Repeated neuroendoscopic palliative surgery in elderly patients with predominantly cystic craniopharyngioma in the third ventricle: three case reports

Transcription

1 DOI /ins Innovative Neurosurgery 2013; 1(1): Case Report Kimitoshi Sato*, Hidehiro Oka, Satoshi Utsuki and Kiyotaka Fujii Repeated neuroendoscopic palliative surgery in elderly patients with predominantly cystic craniopharyngioma in the third ventricle: three case reports Abstract Objective: The management of craniopharyngioma in the elderly remains controversial. We sought to better characterize the outcomes of elderly patients treated with neuroendoscopic palliative surgery (NPS). Methods: We report the cases of three patients older than 80 years, whose cystic craniopharyngioma was treated by repeated NPS. Results: One male and two female patients, ranging in age from 80 to 83 years (mean = 81.3 years) had predominantly cystic lesions in the third ventricle and a suprasellar enhanced mass. Two patients underwent two NPS procedures and the other received three. The operating time ranged from 40 to 140 min (mean = 93.4 min), and the interval between operations ranged from 2 to 24 months (mean = 11.3 months). The length of postoperative hospitalization ranged from 4 to 28 days (mean = 11.1 days). There were no perioperative complications, and all patients experienced symptom improvement immediately after the operation. Conclusion: Repeated NPS can be an effective alternative treatment for cystic craniopharyngioma in the third ventricle, especially in elderly patients. Keywords: Craniopharyngioma; cyst; elderly; endoscopy; palliation. * Corresponding author: Kimitoshi Sato, MD, Department of Neurosurgery, Kitasato University School of Medicine, Kitasato, Minami-ku, Sagamihara, Kanagawa , Japan, Tel.: , Fax: , kimitoshi@k8.dion.ne.jp Hidehiro Oka, Satoshi Utsuki and Kiyotaka Fujii: Department of Neurosurgery, Kitasato University School of Medicine, Sagamihara, Japan Introduction Craniopharyngiomas are slow-growing benign tumors, which are thought to be derived from a residual cell nest of the stomodeum. Such tumors are rare in elderly patients. Out of all cases of craniopharyngioma, 5.8% occur in patients over the age of 70 years, and only 0.6% are found in patients over the age 80 years [ 29 ]. Radical surgery for craniopharyngioma carries high morbidity and mortality rates. The risk for visual, endocrinological, and neuropsychological sequelae is high in patients undergoing removal of this tumor [5, 9, 12, 31, 34, 35, 37, 41 ]. The existing scientific literature contains only seven documented cases of craniopharyngioma in patients older than 70 years, and two of these patients died after surgery [10, 17, 30, 32, 33, 43 ]. Given this lack of data, there is no consensus on the management of craniopharyngioma in the elderly, particularly because their risk for perioperative complications is higher than in younger patients, and their expected lifespan is shorter. Neuroendoscopic surgery has been used to address cystic craniopharyngiomas [1, 10, 13, 16, 20 23, 28 ]. However, this treatment is not radical and the tumors tend to recur [ 9, 20 ]. In patients with cystic craniopharyngioma who underwent neuroendoscopic fenestration followed by gamma knife surgery (GKS), the recurrence rate was as high as 53.8% at a mean follow-up time of 32.1 months, and 30% of the patients developed diabetes insipidus [ 24 ]. Despite these shortcomings of neuroendoscopic palliative surgery (NPS) treatment, this treatment option has the benefits of being less invasive and requiring less recovery time than other interventions, making it an attractive treatment option in the elderly. Here, we report three cases of patients with cystic craniopharyngioma in the third ventricle who were older than 80 years. We treated them by repeated NPS, to obtain immediate symptom improvement and to shorten their hospitalization without perioperative complications.

2 68 Sato et al., Neuroendoscopic palliative surgery in patients with craniopharyngioma Table 1 Summary of three patients treated with seven neuroendoscopic palliative surgeries. Case number Age (years)/ sex Symptom Hydrocephalus Time to reop (months) Operation time (min) Days to discharge 1 83/Male D, GD D D, GD /Female VI 79 7 D, GD /Female D, GD D, GD D = Dementia, GD = Gait disturbance, reop=reoperation, VI = Visual impairment. Material and methods In elderly patients with cystic craniopharyngioma, we applied the same treatment strategy (neuroendoscopic partial removal without radiation) even if the tumor was recurrent. Between May 2007 and May 2011, three elderly patients (1 male and 2 females) underwent NPS at Kitasato University Hospital. The patients ranged in age from 80 to 83 years (mean = 81.3 years). Two patients underwent two rounds of NPS procedures each, and the other was treated with NPS three times. Of the seven operations, five were performed to address instances of dementia and gait disturbance, one was used to treat an instance of dementia, and the last was performed to treat visual impairment. The tumors presented as cystic lesions in the third ventricle and a suprasellar enhanced mass. Additional patient data are presented in Table 1. Six months later, he again presented with dementia. The MRI showed a huge cystic mass in the third ventricle and a solid suprasellar mass ( Figure 3 ). At the second NPS, we inserted an Ommaya catheter in the third ventricle. The operation time was 75 min. His symptoms again improved immediately, and there were no complications. He was discharged 1 week after the operation. Postoperative MRI showed a collapsed cyst ( Figure 4 ). Eleven months after the second NPS, the patient presented with dementia, gait disturbance, and incontinence. Computed tomography (CT) showed a huge cystic mass in the third ventricle ( Figure 5 ), the contents of which NPS procedure With the patient under general anesthesia, we first placed a peelaway sheath into the anterior horn of the lateral ventricle via a frontal burr hole and then inserted a steerable flexible fiberscope (diameter 2.7 mm; Olympus Optical Co., Tokyo, Japan). A cyst wall in the third ventricle was partially removed with forceps, and a specimen of the cyst wall was obtained. The cystic contents had the color and consistency of motor oil, reinforcing suspicions of craniopharyngioma. The cyst was rinsed out carefully. Case presentations Case 1 An 83-year-old man presented with dementia and gait disturbance. Magnetic resonance imaging (MRI) revealed hydrocephalus and a cystic suprasellar mass that extended into the third ventricle ( Figure 1 ). The operation time for the first NPS procedure was 105 min. His symptoms improved immediately. There were no complications, and he was discharged 1 week after the operation. Postoperative MRI showed a collapsed cyst and a decrease in the size of the lateral ventricle ( Figure 2 ). Figure 1 Preoperative MRI performed at the first operation showing a cystic craniopharyngioma and hydrocephalus.

3 Sato et al., Neuroendoscopic palliative surgery in patients with craniopharyngioma 69 Figure 4 Postoperative MRI showing the collapsed cyst. Figure 2 Postoperative MRI after cyst reduction by NPS showing the collapsed cyst and a decrease in the size of the lateral ventricle. could not be aspirated via the Ommaya catheter. At the third NPS, we again inserted an Ommaya catheter into the third ventricle. The operation time was 120 min. Again, his symptoms improved immediately, there were no complications, and he was discharged on the 4 th postoperative day. Postoperative CT showed a collapsed cyst and a decrease in the size of the lateral ventricle ( Figure 6 ). Twenty months after the third NPS, the patient began suffering from appetite loss. Imaging studies showed liver carcinoma and multiple bone metastases. MRI showed a Figure 3 Preoperative MRI prior to the second operation showing a cystic mass in the third ventricle. Figure 5 Preoperative CT image at the time of the third operation showing a cystic mass in the third ventricle and hydrocephalus.

4 70 Sato et al., Neuroendoscopic palliative surgery in patients with craniopharyngioma also hydrocephalus. The operation time of the first NPS was 140 min. Her symptoms improved immediately. There were no complications, and she was discharged 2 weeks after the operation. Postoperative CT showed a collapsed cyst and a decrease in the size of the lateral ventricle. Two years later, she again presented with dementia and gait disturbance. There was MRI evidence of cystic masses in the third ventricle and suprasella. The operation time for the second NPS was 40 min. Her symptoms improved immediately, there were no complications, and she was discharged 11 days after the operation. At 18-month follow-up, she was free of recurrence. Results Figure 6 Postoperative CT image after cyst reduction by NPS showing the collapsed cyst and a decrease in the size of the lateral ventricle. huge cystic mass in the third ventricle. His family refused additional treatments. Case 2 An 81-year-old woman presented with visual impairment. MRI revealed a cystic mass in the third ventricle and a solid suprasellar mass. The operation time for the first NPS was 79 min. Her symptoms improved, there were no complications, and she was discharged 1 week after the operation. Postoperative CT revealed a collapsed cyst. Two months later, she presented with dementia and gait disturbance. MRI revealed cystic masses in the third ventricle and suprasella. The operation time for the second NPS was 95 min. Her symptoms gradually improved, she experienced no complications, and she was discharged 4 weeks after the second operation. At her 3-year follow-up appointment, there was no evidence of recurrence. Case 3 An 80-year-old woman presented with dementia and gait disturbance. MRI revealed multiple cystic masses in the third ventricle and a solid suprasellar mass. There was We treated three elderly patients with predominantly cystic craniopharyngioma by repeated NPS ( Table 1 ). The operating time for the seven procedures ranged from 40 to 140 min (mean = 93.4 min). The interval between the last operation and recurrence varied from 2 to 24 months (mean = 11.3 months) and we encountered no perioperative complications. In all three patients, the preoperative symptoms and CT/MRI findings improved immediately after each operation. The postoperative hospital stay ranged from 4 to 28 days (mean = 11.1 days). Discussion Total tumor resection is the ideal primary treatment for patients with craniopharyngioma. However, even after macroscopic total resection, the recurrence rate is as high as 50% [ 26 ]. The recurrence rate is even higher after incomplete tumor removal [2, 9, 38, 41, 42 ]. The complete resection rate for tumors in the third ventricle is only 20.8% and if there is hydrocephalus, the extent of tumor resection is lower still [ 9 ]. The management of craniopharyngioma remains controversial, especially in elderly patients, because their risk for perioperative complications is higher than it is for younger patients and their expected lifespan is shorter. Signs and symptoms of cystic craniopharyngioma are usually elicited directly by the cyst and/or the associated hydrocephalus. Decompression of the cyst and management of hydrocephalus can alleviate symptoms and improve the patient s activities of daily living, even without tumor resection [ 9, 10 ]. However, the efficacy and safety of stereotactic cyst decompression remains problematic, because the procedure is blind and the cystic lesions may shift. NPS, by contrast, circumvents this problem because it is a visually guided procedure.

5 Sato et al., Neuroendoscopic palliative surgery in patients with craniopharyngioma 71 Refilling of the cyst after a few months has been reported, even in the presence of a permanent drainage catheter [ 9, 20 ]. The insertion of Ommaya catheters in patients with craniopharyngioma has been reported to be safe and effective [ 13 ]. We placed such catheters in four out of seven operations. However, unfortunately the aspiration of cystic fluid was not possible in any of the cases. Others have reported cases in which there was no recurrence of craniopharyngioma in the course of 3 and 4 years after neuroendoscopic cyst aspiration [ 10, 16 ]. The most significant factor associated with craniopharyngioma recurrence was the extent of surgical resection [ 41 ]. All three of our patients suffered recurrence; the interval between the last operation and recurrence varied from 2 to 24 months (mean = 11.3 months). Although the postoperative time to recurrence was different in our series, the duration of the recurrence-free interval increased with the number of preceding procedures. Locatelli et al. documented the efficacy of stents endoscopically implanted in the cystic cavity of craniopharyngiomas [ 18 ]. In Case 2, we performed only one fenestration with forceps at the first operation to avoid bleeding. In subsequent procedures, we opened multiple fenestrations on the cyst wall. The placement of more and larger fenestrations may delay their occlusion. A further accumulation of case data is needed, to identify factors associated with the interval between the surgical procedure and tumor recurrence, so that these recurrences can be minimized in the future. Postoperative conventional radiotherapy for residual craniopharyngioma has been reported to reduce the incidence of tumor recurrence [ 11, 25, 36, 40 ]. Rajan et al. reported that the recurrence rate was 15% in patients with craniopharyngioma who underwent external beam radiotherapy after incomplete tumor removal. The recurrence rate was 33 69% in patients whose treatment consisted of only surgery without total removal [ 27 ]. Compared with conventional radiotherapy, postoperative stereotactic radiosurgery (SRS) or stereotactic radiotherapy (SRT) for residual craniopharyngioma, increased the survival rate and elicited fewer complications related to visual, endocrinological, and neurophysiological functions [6 8, 15, 19, 39, 40 ]. By contrast, SRS does not effectively address the cystic components of brain tumors [ 14 ]. In patients with cystic craniopharyngioma who underwent neuroendoscopic fenestration before GKS, the recurrence rate was as high as 53.8% at a mean follow-up time of 32.1 months, and 30% developed diabetes insipidus [ 24 ]. Serious complications can arise from intraventricular neuroendoscopy (particularly with third ventriculostomy), including hemorrhage, infarction, subdural hematoma, neural injury, and infection [ 3, 4, 20 ]. However, we did not encounter any of these issues in our patients subjected to NPS. In patients receiving conventional radiotherapy before surgery or at tumor recurrence, the survival rate was no higher than that encountered in our patients [ 25, 36 ]. In patients manifesting with growth of the cystic component and solid mass at recurrence, SRS or SRT after NPS may need considering. We found that our treatment method resulted in immediate postoperative symptom improvement. It also allowed for shorter hospitalization and did not raise perioperative complications. These features suggest that repeated NPS is a viable treatment alternative for elderly patients with predominantly cystic craniopharyngioma in the third ventricle. Acknowledgments: This study was supported in part by a Kitasato University Research Grant for Young Researchers. Received October 22, Accepted November 9, Previously published online December 18, 2012 References [1] Barajas MA, Ramirez-Guzman G, Rodriguez-Vazquez C, Toledo-Buenrostro V, Velasquez-Santana H, del Robles RV, et al. Multimodal management of craniopharyngiomas: neuroendoscopy, microsurgery, and radiosurgery. J Neurosurg. 2002;97(5 Suppl): [2] Baskin DS, Wilson CB. Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg. 1986;65:22 7. [3] Bouras T, Sgouros S. Complication of endoscopic third ventriculostomy. A review. J Neurosurg. 2011;7: [4] Bouras T, Sgouros S. Complication of endoscopic third ventriculostomy: a systemic review. Acta Neurochir Suppl. 2012;113: [5] Brada M, Thomas DG. Craniopharyngioma revisited. Int J Radiat Oncol Biol Phys. 1993;27: [6] Chiou SM, Lunsford LD, Niranjan A, Kondziolka D, Flickinger JC. Stereotactic radiosurgery of residual or recurrent craniopharyngioma, after surgery, with or without radiation therapy. Neuro Oncol. 2001;3: [7] Chung WY, Pan HC, Shiau CY, Guo WY, Wang LW. Gamma knife radiosurgery for craniopharyngiomas. J Neurosurg. 2000; 93(3 Suppl): [8] Combs SE, Thilmann C, Huber PE, Hoess A, Debus J, Schulz- Ertner D. Achievement of long-term local control in patients with craniopharyngiomas using high precision stereotactic radiotherapy. Cancer. 2007;109: [9] Fahlbusch R, Honegger J, Paulus W, Huk W, Buchfelder M. Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg. 1999;90:

6 72 Sato et al., Neuroendoscopic palliative surgery in patients with craniopharyngioma [10] Fujimoto Y, Fujimoto Y, Kato A, Yoshimine T. Neuroendoscopic palliation for large cystic craniopharyngioma in an elderly patient. Br J Neurosurg. 2007;2: [11] Habrand JL, Ganry O, Couanet D, Rouxel V, Levy-Piedbois C, Pierre-Kahn A. The role of radiation therapy in the management of craniopharyngioma: a 2 year experience and review of the literature. Int J Radiat Oncol Biol Phys. 1999;44: [12] Honegger J, Buchfelder M, Fahlbusch R. Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg. 1999;90: [13] Joki T, Oi S, Babapour B, Kaito N, Ohashi K, Ebara M, et al. Neuroendoscopic placement of Ommaya reservoir into a cystic craniopharyngioma. Childs Nerv Syst. 2002;18: [14] Kim MS, Lee SI, Sim JH. Brain tumors with cysts treated with gamma knife radiosurgery: is microsurgery indicated? Stereotact Funct Neurosurg. 1999;72: [15] Kobayashi T, Kida Y, Mori Y, Hasegawa T. Long-term results of gamma knife surgery for the treatment of craniopharyngioma in 98 consecutive cases. J Neurosurg. 2005;103: [16] Kuramoto T, Uchikado H, Tajima Y, Tokutomi T, Shigemori M. Neuroendoscopic placement of the reservoir in an elderly patient with recurrent craniopharyngioma: case report. No Shinkei Geka. 2005;33: (in Japanese). [17] Lederman GS, Recht A, Loeffler JS, Dubuisson D, Kleefield J, Schnitt SJ. Craniopharyngioma in an elderly patient. Cancer. 1987;60: [18] Locatelli D, Levi D, Rampa F, Pezzotta S, Castelnuovo P. Endoscopic approach for the treatment of relapses in cystic craniopharyngiomas. Childs Nerv Syst. 2004;20: [19] Minniti G, Saran F, Traish D, Soomal R, Sardell S, Gonsalves A, et al. Fractionated stereotactic conformal radiotherapy following conservative surgery in the control of craniopharyngiomas. Radiother Oncol. 2007;82:90 5. [20] Nakahara Y, Koga H, Maeda K, Takagi M, Tabuchi K. Neuroendoscopic transventricular surgery for suprasellar cystic mass lesions such as cystic craniopharyngioma and Rathke cleft cyst. Neurol Med Chir (Tokyo). 2004;44: [21] Nakamizo A, Inamura T, Nishio S, Inoha S, Ishibashi H, Fukui M. Neuroendoscopic treatment of cystic craniopharyngioma in the third ventricle. Minim Invasive Neurosurg. 2001;44:85 7. [22] Nicolato A, Foroni R, Rosta L, Gerosa M, Bricolo A. Multimodality stereotactic approach to the treatment of cystic craniopharyngiomas. Minim Invasive Neurosurg. 2004;47: [23] Novak Z, Chrastina J, Feitova V, Lzicarova E, Riha I. Minimally invasive treatment of posterior fossa craniopharyngioma by means of navigated endoscopy. Minim Invasive Neurosurg. 2008;51: [24] Park YS, Chang JH, Park YG, Kim DS. Recurrence rates after neuroendoscopic fenestration and gamma knife surgery in comparison with subtotal resection and gamma knife surgery for the treatment of cystic craniopharyngiomas. J Neurosurg. 2011;114: [25] Pemberton LS, Dougal M, Magee B, Gattamaneni HR. Experience of external beam radiotherapy given adjuvantly or at relapse following surgery for craniopharyngioma. Radiother Oncol. 2005;77: [26] Puget S, Garnett M, Wray A, Grill J, Habrand JL, Bodaert N, et al. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg. 2007;106(1 Suppl):3 12. [27] Rajan B, Ashley S, Gorman C, Jose CC, Horwich A, Bloom HJ, et al. Craniopharyngioma: a long-term results following limited surgery and radiotherapy. Radiother Oncol. 1993;26:1 10. [28] Reda WA, Hay AA, Ganz JC. A planned combined stereotactic approach for cystic intracranial tumors. Report of two cases. J Neurosurg. 2002;97(5 Suppl): [29] Report of brain tumor registry of Japan ( ): Part I. General features of brain tumors. Neurol Med Chir (Tokyo). 2009;49(Suppl):S1 25. [30] Russell RWR, Pennybacker JB. Craniopharyngioma in the elderly. J Neurol Neurosurg Psychiat. 1961;24:1 13. [31] Sainte-Rose C, Puget S, Wray A, Zerah M, Grill J, Brauner R, et al. Craniopharyngioma: the pendulum of surgical management. Childs Nerv Syst. 2005;21: [32] Sato A, Sakurada K, Kokubo Y, Sato S, Kayama T. An elderly case of craniopharyngioma: treatment by limited surgery and radiation therapy. No Shinkei Geka. 2005;33: (in Japanese). [33] Sekiya T, Ito K, Akasaka K, Suzuki S. Two operated cases of craniopharyngioma in patients over 70 years old. Neurol Surg. 1995;23: (in Japanese). [34] Shi XE, Wu B, Fan T, Zhou ZQ, Zang YL. Craniopharyngioma: surgical experience of 309 cases in China. Clin Neurol Neurosurg. 2008;110: [35] Stephanian E, Lunsford LD, Coffey RJ, Bissonette DJ, Flickinger JC. Gamma knife surgery for sellar and suprasellar tumors. Neurosurg Clin North Am. 1992;3: [36] Stripp DC, Maity A, Janss AJ, Belasco JB, Tochner ZA, Goldwein JW, et al. Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults. Int J Radiat Oncol Biol Phys. 2004;58: [37] Suh JH, Gupta N. Role of radiation therapy and radiosurgery in the management of craniopharyngiomas. Neurosurg Clin N Am. 2006;17: [38] Symon L. Microsurgery of the hypothalamus with special reference to craniopharyngioma. Neurosurg Rev. 1983;6:43 9. [39] Ulfarsson E, Lindquist C, Roberts M, Rahn T, Lindquist M, Thoren M, et al. Gamma knife radiosurgery for craniopharyngiomas: long-term results in the first Swedish patients. J Neurosurg. 2002;97: [40] Varlotto JM, Flickinger JC, Kondziolka D, Lunsford LD, Deutsch M. External beam irradiation of craniopharyngiomas: long-term analysis of tumor control and morbidity. Int J Radiat Oncol Biol Phys. 2002;1: [41] Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, et al. Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery. 1994;35: [42] Wen DY, Seljeskog EL, Haines SJ. Microsurgical management of craniopharyngiomas. Br J Neurosurg. 1992;6: [43] Witt JA, MacCarty CS, Keating FR Jr. Craniopharyngioma (pituitary adamantinoma) in patients more than 60 years of age. J Neurosurg. 1955;12: The authors stated that there are no conflicts of interest regarding the publication of this article.