Treatment Of Cystic Craniopharyngiomas Research Articles

Brachytherapy in craniopharyngiomas: a systematic review and meta-analysis of long-term follow-up

ObjectiveBrachytherapy has been indicated as an alternative option for treating cystic craniopharyngiomas (CPs). The potential benefits of brachytherapy for CPs have not yet been clarified. The purpose of this work was to conduct a meta-analysis to analyze the long-term efficacy and adverse reactions profile of brachytherapy for CPs.Materials and methodsThe relevant databases were searched to collect the clinical trials on brachytherapy in patients with CPs. Included studies were limited to publications in full manuscript form with at least 5-year median follow-up, and adequate reporting of treatment outcomes and adverse reactions data. Stata 12.0 was used for data analysis.ResultsAccording to the inclusion and exclusion criteria, a total of 6 clinical trials involving 266 patients with CPs were included in this meta-analysis. The minimum average follow-up was 5 years. The results of the meta-analysis showed that 1-year, 2–3 years and 5 years progression free survival rates (PFS) are 75% (95%CI: 66-84%), 62% (95%CI: 52-72%) and 57% (95%CI: 22-92%), respectively. At the last follow-up, less than 16% of patients with visual outcomes worser than baseline in all included studies. While, for endocrine outcomes, less than 32% of patients worser than baseline level.ConclusionIn general, based on the above results, brachytherapy should be considered as a good choice for the treatment of CP.

Benefit of Intracystic Bleomycin for Symptomatic Recurrent Rathke Cleft Cyst.

Management of recurrent Rathke cleft cysts (RCC) is problematic. The mainstay of treatment has been reoperation with cyst drainage. Radical cyst resection, although effective, results in a high incidence of diabetes insipidus. Several case reports suggest a potential benefit to radiation therapy or the use of intracystic bleomycin. The intracystic application of bleomycin is known to be beneficial in the treatment of cystic craniopharyngioma; however, its usefulness in the treatment of recurrent RCC has yet to be proven. To present our 6-yr experience using intracystic bleomycin for recurrent RCC. We performed a retrospective chart review of patients with RCC who underwent surgical resection between January 2010 and May 2016 by a single surgeon. Specific attention was paid to patients with recurrent RCC. Of the 59 patients operated on for RCC during this 6-yr interval, 6 patients with symptomatic recurrent RCC were identified and received intracystic bleomycin at the time of reoperation. To date, no symptomatic cyst recurrence has been documented in the patients receiving bleomycin (mean=38.8 mo, range 21.2-79.8 mo). The use of intracystic bleomycin appears to be a safe and potentially effective treatment option in patients with recurrent RCC. Additional studies with longer follow-up are needed to further define the role of bleomycin in recurrent RCC.

Long-term outcomes of stereotactic intracavitary brachytherapy for cystic craniopharyngiomas

Objective To assess the long-term outcomes of stereotactic intracystic brachytherapy for the treatment of cystic craniopharyngiomas. Methods A total of 141 consecutive patients with cystic craniopharyngioma treated by stereotactic intracystic phosphorus-32 at Department of Neurosurgery, Navy General Hospital of PLA from January 1996 to December 2015. There were 85 males and 56 females and the mean age was 40.1 years (range: 3-72 years). The calculated cumulative beta dose aimed at the inner surface of the cyst wall was 250 Gy. Results All patients were followed up for 1.0-19.5 years with an average of 9.7±4.5 years. The decreased visual acuity had improved in 68.6% (59/86) of the patients in long-term result. The total tumor response rate was 86.5% (122 cases) including the total response accounting for 44.0% (62 cases), partial response accounting for 36.2% (51 cases) and stable response accounting for 6.3% (9 cases). The rate of no response was 13.5% (19 cases). The recurrence rate was 17% (24 cases). The side effects that occurred during follow-up period included worsening of visual acuity in 13 (9.2%), the third nerve palsy in 2 (1.4%), internal carotid artery occlusion in 1(0.7%), new-onset sexual dysfunction in 5 (3.5%), diabetes insipidus in 7 (5.0%), obesity in 12 (8.5%) and cognitive dysfunction in 10 (7.1%) patients, respectively. The late complication rate was 35.5% (50/141). The mean survival after treatment was 120 months. The actuarial 5-, 10- and 15-yr survival rates were 97.9% (138 cases), 92.9% (131 cases) and 89.3% (126 cases), respectively. Conclusion Stereotactic intracavitary brachytherapy is a valuable treatment alternative for cystic craniopharyngioma, particularly for patients with high surgical risk and recurrent tumors. Key words: Cystic craniopharyngioma; Stereotactic radiosurgery; Phosphorus radioisotopes; Treatment outcome; Long-term

Treatment of Cystic Craniopharyngioma with Intracystic Stereotactic Instillation of Phosphorus 32.

Cystic craniopharyngiomas are considered the most common intracranial nonglial tumor in children with the tendency for cyst formations. The aim of this study was to evaluate the effect of intracystic phosphorus 32 (P32) therapies on controlling the growth of the cystic component of craniopharyngioma. This clinical study was conducted on 47 patients with cystic craniopharyngioma from March 1998 to June 2012 at Shohada Tajrish Hospital, Tehran, Iran. Patients were treated with stereotactic intracystic P32. The mean cyst volume was 23.5 ml, and the dose of radiation to the inner cyst wall was 250 Gy. The overall response rate was 78.1% and the mean survival was 113.1±11months. The survival rate at 1, 3, 5, and 10 years after p32 therapy was 91%, 77%, 73%, and 52%, respectively. There was no mortality related to the procedure and no visual or endocrinal deterioration. Visual improvement occurred in 88% of patients presented with recent deterioration due to the cyst enlargement. Intracystic p32 therapy was an effective and almost safe procedure for the treatment of cystic component of craniopharyngioma.

Cystic Craniopharyngiomas: Microsurgical or Stereotactic Treatment?

Prognosis and treatment of cystic craniopharyngiomas are poorly defined. To analyze progression-free survival (PFS) and safety profile of cystic craniopharyngiomas undergoing resection or minimally invasive drainage procedures. We compared further outcome measurements for cystic and solid tumors undergoing resection to elucidate the impact of the initial tumor composition on both PFS and the toxicity profile. All patients with craniopharyngiomas consecutively treated between 1999 and 2014 were included. A treatment decision in favor of microsurgery or stereotactic treatment was made interdisciplinarily. For stereotactic drainage, a catheter was implanted, allowing both permanent upstream (into ventricular spaces) and downstream (into prepontine cistern) drainage. Study endpoints were tumor progression, functional outcome, and treatment toxicity. Functional endocrinological and visual outcome analyses referred to data obtained preoperatively and 6weeks after treatment. The Kaplan-Meier method was used for survival analysis. Prognostic factors were obtained from proportional hazard models. Seventy-nine patients were included. The distribution of clinical and tumor-related data was well balanced among patients with solid (n = 35) and cystic (n = 44) tumors and those undergoing microsurgical or stereotactic treatment. Cystic tumors had shorter PFS (5-year PFS: 53.6% vs 66.8%, P = .10) and needed significantly more therapeutic interventions, which was independent of the initial treatment mode. The endocrinological deterioration rate was high for both solid and cystic tumors after microsurgery (59.4% and 85.7%, respectively), whereas it was significantly lower for cystic tumors undergoing stereotactic treatment (23.1%, P < .001). Stereotactic bidirectional drainage of cystic craniopharyngiomas is effective and provides a better endocrinological outcome than conventional microsurgery.

Neuroendoscopy Followed by Radiotherapy in Cystic Craniopharyngiomas—a Long-Term Follow-Up

Treatment of cystic craniopharyngiomas is often complicated because of adherence of craniopharyngiomas to adjacent brain structures. A strategy involving neuroendoscopic procedures followed by stereotactic radiotherapy is less invasive, avoiding brain damage. However, long-term follow-up of this strategy has not been investigated in depth. Cystic craniopharyngiomas in 9 patients were treated with neuroendoscopic cyst aspiration and fenestration, followed by fractionated stereotactic radiotherapy (FSRT). The neuroendoscopic procedure focused on widening of cyst fenestration and extensive irrigation of the cyst contents. FSRT was performed using 6 fixed beams, an 8-mm margin to cover the gross tumor volume with a 95% isodose line, and a target delivered dose of 50.4 Gy in 28 fractions. The median follow-up period was 72.9 months. Tumor control was achieved in 8 of 9 patients (88.9%). Marked tumor volume reduction was obtained with the neuroendoscopic procedure alone (26.1%) at 6 months (20.4%), 1 year (11.0%), and 2 years (3.1%). One recurrent case showed multilobulated cysts, and a second surgery was required 1 year after the treatment. Clinical symptoms such as headache and visual disruption were rapidly alleviated after the neuroendoscopic procedure. No new visual disturbances, endocrinopathy, or hypothalamic dysfunction was observed during follow up. Neuroendoscopic cyst aspiration and fenestration followed by FSRT is a less invasive, powerful strategy for treating adult cystic craniopharyngiomas.

Treatment of Cystic Craniopharyngioma by Endocavitary Instillation of Yttrium90 Radioisotope—Still a Valuable Treatment Option

Craniopharyngiomas are a challenging pathology in neurosurgery because of their anatomic localization in the (supra)sellar region and their contact with diencephalic structures around the third ventricle. Among the different treatment modalities, stereotactic intracavitary treatment by instillation of yttrium⁹⁰ radioisotope is a minimally invasive technique of particular use in the treatment of cystic or partially cystic craniopharyngiomas. It can be performed under local anesthesia during a short hospitalization and has a long-lasting effect. This treatment can be repeated or used in combination with other treatment modalities such as microsurgery, endoscopy, conformal external radiation therapy, or stereotactic radiosurgery. Thus, this old and perhaps almost forgotten treatment option is still valuable in the treatment of cystic craniopharyngiomas.

Surprising outcome of ommaya reservoir in treating cystic craniopharyngioma: a retrospective study

Background. Surgical removal (gross total or partial removal), radiotherapy and cyst evacuation have all been used individually or in combination to treat cystic craniopharyngioma, although it is unclear which is the best method. Objective. To report the results of treating cystic craniopharyngioma by insertion of an Ommaya reservoir system (ORS) and aspiration of the cyst. Patients and methods. As from 1990, patients admitted to the neurosurgical department at Assiut University Hospital, Egypt, with grossly cystic craniopharyngioma and who had a single cyst and did not have previous surgery were treated by insertion of an ORS and drainage of the cyst. By 2010, 52 patients had received this treatment with the cystic fluid aspirated completely on the day of surgery. The main presenting symptoms were raised intracranial pressure and visual changes with hormonal changes observed in some patients. The minimal follow-up period was 7 years. Results. To our surprise, 38 (73%) patients did not develop any recollection of the cyst and showed significant clinical improvement. The only possible explanation is that the part of the catheter of the ORS, with holes in, has established communication between the cyst and the CSF spaces around it after the collapse of the cyst with no adverse effect on the patient at any time. Ten (19%) patients needed reaspiration every 6 months and four (8%) patients showed rapid recollection of cystic fluid and were treated with intracystic bleomycin. Conclusion. Treatment of cystic craniopharyngioma by drainage through an ORS is very effective. The majority of patients do not need any further treatment. Those who develop re-accumulation of cystic fluid are easily treated by simple aspiration of the fluid through the reservoir. It is a simple and safe method, which lacks the risks associated with surgery or chemotherapy.

Intracystic Bleomycin for Cystic Craniopharyngiomas in Children (Abridged Republication of Cochrane Systematic Review)

Craniopharyngiomas are the most common benign histological tumors to involve the hypothalamopituitary region in childhood. When the tumor location is unfavorable, a gross total or partial resection followed by radiotherapy is the main treatment option in adults. However, it presents the risk of morbidity, especially for children. Intracystic bleomycin has been used to potentially delay the use of radiotherapy or radical resection to decrease morbidity. To determine the benefit and harm of intracystic bleomycin vs other treatments for cystic craniopharyngiomas in children. We searched the electronic databases of CENTRAL, MEDLINE/PubMed, and EMBASE/Ovid with prespecified terms. In addition, we searched reference lists of relevant articles and reviews, conference proceedings, and ongoing trial databases. We could not identify any studies in which the only difference between the treatment groups was the use of intracystic bleomycin. We did identify a randomized, controlled trial comparing intracystic bleomycin with intracystic P (n = 7 children). The trial had a high risk of bias. Survival could not be evaluated. There was no evidence of a significant difference in cyst reduction, neurological status, third nerve paralysis, fever, or total adverse effects between the treatment groups. There was a significant difference in favor of the P group for the occurrence of headache and vomiting. Based on the currently available evidence, we are not able to give recommendations for the use of intracystic bleomycin in the treatment of cystic craniopharyngiomas in children. High-quality randomized, controlled trials are needed.

Vasculopathy after intracystic bleomycin administration for a recurrent cystic craniopharyngioma

Intracystic bleomycin (ICB) administration is known to be effective in the treatment of cystic craniopharyngiomas (CRPs) and rarely causes serious complications. The authors report a case of vasculopathy after ICB injection for a recurrent cystic CRP. A 5-year-old boy presented with the cystic recurrence of a CRP. A catheter and Ommaya system were inserted into the cyst, followed by a leakage test 2 weeks later. Bleomycin was delivered at 3 mg per week for 14 weeks (total dose 42 mg). Four months later, the patient's activity decreased and right hemiparesis occurred. Magnetic resonance imaging and cerebral angiography demonstrated a recurrent multicystic mass and infarction at the left middle cerebral artery territory with stenosis/occlusion of the left distal internal carotid artery and posterior cerebral artery. During the operation to remove the recurrent cystic mass, fibrotic stenoocclusion of the left intracranial arteries was identified. The cyst was totally removed. However, additional multiple border zone infarctions occurred in the left hemisphere, and the patient became wheelchair bound. Leakage of ICB was considered as the most probable cause of the cerebral vasculopathy and corresponding infarction. A leakage test may not always guarantee a leakage-free status even if the test is negative. Direct bypass surgery should have been considered to prevent a high risk of postoperative infarction in this case of a recurrent CRP with confirmed vasculopathy.

Does the choice of surgical approach to insert an intratumoral catheter influence the results of intratumoral cystic treatment?

Background In the literature, only a few articles are related to the surgical approach for insertion of an intratumoral catheter. No one has evaluated the complications related to the different surgical techniques for the treatment of pediatric cystic craniopharyngiomas. Methods A cooperative, multicenter (France and Brazil) study was carried out and included 50 patients (aged between 9 months and 21 years) diagnosed as having cystic craniopharyngioma treated between 1990 and 2000. Forty-nine children were available for the final analysis. The patients were divided into 3 groups: group I, 24 children who underwent a craniotomy and catheter placement under direct vision using a surgical microscope; group II, 14 children who were submitted to a stereotactic approach for the placement of the intratumoral catheter; and group III, 11 children whose catheters were placed by a freehand approach through a burr hole. Results Eight children (16.3%) presented complications related to the placement of the catheter, namely, misplacement or leakage. The rate of the complications did not appear to be related to one specific modality of the catheter insertion. Conclusion Intracystic antiblastic drug injection is one available option in the treatment of cystic craniopharyngiomas. Because of the toxic effect of these drugs on the brain, determination of the exact placement to avoid leakage is mandatory for correct treatment. However, the results of the present study appear to indicate an excessively high incidence of complications whichever technique is used.

Management of cystic craniopharyngiomas with intracavitary irradiation with 32P.

Cystic craniopharyngiomas are the most frequent intracranial neoplasm of nonglial origin in children. Follow-up data were analyzed to assess the value of intracavitary irradiation with stereotactically applied 32P radioisotopes for the treatment of patients with craniopharyngioma cysts admitted to Shohada Tajrish Hospital, Tehran, Iran, between 1998 and 2005. Patients with predominantly cystic craniopharyngiomas, who underwent stereotactic intracavitary irradiation, were followed for tumor response and complications. Beta-emitting 32P isotopes were injected into cysts using a computed tomography-guided and computer-assisted three-dimensional stereotactic treatment planning and application system. The cumulative dose to the inner surface of the cyst wall was 250 Gy. Twenty-two (12 females and 10 males) patients with a mean+/-SD age of 14.0+/-6.6 (range: six to 35) years were studied. The tumor response rate gained with 32P-labeled chromic phosphate was 73% (16 of 22 cysts). The mean+/-SD survival after intracavitary irradiation was 25.4+/-6.8 (95% CI: 12.0 - 38.7) months. Intracavitary irradiation using 32P is highly effective in the treatment of cystic craniopharyngiomas. In patients with solitary cyst treated exclusively with this method, it has been the only necessary therapy over a long period. It seems reasonable to recommend intracavitary irradiation as the initial treatment for selected patients and as palliative therapy in those with recurrence.

Craniopharyngiome kystique et irradiation endocavitaire par du 186-rhenium : etude rétrospective sur 50 patients

Treatment of cystic craniopharyngiomas is often complicated because of adherence of craniopharyngiomas to adjacent brain structures. A strategy involving neuroendoscopic procedures followed by stereotactic radiotherapy is less invasive, avoiding brain damage. However, long-term follow-up of this strategy has not been investigated in depth.Cystic craniopharyngiomas in 9 patients were treated with neuroendoscopic cyst aspiration and fenestration, followed by fractionated stereotactic radiotherapy (FSRT). The neuroendoscopic procedure focused on widening of cyst fenestration and extensive irrigation of the cyst contents. FSRT was performed using 6 fixed beams, an 8-mm margin to cover the gross tumor volume with a 95% isodose line, and a target delivered dose of 50.4 Gy in 28 fractions.The median follow-up period was 72.9 months. Tumor control was achieved in 8 of 9 patients (88.9%). Marked tumor volume reduction was obtained with the neuroendoscopic procedure alone (26.1%) at 6 months (20.4%), 1 year (11.0%), and 2 years (3.1%). One recurrent case showed multilobulated cysts, and a second surgery was required 1 year after the treatment. Clinical symptoms such as headache and visual disruption were rapidly alleviated after the neuroendoscopic procedure. No new visual disturbances, endocrinopathy, or hypothalamic dysfunction was observed during follow up.Neuroendoscopic cyst aspiration and fenestration followed by FSRT is a less invasive, powerful strategy for treating adult cystic craniopharyngiomas.

Use of interferon alpha in intratumoral chemotherapy for cystic craniopharyngioma

This study analyzed the intratumoral activity of interferon alpha (IFN-alpha) in the treatment of cystic craniopharyngiomas. From January 2000 to January 2004, nine patients presenting with cystic craniopharyngiomas were treated with intratumoral injection of IFN-alpha at the Pediatric Oncology Institute of the Federal University of São Paulo-Escola Paulista de Medicina. Age ranged from 1 year and 10 months to 18 years (mean 10 years). All intratumoral catheters were inserted by a subfrontal approach. Doses varied from 36 to 108 MU. There was complete disappearance of the lesion in seven cases. In two cases, partial reduction of tumor size was observed at follow-up. Follow-up varied from 1 year to 3 years and 6 months (mean 1 year 8 months). IFN-alpha proved to be an effective drug in the control of cystic craniopharyngiomas. Additional studies should be carried out to determine the optimal dose of IFN-alpha in the treatment of cystic craniopharyngioma. In addition, other drugs possessing high efficacy and low neurotoxicity should be analyzed.

Fractionated stereotactic radiotherapy for craniopharyngiomas

Purpose : To investigate outcome and toxicity after fractionated stereotactic radiation therapy (FSRT) in patients with craniopharyngiomas. Methods and Materials : Twenty-six patients with craniopharyngiomas were treated with FSRT between May 1989 and February 2001. Median age was 33.5 years (range: 5–57 years). Nine patients received FSRT after surgery as primary treatment, and 17 patients were irradiated for recurrent tumor or progressive growth after initial surgery. Median target dose was 52.2 Gy (range: 50.0–57.6 Gy) with conventional fractionation. Follow-up included MRI and neurologic, ophthalmologic, and endocrinologic examinations. Results : The median follow-up was 43 months (range: 7–143 months). The actuarial local control rate and actuarial overall survival rates were 100% and 100%, respectively, at 5 years and 100% and 83%, respectively, at 10 years. Four patients showed complete response, 14 patients showed partial response, and 8 patients remained stable. In 5 patients, vision improved after radiation therapy. Acute toxicity was mild. One patient required cyst drainage 3 months after radiotherapy. Late toxicity after radiotherapy included impairment of hormone function in 3 out of 18 patients at risk. We did not observe any vision impairment, radionecrosis, or secondary malignancies. Conclusions : FSRT is effective and safe in the treatment of cystic craniopharyngiomas. Toxicity is extremely low using this conformal technique.

Intracystic chemotherapy with bleomycin in the treatment of craniopharyngiomas

Since bleomycin has not yet been used very frequently in the treatment of patients with craniopharyngioma, it seemed important to document the course of a series of such patients treated with this preparation. Local chemotherapy with bleomycin was performed in 24 patients (20 children and 4 adults), 16 of whom presented with cystic or mixed (solid/cystic) craniopharyngioma and 8, with recurrent cystic craniopharyngioma. The drug was administered through an Ommaya reservoir, which was placed either by using a direct surgical approach (6 patients) or a stereotactic approach (16 patients), or with endoscopic assistance in patients with hydrocephaly (2 patients). Injection of bleomycin was always preceded by a water-tightness test. Each patient received a 3-mg dose of bleomycin every other day. The total dose of bleomycin injected ranged from 28 mg to 150 mg. Most patients (17, or 70%) were treated only with intracystic chemotherapy. Chemotherapy was followed by surgery in 7 patients. Five were operated on at the beginning of our study, and 2 required surgery because chemotherapy yielded poor results. A toxic dose was injected in 1 patient only: a severe complication, i.e. blindness, was observed. The follow-up period ranged from 2 years to 10 years. Our results show that bleomycin can be an alternative in the treatment of cystic craniopharyngiomas or cystic recurrences, as it reduces surgical morbidity and improves clinical results.

Cystic craniopharyngioma: long-term results after intracavitary irradiation with stereotactically applied colloidal beta-emitting radioactive sources.

Long-term follow-up data were analyzed to assess the value of intracavitary irradiation with stereotactically applied beta-emitting radioisotopes for the treatment of craniopharyngioma cysts. Sixty-two of 70 consecutive patients with predominantly cystic craniopharyngiomas were selected for retrospective analysis. Beta-Emitting isotopes were injected intracystically using a computed tomography-guided and computer-assisted three-dimensional stereotactic treatment planning and application system (cumulative dose to the inner surface of the cyst wall, 200 Gy). The tumor response rate gained with yttrium-90-labeled silicate (66 of 78 cysts) or phosphorous-32-labeled chromic phosphate (8 of 78 cysts) was 79.5%. Four cysts treated with rhenium-186-labeled sulfate did not respond. Mean survival after intracavitary irradiation was 9.0 +/- 0.9 years (median follow-up, 11.9 yr). In patients with solitary cysts, the mean survival was 12.5 +/- 1.4 years (actuarial 5- and 10-yr survival rates, 80 and 64%, respectively). Six months postoperatively, visual deficits (38 of 62 patients) had improved in 23 patients and were stable in 15 patients. The side effects that occurred 6 to 12 months after treatment with yttrium-90 were complete blindness (three patients), worsening of visual field cuts (one patient), third nerve palsy (one patient), and diabetes insipidus and/or panhypopituitarism (three patients). Intracavitary irradiation using yttrium-90 or phosphorous-32 is highly effective in the treatment of cystic craniopharyngiomas. If applied as initial treatment in patients with solitary cysts, it is the only required therapy over a long period.

Intracapsular irradiation therapy of craniopharyngiomas with radioactive gold: indication and follow-up results.

Sixteen cases out of 27 patients with craniopharyngiomas were arbitrarily subjected to combined treatment of simple surgical evacuation and intracapsular irradiation with 198-Au. Follow-up studies were performed on 15 cases and they ranged from 6 months to 11 years. One patient was omitted from the study because of a short postoperative period. Immediate postoperative morbidity and the endocrine functions at the end of the follow-up study were compared with those of the patients who underwent extensive surgical resection of the tumors. Intracapsular irradiation with 198-Au was found to have satisfactory effects in the treatment of cystic craniopharyngioma, especially in recurrent cases of initially solid tumors, with respect to the preservation of the endocrine functions and the daily activity of the patients. The immediate postoperative hazards in the patients' care were also much less and they were found to be easily manageable. The patients, who had been followed up for over 5 years, maintained an occupational IQ score in the normal range and the patients under school age were all able to continue their school lives. One of the female patients, who had been married after the treatment, could have two children without any specific replacement therapy, and another patient in childhood who had shown physical retardation due to HGH deficiency, showed favorable results with crescormon administration in comparison with cases of extensive resection. Although the dosimetric value of 198-Au should be varied according to the size and thickness of the capsule, it was found that 15 to 30 mCi of 198-Au was the appropriate dosis for treatment.