0 (0)
0 (0)
2 (22.2)
2 (8.4)
Mortalities
0 (0)
0 (0)
0 (0)
0 (0)
10 (41.7)
MRI, magnetic resonance imaging.
of surgical video record. The intraoperatively observed attachment grade was found to correlate with the
postoperative radiologic outcome, the endocrine function, and the necessity of hormone-replacement therapy. As the
Figure 2. Endocrine functional deficits in patients who underwent gross total removal (GTR) and subtotal removal (STR). (A) Pre- and postoperative rates of endocrine deficits in patients who underwent GTR and STR. (B) Incidence of new endocrine deficiencies after GTR and STR. A significant difference between GTR and STR was observed in new DI. DI, diabetes insipidus; COR, cortisol; TSH, thyroid-stimulating hormone; GON, gonadotropin; PRL, prolactin; PAN, panhypopituitarism.
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main available preoperative examination, preoperative MRI was evaluated based on the extent of third ventricular deformation instead of visibility of tumor border. The underlying consideration for this criterion is the potential stretch lesion caused by prominently bulging of third VF might affect the hypothalamic function and postoperative outcome (15). On the basis of our results, hypothalamic deformation in MRI findings is a strong indicator for prognosis. The grade 2 group was found to have significantly worse outcomes compared with grade 1 and 0 groups (P ¼ 0.001, P ¼ 0.03). Surgical Approaches of IVC Because of its special location, IVC requires different approaches in addition to those already established for CP in other locations. Transsphenoidal surgery or extended transsphenoidal surgery has been successful in the removal of CPs located either totally or partially within an enlarged sella or subdiaphragmaticly (13); however, IVCs could not be accessed through these 2 approaches because of the presence of an intact pituitary stalk and third VF. In addition, transsphenoidal approaches are not able to offer an overview of retrochiasmatic region, which makes it difficult to remove the tumor part adherent posteriorly to the optic nerve. Recently, endoscopic endonasal surgery and an expanded endonasal approach have been introduced for CP with suprasellar location (6, 11). Nevertheless, the authors pointed out that although the retroinfundibular area could be reached with an endoscope, the intact pituitary stalk blocks the access towards third ventricle pure IVC (7). Previously, the transcallosal approach, transcortical-transventricular approach, pterional approach with incision of the lamina terminalis, and any combination of these approaches has proved to be effective in the removal of IVC (2, 4, 5, 8-10, 12). Therefore, we adopted a transcallosal or transventricular approach for tumors extending to the upper and posterior third ventricle and a translamina terminalis approach for tumors located in the lower half of third ventricle in our series. The observed low mortality and morbidity rates and good outcome verified the importance of transcranial approaches in IVC removal. In line with our study, Fahlbusch et al. (5)
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tumors with high attachment grade or/and high preoperative MRI grade. Therefore, we recommend that individual surgical strategies should be developed on the basis of surgical risk. For IVC with firm attachment and severe hypothalamus deformation, STR instead of GTR should be intended. ACKNOWLEDGMENTS The authors thank Dr. Zhenqiang Liu for valuable advice in proofreading. REFERENCES 1. Asari S, Sakurai M, Suzuki K, Hamasaki M, Sadamoto K: [Craniopharyngioma in the third ventricle (author’s transl; in Japanese)]. Neurol Med Chir (Tokyo) 20 (10):1039-1047, 1980. Figure 3. Comparison of the incidence of new endocrine deficits via different attachment grades. The rate (66.7%) of newly developed panhypopituitarism in grade 2 was significantly greater than attachment grade 0 (0%) (P value of 0.03). The rate of deterioration of attachment grade 2 (66.7%) and grade 1 (70%) was significantly greater than grade 0 (0/5) (grade 2 vs. 0, P ¼ 0.031; grade 1 vs. 0, P ¼ 0.026). Significantly more cases in grade 2 (100%) developed diabetes insipidus (DI) postoperatively than in grade 0 (20%), P ¼ 0.005, and grade 1 (59%), P ¼ 0.03. COR, cortisol; TSH, thyroid-stimulating hormone; GON, gonadotropin; PRL, prolactin; PAN, panhypopituitarism.
2. Behari S, Banerji D, Mishra A, Sharma S, Chhabra DK, Jain VK: Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature. Surg Neurol 60:245-252; discussion 252-243, 2003. 3. Cashion EL, Young JM: Intraventricular craniopharyngioma. Report of two cases. J Neurosurg 34:84-87, 1971. 4. Davies MJ, King TT, Metcalfe KA, Monson JP: Intraventricular craniopharyngioma: a long-term follow-up of six cases. Br J Neurosurg 11:533-541, 1997.
published recently a study of CP, in which a subfrontal interhemispheric midline approach was applied for tumors with third ventricle invasion, resulting in 85.3% GTR and good outcomes. Extent of Excision Traditionally, the CP has been radically removed to minimize the risk of recurrence. Nevertheless, devastating complications and significantly worsened functional deficits have been increasingly observed after GTR (19, 20). In tumors that attach to neurovascular structures, severe damage to the pituitary stalk and hypothalamus has been noted by several authors (22, 23). More and more surgeons have stressed the importance of hypothalamic structure protection (25). In addition, IVC is found more often in elderly patients, whereas CP found in other locations is the greatest in younger patients (15). Surgery-associated complication rates and mortality in IVC are 3 times greater compared with endosellar and suprasellar CP (16). These results do not support the use of GTR in IVC because elderly patients are less likely to experience recurrence if
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the postoperative outcome is poor. Even in pediatric CPs, evidence of a comparable tumor control and better quality of life after STR is increasing (17, 18). In our study, the recurrence rate (21.1%, 4/19) in the GTR group was not significantly different with that in the STR group (40%, 2/5). In the 9 cases with wide and close attachment, patients with GTR had a significantly worse endocrine outcome with a greater frequency of developing new panhypopituitarism and corticotroph deficiency compared with patients with STR. Furthermore, high morbidity and mortality rates (44.4%, 4 of 9 cases) were found in the GTR group.
5. Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M: Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90:237-250, 1999.
CONCLUSIONS
9. Klein HJ, Rath SA: Removal of tumors in the III ventricle using the lamina terminalis approach. Three cases of isolated growth of craniopharyngiomas in the III ventricle. Childs Nerv Syst 5:144-147, 1989.
Purely IVCs often present a different extent of attachment to the infundibulum and hypothalamus. Firm attachment and severe hypothalamus deformation on MRI findings are associated significantly with endocrine, functional, and postoperative radiologic outcome. Furthermore, GTR also was observed to correlate with worse postoperative outcome, especially in
6. Kassam A, Snyderman CH, Mintz A, Gardner P, Carrau RL: Expanded endonasal approach: the rostrocaudal axis. Part II. Posterior clinoids to the foramen magnum. Neurosurg Focus 19:E4, 2005. 7. Kassam AB, Gardner PA, Snyderman CH, Carrau RL, Mintz AH, Prevedello DM: Expanded endonasal approach, a fully endoscopic transnasal approach for the resection of midline suprasellar craniopharyngiomas: a new classification based on the infundibulum. J Neurosurg 108:715-728, 2008. 8. King TT: Removal of intraventricular craniopharyngiomas through the lamina terminalis. Acta Neurochir (Wien) 45:277-286, 1979.
10. Konovalov AN, Vikhert TM, Korshunov AG, Gorelyshev SK: Evaluation of the radicalness of the removal of craniopharyngioma of the 3d ventricle in children and the possible sources of their continued growth and recurrence [in Russian]. Zh Vopr Neirokhir Im N N Burdenko 6: 7-12, 1988.
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11. Koutourousiou M, Gardner PA, FernandezMiranda JC, Paluzzi A, Wang EW, Snyderman CH: Endoscopic endonasal surgery for giant pituitary adenomas: advantages and limitations. J Neurosurg 118:621-631, 2013. 12. Long DM, Chou SN: Transcallosal removal of cranio-pharyngiomas within the third ventricle. J Neurosurg 39:563-567, 1973. 13. Maira G, Anile C, Colosimo C, Cabezas D: Craniopharyngiomas of the third ventricle: translamina terminalis approach. Neurosurgery 47: 857-863; discussion 863-855, 2000. 14. Pan J, Qi S, Lu Y, Fan J, Zhang X, Zhou J, Peng J: Intraventricular craniopharyngioma: morphological analysis and outcome evaluation of 17 cases. Acta Neurochir (Wien) 153:773-784, 2011. 15. Pascual JM, Prieto R, Carrasco R: Craniopharyngiomas involving the floor of the third ventricle. Acta Neurochir (Wien) 153 (12):2447-2450; author reply 2451-2442, 2011. 16. Pascual JM, Prieto R, Carrasco R: Infundibulotuberal or not strictly intraventricular craniopharyngioma: evidence for a major topographical category. Acta Neurochir (Wien) 153:2403-2425; discussion 2426, 2011. 17. Puget S, Garnett M, Wray A, Grill J, Habrand JL, Bodaert N, Zerah M, Bezerra M, Renier D, PierreKahn A, Sainte-Rose C: Pediatric
craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg 106 (Suppl 1):3-12, 2007. 18. Scott RM, Hetelekidis S, Barnes PD, Goumnerova L, Tarbell NJ: Surgery, radiation, and combination therapy in the treatment of childhood craniopharyngioma—a 20-year experience. Pediatr Neurosurg 21 (Suppl 1):75-81, 1994. 19. Shirane R, Ching-Chan S, Kusaka Y, Jokura H, Yoshimoto T: Surgical outcomes in 31 patients with craniopharyngiomas extending outside the suprasellar cistern: an evaluation of the frontobasal interhemispheric approach. J Neurosurg 96: 704-712, 2002. 20. Steno J, Malacek M, Bizik I: Tumor-third ventricular relationships in supradiaphragmatic craniopharyngiomas: correlation of morphological, magnetic resonance imaging, and operative findings. Neurosurgery 54:1051-1058; discussion 10581060, 2004. 21. Tavangar SM, Larijani B, Mahta A, Hosseini SM, Mehrazine M, Bandarian F: Craniopharyngioma: a clinicopathological study of 141 cases. Endocr Pathol 15:339-344, 2004. 22. Van Effenterre R, Boch AL: Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg 97:3-11, 2002. 23. Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-
Maher T, Flamm ES, Epstein FJ, Miller DC: Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery 35:1001-1010; discussion 1010-1001, 1994. 24. Yasargil MG, Curcic M, Kis M, Siegenthaler G, Teddy PJ, Roth P: Total removal of craniopharyngiomas. Approaches and long-term results in 144 patients. J Neurosurg 73:3-11, 1990. 25. Zhang YQ, Ma ZY, Wu ZB, Luo SQ, Wang ZC: Radical resection of 202 pediatric craniopharyngiomas with special reference to the surgical approaches and hypothalamic protection. Pediatr Neurosurg 44 (6):435-443, 2008.
Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Received 13 December 2013; accepted 10 June 2014; published online 14 June 2014 Citation: World Neurosurg. (2014) 82, 6:1209-1215. http://dx.doi.org/10.1016/j.wneu.2014.06.015 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2014 Elsevier Inc. All rights reserved.
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Intraventricular Craniopharyngiomas: Surgical Management and Outcome Analyses in 24 Cases
Key words Attachment grade - Extent of resection - Intraventricular craniopharyngiomas -
Abbreviations and Acronyms BMI: Body mass index CP: Craniopharyngioma GTR: Gross total removal IVC: Intraventricular craniopharyngioma MRI: Magnetic resonance imaging STR: Subtotal removal VF: Ventricular floor From the 1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; 2 Max-Planck-Institute of Experimental Medicine, Göttingen, Germany; and 3Department of Neuropathology, Beijing Neurosurgical Institute, Beijing, China To whom correspondence should be addressed: Song Lin, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2014) 82, 6:1209-1215. http://dx.doi.org/10.1016/j.wneu.2014.06.015 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2014 Elsevier Inc. All rights reserved.
- OBJECTIVE:
Pure intraventricular craniopharyngioma is a rare subtype of craniopharyngioma that attaches frequently to the hypothalamus. The main challenge in tumor removal is protection of hypothalamic structure. The aim of our study was to set up a feasible risk-evaluation approach to help the surgeons make an individual treatment plan.
- METHODS:
We reviewed retrospectively 24 patients with pure intraventricular craniopharyngioma who underwent surgical therapy. Third ventricular deformation and thalamic attachment of the tumor were assessed by preoperative magnetic resonance imaging and intraoperative inspection. Correlations between the outcome, extent of removal, and different attachment grades were analyzed.
- RESULTS:
Preoperative magnetic resonance imaging grade had a significant correlation with functional outcome. Attachment grade was significantly correlated with endocrine, functional, and radiologic outcome. In the gross total removal (GTR) group, significantly more newly developed endocrinologic deficits were observed compared with patients who underwent subtotal removal (STR). All 6 GTR cases with the greatest attachment grade (grade 2) acquired newly developed endocrine insufficiency.
- CONCLUSIONS:
Attachment and deformation grade evaluated based on our criteria are significantly correlated with postoperative outcome. GTR of tightly attached tumor is associated with worse endocrinologic, functional, and radiologic outcomes compared with STR. Hence, we suggest that individual surgical plans should be made according to the grade of tumor attachment and hypothalamic deformation. STR should used in tumors with a high grade to achieve a good long-term outcome and avoid severe postoperative sequelae.
INTRODUCTION Craniopharyngioma (CP) accounts for 2%e 4% of the intracranial neoplasms. Yasargil et al. (24) devided CPs into 6 subtypes: 1) purely intrasellar-infradiaphragmatic; 2) intra- and suprasellar, infra- and supradiaphragmatic; 3) supradiaphragmatic parachiasmatic, extraventricular; 4) intra- and extraventricular; 5) paraventricular; and 6) pure intraventricular craiopharyngioma (IVC). Pure IVC is strictly defined as a CP exclusively located in the third ventricle that does not penetrate the third ventricular floor (VF). The presence of an intact pituitary stalk and third VF differentiate pure IVC distinctly from CP in other locations. However, only 0.5%e11% of CPs are pure IVC. Because of its rarity, a systemic approach to this tumor is still needed (1-3).
METHODS Patient Population Between January 2007 and April 2012, 830 patients with CP underwent surgery in the neurosurgery department of Beijing Tiantan Hospital; among these, 24 patients presented with pure IVC. The postoperative follow-up was conducted in the first month, every 3 months during the first year, and annually thereafter. The mean follow-up period was 42 months (range, 11e58 months). Radiologic Assessment Exclusive intraventricular location was assured by the presence of a patent
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suprasellar cistern, a normal pituitary stalk, and the absence of sellar abnormality in magnetic resonance imaging (MRI) findings as described previously (2). Maximal diameters in the vertical, horizontal, and sagittal axis were determined as Dx, Dy, and Dz. Tumor volume was estimated approximately by the formula (Dx Dy Dz)/2. Gross total removal (GTR) was defined when the residual tumor was not visible on postoperative MRI scans or its volume was smaller than 5% of the preoperative volume. Postoperative radiologic examinations were performed within 1 week. Degree of Hypothalamus Deformation. Preoperative MRI was graded as follows: grade
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0, completely identifiable third ventricular walls without bulging of lamina terminalis; grade 1, (partially) identifiable third walls with bulging of lamina terminalis; and grade 2, severe deformation, or third ventricle and lamina terminalis were not identifiable (Figure 1). Degree of Postoperative Hypothalamic Damage. The degree of postoperative hypothalamic damage was defined with the help of a neuroradiologist, who graded the damage according to postoperative MRI
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findings as follows (Figure 1): grade 0, no hypothalamic damage (intact third VF); grade 1, slight hypothalamic damage (identifiable but not intact third VF); and grade 2, severe hypothalamic damage (third VF not identifiable). Attachment Grading Intraoperatively, the extent of tumor attachment to hypothalamus was graded based on surgeons’ inspection and confirmed by review of surgical video record after the surgery: grade 0, no visible
attachment to third ventricular wall or floor, and the tumor border was well defined; grade 1, tumor presented a pedicle or mild attachment to the anterior third VF and presented an otherwise well-dissectible boundary; and grade 2, tumor presented widely and with close attachment to third VF or/and wall. Clinical Evaluation Presenting symptoms, psychological, ophthalmologic, and endocrinologic status of the patients were analyzed preoperatively
Figure 1. Pre- and postoperative magnetic resonance imaging (MRI) grading: Left Preoperative MRI: Assessment of hypothalamic deformation. grade 0, third ventricular structures clear identifiable, without the lamina terminalis bulging; grade 1, Lamina terminalis bulging, identifiable third ventricular structure; Grade 2, severe deformation of third ventricle lamina terminalis not identifiable. Right Postoperative MRI: Grade 0, no hypothalamic damage (intact third ventricular floor); grade 1 slight hypothalamic damage (identifiable but not intact third ventricular floor); grade 2 severe hypothalamic damage (third ventricular floor not identifiable).
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and by each follow-up. Functional outcome was evaluated according to Fahlbusch et al. (5) into 5 categories: good (independent and without impairment); moderate (independent and with a certain degree of impairment); fair (partially dependent with functional disability); poor (totally dependency); or death at the time point of follow-up. For each patient, body mass index (BMI) was calculated and recorded. For adults, patients with a BMI between 25 and 29.9 were considered as overweight and patients with BMI >30 as obese. Endocrinologic Evaluation For evaluation of anterior pituitary function, basal plasma levels of cortisol, growth hormone, thyroid-stimulating hormone, triiodothyroxine (T3), thyroxine (T4), luteinizing hormone, follicle-stimulating hormone, prolactin, testosterone, estradiol, and progesterone were measured. Panhypopituitarism was defined when the secretion of all anterior pituitary hormones (except for prolactin) was deficient. Posterior pituitary function was assessed by monitoring fluid intake and urine output. Serum electrolyte balance was monitored postoperatively until it had reached the normal range. Treatment Strategy No patient received radiotherapy before the initial surgery. The operations were performed by senior surgeons in our institute. Four different transcranial approaches were applied according to the location of lesions in the third ventricle. The frontal transcallosal approach was adopted in 15 cases in which the lesions extended to the upper half of the third ventricle. After splitting the corpus callosum from the midline, we incised the septum pellucidum and the fornix to approach the tumors in 11 cases. In the other 4 cases, one of the lateral ventricles expended medially over the midline, so we entered the lateral ventricle and exposed the tumors through dilated foramen of Monro. The frontal transcortical-transventricular approach was applied in 2 cases in which one frontal horn of the lateral ventricle was significantly enlarged. The tumors were resected through the dilated foramen of Monro. In the other 7 cases, tumors were situated in anterior and lower half of the third ventricle and created a bulge the
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lamina terminalis. With the pterional approach or subfrontal approach, we incised the lamina terminalis to access the third ventricle. Three of these 7 tumors were solid, 2 were purely cystic, and the other 2 had both solid and cystic components. After evacuation of the cyst contents, cyst wall was separated from the third ventricle in the cystic tumors. External drainage was placed in the third ventricle postoperatively for 24e48 hours to prevent potential hydrocephalus, intraventricular hemorrhage, and intracranial hypertension. Statistics Statistical analyses were performed with the Statistical Package for Social Sciences (version 17.0, SPSS, Chicago, Illinois, USA). The Exact Pearson c2 test, Pearson correlation analysis, or 2-tailed Fisher exact test was applied to analyze the data. Differences were considered statistically significant at the P < 0.05 level.
Table 1. Characteristics of Patients Characteristic
No. (%)
Number of cases
24
Age, years Median
40.2
Range
15e61
M/F ratio
10.5:1
Presenting complaints on admission Headache
16 (66.7)
Ophthalmologic deficits
10 (41.7)
Sexual dysfunction or amenorrhoea 8 (33.3) Mental disturbances or drowsiness
8 (33.3)
Diabetes insipidus
5 (20.8)
Intracranial hypertension
3 (12.5)
Preoperative MRI grade Grade 0
2 (8.3)
Grade 1
12 (50)
Grade 2
10 (41.7)
Intraoperative attachment grade
RESULTS Clinical Features Clinical features of the patients are summarized in Table 1.
Grade 0
5 (20.8)
Grade 1
10 (41.7)
Grade 2
9 (37.5)
F, female; M, male; MRI, magnetic resonance imaging.
Radiologic Findings The diameter of the tumors ranged from 3.0 to 4.0 cm (mean, 3.64 cm). Rounded or elliptic pure solid tumor was found in 20 cases; 2 purely cystic and 2 basal solid/ upper cystic tumors were found. The solid tumors and the wall of cystic tumors demonstrated contrast enhancement in gadolinium-diethylene triamine pentaacetic acid MRI. Lateral ventricle dilation presented in 12 cases (50.0%). Total calcification was found in 11 cases (45.8%), 7 (29.2%) in the tumor mass, and 4 (16.7%) on the capsule. Tumor volume in this cohort ranged from 9 cm3 to 44.1 cm3 (mean, 19.7 cm3). Preoperative MRI Grading In 2 (8.3%) cases, no bulging of the lamina terminalis was observed (grade 0); in 12 (59%) cases, grade 1 was noted on preoperative MRI. In the other 10 (41.6%) cases, the third ventricle was deformed, and third ventricular border was not identifiable (grade 2).
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Degree of Attachment On the basis of the criteria described previously, 5 cases (20.8%) were evaluated as attachment grade 0, 10 cases (41.7%) as attachment grade 1, and 9 (37.5%) cases as attachment grade 2.
Histopathologic Findings All tumor specimens were reviewed by 2 neuropathologists from the pathology department. According to previous studies, the papillary CP was located predominantly intraventricularly whereas the majority of adamantinomatous type more frequently had a suprasellar or parasellar location (21). In children, most craniopharyngimoas were the adamantinomatous type, whereas in adults, both types were observed with nearly equal frequency (23). In our series, 41.7% papillary type and 58.3% adamantinomotous type were found in the 24 cases. The histopathologic pattern of the
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tumor did not show significant association with attachment grade.
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Surgical Outcomes Total removal was the intended for all the cases; however, only GTR was achieved in 19 (79.2%) of 24 cases. Subtotal removal (STR) was achieved in 5 cases (20.8%) because of a wide and close attachment to the hypothalamus (3/5), inadequate view of the tumor (1/5), and not dissectible fine capsule remnant (1/5). Mortality and Morbidity Mortality. No patient died within the first 3 months after surgery. At the date of last follow-up, 5 patients died. One patient died of severe endocrine dysregulation at 6 months. The other 4 patients experienced recurrence and died after conservative therapy or radiotherapy at 44 months, 12 months, 12 months, and 43 months, respectively. Morbidity. Two (8.3%) patients presented with complications in the first month after surgery (the early postoperative period). One patient developed mental disturbances and memory dysfunction. A seizure occurred in another patient and was controlled effectively afterward. Third ventricular drainage was performed in 15 cases. Hydrocephalus was completely resolved in all cases, and extubation was performed in 24e48 hours. Recurrence At the time point of last follow-up, 4 cases from the GTR group (4/19) and 2 from the STR (2/5) group experienced recurrence. The recurrence rates of GTR group and STR group did not differ significantly. Postoperative MRI Grading Patients with widely dispersed and strongly attached tumors (attachment grade 2) had significantly more severe damage than patients with tumors of attachment grade 0 and attachment grade 1 (P ¼ 0.026) (Table 1). Clinical Outcome Visual ability was improved in 7 cases, remained unchanged in 3 cases, and no deterioration was observed within 1 month after the surgery. Mean weight at the first follow-up was 24.2 kg (3.1 kg). Obesity and overweight were observed in 8.3% (2/
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Treatment of Residual and Recurrent Tumor In the 6 cases with recurrence, one patient underwent a second operation with good functional outcome. One patient with cystic tumor recurrence received stereotactic interstitial irradiation. One patient received radiosurgery, which failed to reduce the size of the tumor. The other 3 patients received conservative therapy because of severe hypothalamic dysregulation and poor health conditions. At last follow-up, the latter 4 patients died.
24) and in 54.2% (13/24) of the patients, respectively. Two patients experienced emaciation. Newly developed psychological disturbances were documented in 3 patients. Three months after surgery, the functional outcome of patients was evaluated and served as follow-up baseline. Both preoperative MRI grade and attachment grade were correlated significantly with functional outcome (Tables 2 and 3).
Endocrinologic Outcome Considering all endocrine functions, significant deterioration was observed after surgery (P < 0.001, McNemar test; Figure 2). The rate of patients who underwent GTR and developed diabetes insipidus was significantly greater than that of the STR group (P value of 0.047). Comparing the different attachment grades, we found that the rate of newly developed panhypopituitarism in the grade 2 group was significantly greater compared with the attachment grade 0. The deterioration rate of corticotroph function in the attachment grade 2 and grade 1 groups were significantly greater compared with the grade 0 group. Significantly more cases in grade 2 group developed diabetes insipidus and needed endocrine-replacement medication than in grade 0 and grade 1 groups (Table 3 and Figure 3). Remarkably, among all the 9 cases with attachment grade 2 tumors, only the 6 cases with GTR developed panhypopituitarism and corticotroph insufficiency.
DISCUSSION Preoperative and Intraoperative Evaluation The association of prognosis when tumor is attached to the third ventricular wall has been reported previously (14); however, because of the obscuring effect of the border between third VF and tumor capsule, the attachment of the tumor to the hypothalamus could not be evaluated preoperatively (16). Controversy still exists regarding the correlation between hypothalamic involvement visible in MRI findings and outcome (14). IVCs that seemed to be closely attached to hypothalamic walls on preoperative MRI often were found to have an intact capsule separating the tumor mass from third ventricular wall (15). In our study, the extent of tumor attachment was graded based on surgeons’ inspection and confirmed by review
Table 2. Association Preoperative MRI Grades with Functional Outcome Preoperative MRI Grade Characteristics
0
1
2
Total
Total
2
12
10
24
Good
2 (100)
8 (66.7)
0 (0)
10 (41.6)
Moderate
0 (0)
4 (33.3)
5 (50)
9 (37.5)
Fair
0 (0)
0 (0)
3 (30)
3 (1.3)
Poor
0 (0)
0 (0)
2 (20)
2 (8.3)
Mortatlities
0 (0)
0 (0)
0 (0)
0 (0)
Functional outcome, n (%)
P Value
0.014
MRI, magnetic resonance imaging.
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Table 3. Outcomes Associated with Attachment Grades Intraoperative Attachment Grade Characteristics Total Tumor volume, mean SD, cm
3
0
1
2
Total
P Value
5
10
9
24
20.6 10
15 5.3
24.3 10.6
19.7 9.3
0.053
Gross total removal, n (%)
5 (100)
8 (80)
6 (66.7)
19 (79.2)
0.386
Recurrence, n (%)
1 (16.7)
1 (16.7)
4 (66.7)
6 (25)
0.235
0
4 (80)
4 (40)
0 (0)
8 (33.3)
1
1 (20)
5 (50)
6 (66.7)
2
0 (0)
1 (10)
3 (33.3)
Panhypopituitarism
0 (0)
2 (20)
6 (66.7)
8 (33.3)
0.02
Corticotroph insufficiency
0 (0)
7 (10)
6 (66.7)
13 (54.2)
0.03
Diabetes insipidus
2 (40)
6 (60)
7 (100)
15 (62.5)
0.006
1 (20)
2 (20)
8 (88.9)
11 (45.8)
0.003
Postoperative MRI grade, n (%)
0.026
12 (50) 4 (16.7)
New endocrine deficits, n (%)
Endocrine replacement, n (%) Functional outcome, n (%)
0.004
Good
3 (60)
6 (60)
0 (0)
Moderate
2 (40)
4 (40)
4 (44.4)
9 (37.5)
Fair
0 (0)
0 (0)
3 (33.3)
0 (0)
Poor
0 (0)
0 (0)
2 (22.2)
2 (8.4)
Mortalities
0 (0)
0 (0)
0 (0)
0 (0)
10 (41.7)
MRI, magnetic resonance imaging.
of surgical video record. The intraoperatively observed attachment grade was found to correlate with the
postoperative radiologic outcome, the endocrine function, and the necessity of hormone-replacement therapy. As the
Figure 2. Endocrine functional deficits in patients who underwent gross total removal (GTR) and subtotal removal (STR). (A) Pre- and postoperative rates of endocrine deficits in patients who underwent GTR and STR. (B) Incidence of new endocrine deficiencies after GTR and STR. A significant difference between GTR and STR was observed in new DI. DI, diabetes insipidus; COR, cortisol; TSH, thyroid-stimulating hormone; GON, gonadotropin; PRL, prolactin; PAN, panhypopituitarism.
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main available preoperative examination, preoperative MRI was evaluated based on the extent of third ventricular deformation instead of visibility of tumor border. The underlying consideration for this criterion is the potential stretch lesion caused by prominently bulging of third VF might affect the hypothalamic function and postoperative outcome (15). On the basis of our results, hypothalamic deformation in MRI findings is a strong indicator for prognosis. The grade 2 group was found to have significantly worse outcomes compared with grade 1 and 0 groups (P ¼ 0.001, P ¼ 0.03). Surgical Approaches of IVC Because of its special location, IVC requires different approaches in addition to those already established for CP in other locations. Transsphenoidal surgery or extended transsphenoidal surgery has been successful in the removal of CPs located either totally or partially within an enlarged sella or subdiaphragmaticly (13); however, IVCs could not be accessed through these 2 approaches because of the presence of an intact pituitary stalk and third VF. In addition, transsphenoidal approaches are not able to offer an overview of retrochiasmatic region, which makes it difficult to remove the tumor part adherent posteriorly to the optic nerve. Recently, endoscopic endonasal surgery and an expanded endonasal approach have been introduced for CP with suprasellar location (6, 11). Nevertheless, the authors pointed out that although the retroinfundibular area could be reached with an endoscope, the intact pituitary stalk blocks the access towards third ventricle pure IVC (7). Previously, the transcallosal approach, transcortical-transventricular approach, pterional approach with incision of the lamina terminalis, and any combination of these approaches has proved to be effective in the removal of IVC (2, 4, 5, 8-10, 12). Therefore, we adopted a transcallosal or transventricular approach for tumors extending to the upper and posterior third ventricle and a translamina terminalis approach for tumors located in the lower half of third ventricle in our series. The observed low mortality and morbidity rates and good outcome verified the importance of transcranial approaches in IVC removal. In line with our study, Fahlbusch et al. (5)
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tumors with high attachment grade or/and high preoperative MRI grade. Therefore, we recommend that individual surgical strategies should be developed on the basis of surgical risk. For IVC with firm attachment and severe hypothalamus deformation, STR instead of GTR should be intended. ACKNOWLEDGMENTS The authors thank Dr. Zhenqiang Liu for valuable advice in proofreading. REFERENCES 1. Asari S, Sakurai M, Suzuki K, Hamasaki M, Sadamoto K: [Craniopharyngioma in the third ventricle (author’s transl; in Japanese)]. Neurol Med Chir (Tokyo) 20 (10):1039-1047, 1980. Figure 3. Comparison of the incidence of new endocrine deficits via different attachment grades. The rate (66.7%) of newly developed panhypopituitarism in grade 2 was significantly greater than attachment grade 0 (0%) (P value of 0.03). The rate of deterioration of attachment grade 2 (66.7%) and grade 1 (70%) was significantly greater than grade 0 (0/5) (grade 2 vs. 0, P ¼ 0.031; grade 1 vs. 0, P ¼ 0.026). Significantly more cases in grade 2 (100%) developed diabetes insipidus (DI) postoperatively than in grade 0 (20%), P ¼ 0.005, and grade 1 (59%), P ¼ 0.03. COR, cortisol; TSH, thyroid-stimulating hormone; GON, gonadotropin; PRL, prolactin; PAN, panhypopituitarism.
2. Behari S, Banerji D, Mishra A, Sharma S, Chhabra DK, Jain VK: Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature. Surg Neurol 60:245-252; discussion 252-243, 2003. 3. Cashion EL, Young JM: Intraventricular craniopharyngioma. Report of two cases. J Neurosurg 34:84-87, 1971. 4. Davies MJ, King TT, Metcalfe KA, Monson JP: Intraventricular craniopharyngioma: a long-term follow-up of six cases. Br J Neurosurg 11:533-541, 1997.
published recently a study of CP, in which a subfrontal interhemispheric midline approach was applied for tumors with third ventricle invasion, resulting in 85.3% GTR and good outcomes. Extent of Excision Traditionally, the CP has been radically removed to minimize the risk of recurrence. Nevertheless, devastating complications and significantly worsened functional deficits have been increasingly observed after GTR (19, 20). In tumors that attach to neurovascular structures, severe damage to the pituitary stalk and hypothalamus has been noted by several authors (22, 23). More and more surgeons have stressed the importance of hypothalamic structure protection (25). In addition, IVC is found more often in elderly patients, whereas CP found in other locations is the greatest in younger patients (15). Surgery-associated complication rates and mortality in IVC are 3 times greater compared with endosellar and suprasellar CP (16). These results do not support the use of GTR in IVC because elderly patients are less likely to experience recurrence if
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the postoperative outcome is poor. Even in pediatric CPs, evidence of a comparable tumor control and better quality of life after STR is increasing (17, 18). In our study, the recurrence rate (21.1%, 4/19) in the GTR group was not significantly different with that in the STR group (40%, 2/5). In the 9 cases with wide and close attachment, patients with GTR had a significantly worse endocrine outcome with a greater frequency of developing new panhypopituitarism and corticotroph deficiency compared with patients with STR. Furthermore, high morbidity and mortality rates (44.4%, 4 of 9 cases) were found in the GTR group.
5. Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M: Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg 90:237-250, 1999.
CONCLUSIONS
9. Klein HJ, Rath SA: Removal of tumors in the III ventricle using the lamina terminalis approach. Three cases of isolated growth of craniopharyngiomas in the III ventricle. Childs Nerv Syst 5:144-147, 1989.
Purely IVCs often present a different extent of attachment to the infundibulum and hypothalamus. Firm attachment and severe hypothalamus deformation on MRI findings are associated significantly with endocrine, functional, and postoperative radiologic outcome. Furthermore, GTR also was observed to correlate with worse postoperative outcome, especially in
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10. Konovalov AN, Vikhert TM, Korshunov AG, Gorelyshev SK: Evaluation of the radicalness of the removal of craniopharyngioma of the 3d ventricle in children and the possible sources of their continued growth and recurrence [in Russian]. Zh Vopr Neirokhir Im N N Burdenko 6: 7-12, 1988.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Received 13 December 2013; accepted 10 June 2014; published online 14 June 2014 Citation: World Neurosurg. (2014) 82, 6:1209-1215. http://dx.doi.org/10.1016/j.wneu.2014.06.015 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2014 Elsevier Inc. All rights reserved.
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