Surgery For Craniopharyngioma Research Articles

Anesthesia management in a pediatric patient with craniopharyngioma

Craniopharyngiomas are the most common sellar or suprasellar tumors in childhood. The most critical issue in neuro anesthesia is to ensure adequate cerebral perfusion pressure and appropriate surgical conditions without disturbing the autoregulation of the cerebral circulation. The purpose of this article is to provide an overview of anesthetic management in successful craniopharyngioma surgery in pediatric patients. The purpose of this article is to provide an overview of anesthetic management in successful craniopharyngioma surgery in pediatric patients. Transsphenoidal pituitary surgery was planned. The patient, who had no intraoperative complications, was extubated postoperatively and transferred to the intensive care unit for close monitoring. Non-invasive monitoring (pulse oximetry, ECG, blood pressure, and temperature monitoring) was performed. A precordial USG was prepared to detect air embolism. Moderate hypothermia of 34-36°C was achieved. The patient, who had no intraoperative complications, was extubated postoperatively and transferred to the intensive care unit for close monitoring. The goals of anesthesia for transsphenoidal pituitary surgery include optimizing cerebral oxygenation, maintaining hemodynamic stability, managing intraoperative complications, and facilitating a rapid and smooth recovery.

Transnasal endoscopic surgery of craniopharyngiomas in children

Transnasal endoscopic surgery of craniopharyngiomas in children

Qualitative descriptive study during the COVID-19 pandemic on the needs of informal caregivers of Chinese minors who underwent craniopharyngioma surgery

PurposeTo determine the needs of informal caregivers during the long-term health management of minors who have undergone craniopharyngioma surgery.Design and methodologyThis is a qualitative and descriptive research study. Purposive sampling was used to select participants, and semi-structured interviews were conducted to explore the needs of 21 informal caregivers for postoperative minors. Due to the COVID-19 pandemic, the respondents were provided with the options of online video call or offline one-on-one interviews. Following this, a content analysis method was conducted. ResultsFour themes and 14 sub-themes were extracted from the results of the study, including needs for relieving psychological stress (including psychological pressure on both minors and on caregivers); requirement for on-campus assistance (physical activity, eliminating verbal violence in schools, special education needs for child, healthcare services provided by school hospitals); demands for medical help (acquiring medical knowledge, need for medication management, convenience and reliable access to medical services, need for technological development, expectations of multidisciplinary cooperation, the necessity of health review reminders); and the desire for financial aid (charity platform assistance, inclination of government policy). ConclusionsIn China, informal caregivers of young patients with onset craniopharyngioma require both the multidisciplinary cooperation of medical institutions and the multi-departmental cooperation of society. Information and medical technology advancements may benefit families with young patients. Improving community hospitals' medical services and encouraging the practical use of online medical treatment and prescriptions are both necessary in the context of COVID-19. Practice implicationsBy identifying the needs of informal caregivers, medical professionals are able to develop care plans and interventions aimed at reducing the burden of care for minors who have undergone craniopharyngioma surgery.

The importance of preserving the superior hypophyseal artery infundibular branch in craniopharyngioma surgery.

Postoperative pituitary dysfunction, a critical problem in the treatment of craniopharyngiomas, can occur even when the pituitary stalk is preserved. We hypothesized that compromise of the primary superior hypophyseal artery (pSHA) might be related to this occurrence. We performed a retrospective review of 131 patients with craniopharyngioma who underwent surgery from April 2009 to September 2021. The inclusion criteria were initial surgery, endoscopic transsphenoidal surgery, preoperative normal pituitary function or pituitary dysfunction in one axis, and morphological preservation of the pituitary stalk. The branches of the pSHA consist mainly of the chiasmatic branches (Cb), infundibular branches (Ib), and descending branches (Db). We analyzed the association between postoperative pituitary function and preservation of these branches. Twenty patients met the criteria. Preoperative anterior pituitary function was normal in 18 patients, and there was isolated growth hormone deficiency in two patients. No patient had preoperative diabetes insipidus (DI). Anterior pituitary function was unchanged postoperatively in eight patients. Of these eight patients, bilateral preservation of pSHA Ib was confirmed in seven patients. Bilateral preservation of pSHA Ib was the only factor associated with preserved anterior pituitary function (p < 0.01). Fifteen patients were free of permanent DI, and the preservation of any given pSHA branch produced no significant difference in the postoperative occurrence of permanent DI. Our study shows that bilateral preservation of pSHA Ib provides favorable postoperative anterior pituitary function in craniopharyngioma surgery; however, such preservation may have little effect on the postoperative occurrence of DI.

Long-Term Outcome of Surgery and Radiation Treatment for Craniopharyngioma

<h3>Purpose/Objective(s)</h3> Treatment of craniopharyngioma, a rare but benign neoplasm, typically entails gross total resection (GTR) or subtotal resection followed by adjuvant radiation (STR-RT). We analyzed progression free survival (PFS) in the adult population undergoing gross total resection versus subtotal resection followed by radiotherapy (RT). <h3>Materials/Methods</h3> A retrospective review was conducted on 67 consecutive patients who were treated for craniopharyngioma. All patients received surgical resection. Patients that had STR also received postoperative radiation treatment. A median dose of 54 Gy (50.4 – 54 Gy) was used with typical dose per fraction of 1.8 Gy five times per week. Three patients were excluded from analysis due to death shortly after surgery (n=1) and no adjuvant RT following STR (n=2) for a total of 64 patients. Regular follow up examinations included contrast MRI scans, physical exams, and clinical evaluation. <h3>Results</h3> Median age was 43 years (19 – 79 years). GTR was achieved in 35 patients and STR-RT in 30 patients. Median tumor size of GTR was 8.64 cm³ (range 1.8 – 40.1 cm³, mean 13.7 cm³) and 19.6 cm³ (range 2.5 – 116.3 cm³, mean 26.2 cm³) for STR-RT (p = 0.03). At a median follow up of 84 months (4 – 255 months) local control rate for all patients treated with GTR or STR-RT was 89.3% at 2 years, 80.1% at 5 years and 73.9% at 10 years. There was one death a week following initial surgery and 2 patients who had STR with no adjuvant RT both had local failure requiring additional intervention at 9 and 26 months. Of 35 patients that had GTR, local control was 91.4% at 2 years, 74.3% at 5 years, and 65.7% at 10 years. Of 12 patients with GTR requiring additional intervention with surgery, RT, or both 100% (n = 12) are stable at last follow up. For the 30 patients with STR-RT local control was 86.7% at 2 years, 86.7% at 5 years and 83.3% at 10 years. 5 patients that underwent STR and adjuvant RT required additional surgical intervention with 100% of patients (n = 5) stable at last follow up. The hazard ratio of STR and RT compared to GTR is 0.49 (CI 0.19 – 1.26), with no significant difference of progression free survival (p = 0.33). <h3>Conclusion</h3> Long term results for patients with craniopharyngioma that underwent subtotal resection followed by radiotherapy demonstrate equivalent local control as compared to gross total resection.

Utility of hospital frailty risk score for predicting postoperative outcomes in craniopharyngioma.

Frailty is an age-associated decline in functional status leading to increased vulnerability to otherwise innocuous stressors. In neurosurgical patients, frailty has been associated with postoperative complications, increased mortality, longer hospitalization, and increased care costs for a variety of conditions. This study seeks to determine the association between frailty and postoperative outcomes in patients undergoing surgery for craniopharyngioma. The Nationwide Inpatient Sample (NIS) database was queried for patients diagnosed with craniopharyngioma who underwent surgery via either craniotomy or transsphenoidal approach. Comorbid diagnoses were used to calculate the Hospital Frailty Risk Score (HFRS) and assign patients to low (< 5), intermediate (5-15), or high-risk (> 15) categories. Logistic regression was completed to determine whether the HFRS category was predictive of mortality, postoperative complication, extended hospitalization, or increased hospital costs compared to age. Increased frailty score was predictive of increased length of stay, increased hospital costs, and non-home discharge in binary logistic regression with good discrimination on the ROC curve compared to age at admission. HFRS risk categories were significantly predictive of the development of any complication, with 100% of high-risk patients developing a complication compared to 76% of intermediate-risk and 63% of low-risk patients. HFRS risk categories were also predictive of the extended length of stay (71%, 49%, and 11% for high-, intermediate-, and low-risk, respectively) and non-home discharge (86%, 56%, and 17%). Regression analysis was unable to be performed for mortality due to the low number of deaths in the study group. In patients undergoing any surgery for craniopharyngioma, frailty is predictive of increased hospital length of stay and overall care costs. HFRS failed to independently predict mortality because the incidence of mortality is too low in this population to analyze. The HFRS is a valuable tool to identify post-operative outcomes following surgery for craniopharyngioma.

Limited surgery and conformal photon radiation therapy for pediatric craniopharyngioma: long-term results from the RT1 protocol.

Our aim was to estimate long-term disease control and complications after conformal radiation therapy (CRT) in children and adolescents with craniopharyngioma. Pediatric patients with craniopharyngioma (n = 101) were enrolled on or treated according to a phase II single institutional protocol from 1998. Surgery was individualized, and CRT (54Gy) was administered using a 1.0 cm or 0.5 cm clinical target volume margin. Patients were followed for 10 years by serial MR imaging and MR angiography and a battery of tests to measure the effects of treatment. Twenty patients had tumor progression. Twelve patients who had tumor progression died due to tumor (n = 6) or complications related to tumor or treatment (n = 6). With a median follow-up of 14.94 years for survivors, the 10 year estimates (±SE) of progression-free survival (PFS), event-free survival (EFS), and overall survival (OS) were 78.84% ± 4.10%, 77.12% ± 4.19%, and 96.02% ± 1.95%, respectively. OS, EFS, and PFS were significantly associated with race, shunt status, and tumor volume. The 10 year cumulative incidence (±SE) of the secondary tumor (1.99% ± 1.40%), secondary malignant tumor (1.0% ± 1.0%), necrosis (1.98% ± 1.39%), vasculopathy (8.47% ± 2.90%), and permanent neurologic deficits (8.28% ± 3.37%) were estimated by competing risk analysis. Three patients required revascularization surgery. Salvage therapy was successful in 13 patients using surgery and radiosurgery. Limited surgery and CRT using photons results in excellent tumor control. Tumor control and the incidence and severity of complications are associated with host, tumor, and treatment factors.

Long-term tumor control after endoscopic endonasal resection of craniopharyngiomas: comparison of gross-total resection versus subtotal resection with radiation therapy.

Surgical management of craniopharyngiomas (CPAs) is challenging. Controversy exists regarding the optimal goals of surgery. The purpose of this study was to compare the long-term outcomes of patients who underwent gross-total resection with the outcomes of those who underwent subtotal resection of their CPA via an endoscopic endonasal approach. From a prospectively maintained database of all endoscopic endonasal approaches performed at Weill Cornell Medicine, only patients with CPAs with > 3 years of follow-up after surgery were included. The primary endpoint was radiographic progression. Data were collected on baseline demographics, imaging, endocrine function, visual function, and extent of resection. A total of 44 patients with a mean follow-up of 5.7 ± 2.6 years were included. Of these patients, 14 (31.8%) had prior surgery. GTR was achieved in 77.3% (34/44) of all patients and 89.5% (34/38) of patients in whom it was the goal of surgery. Preoperative tumor volume < 10 cm3 was highly predictive of GTR (p < 0.001). Radiation therapy was administered within the first 3 months after surgery in 1 (2.9%) of 34 patients with GTR and 7 (70%) of 10 patients with STR (p < 0.001). The 5-year recurrence-free/progression-free survival rate was 75.0% after GTR and 25.0% after STR (45% in subgroup with STR plus radiotherapy; p < 0.001). The time to recurrence after GTR was 30.2 months versus 13 months after STR (5.8 months in subgroup with STR plus radiotherapy; p < 0.001). Patients with GTR had a lower rate of visual deterioration and higher rate of return to work or school compared with those with STR (p = 0.02). Patients with GTR compared to STR had a lower rate of CSF leakage (0.0% vs 30%, p = 0.001) but a higher rate of diabetes insipidus (85.3% vs 50%, p = 0.02). GTR, which is possible to achieve in smaller tumors, resulted in improved tumor control, better visual outcome, and better functional recovery but a higher rate of diabetes insipidus compared with STR, even when the latter was supplemented with postoperative radiation therapy. GTR should be the goal of craniopharyngioma surgery, when achievable with minimal morbidity.

Primary collision tumors of the sellar region: Experience from a single center

Collision tumors are extremely rare in the sellar region, and their features have not been fully characterized. Here, we report our single-center experience in the diagnosis and management of these tumors, focusing primarily on their clinicopathological features. We first performed a retrospective study of pathological reports from patients who had undergone surgery for pituitary adenoma (PA) or craniopharyngioma (CP) at our hospital. Next, to identify collision tumors, patients with a second pathological diagnosis—such as Rathke’s cleft cyst (RCC), gangliocytoma (GC), meningioma, or atypical teratoid/rhabdoid tumor (AT/RT)—were considered. Finally, the clinicopathological characteristics of these tumors were reviewed and analyzed. The results demonstrated that eleven of 2359 PA or CP cases (0.47 %) were found to exhibit sellar collision tumors; the patient cohort had a median age of 52 years (23–71) and was predominantly female (63.6 %, 7/11). In details, of the 2092 cases of PA, 10 were diagnosed with concurrent lesions (seven of RCC and one each of CP, meningioma, and GC). Of the 267 CP cases, a single patient presented with associated AT/RT. To our knowledge, this is the first reported adult case of this subtype. Notably, the preoperative CT and/or MRI of each patient revealed solely PA or CP. The endoscopic endonasal approach was the preferred surgery. In conclusion, the sellar collision tumors occur with low incidence, and the primary subtype is PA and RCC. Their definitive diagnosis depends primarily on pathological findings.

Endocrine outcomes after limited surgery and conformal photon radiation therapy for pediatric craniopharyngioma: Long-term results from the RT1 protocol.

To estimate the incidence of endocrinopathy in children and adolescents with craniopharyngioma after treatment with photon-based conformal and intensity-modulated radiation therapy (CRT). One hundred one pediatric patients were enrolled on a phase II single-institution protocol beginning in 1998 (n = 76) or followed a similar non-protocol treatment plan (n = 25). Surgery was individualized. CRT (54 Gy) was administered using a 1.0-cm or ≤0.5-cm clinical target volume margin. Patients underwent baseline and serial evaluation of the hypothalamic-pituitary axis. The 10-year cumulative incidence (CI) of growth hormone deficiency (GHD) was 68.42% (±11.27) for black patients and 94.23% (±3.57) for white patients (P = .0286). The CI of thyroid-stimulating hormone deficiency (TSHD) was 70.94% (±8.44) at 10 years for non-shunted patients and 91.67% (±10.40) at 6 years for shunted patients (P = .0260). The CI of TSHD was 100% (±14.29) at 4 years for those with diabetes insipidus (DI) and 71.36% (±8.86) at 10 years for those without DI (P = .0008). The 10-year CI of adrenocortical hormone deficiency was 70.00% (±16.15) for those with DI and 48.39% (±9.19) for those without DI (P = .0080). The 10-year CI of LH/FSH deficiency was 43.33% (±9.32) age <7 years, 61.29% (±9.11) aged 7-10 years, and 78.95% (±6.38) age ≥10 years (P < .0001). BMI was significantly greater prior to CRT in white patients with DI (P = .0004) and preexisting GHD (P = .0275). Hormone deficiencies are common in pediatric patients with craniopharyngioma and are associated with host, tumor, and treatment factors. Understanding the incidence and time to onset may facilitate intervention and patient selection for treatment.

Endoscopic Endonasal Transsphenoidal Surgery for Recurrent Craniopharyngiomas.

ObjectAlthough revision surgery for recurrent craniopharyngiomas is more challenging than primary surgery and often accompanies a higher risk of death and complications, endoscopic endonasal transsphenoidal surgery (EETS) is sometimes still an effective and reliable treatment option. In this study, we introduced the surgical outcomes of EETS for recurrent craniopharyngiomas and summarized the surgical experiences.MethodsBetween 2014 and 2018, 28 patients with recurrent craniopharyngiomas underwent 29 EETS in our department. We regarded the patient undergoing two EETS as two independent patients in statistical analysis. Of the 29 patients, 16 had undergone 1 previous surgery, 10 had undergone 2 previous surgeries, and the remaining 3 patients had undergone 3 surgeries. The extent of resection, visual and endocrine outcomes, and complications of all the patients were collected and analyzed.ResultsGross total resection was accomplished in 16 patients (55.17%), subtotal resection in 11 patients (37.93%), and partial resection in 2 patients (6.9%). Among the 22 patients with preoperative visual acuity and visual field impairment, some degree of vision improvement was observed in 18 patients, 3 patients were without visual change, and perpetual deterioration of vision occurred in one patient. The remaining six patients had normal vision before and after surgery. Postoperative endocrine tests showed that, among five patients with normal preoperative pituitary hormone function, only one patient still had normal pituitary hormone function and the other four patients had one or more hypothalamic-pituitary axes involved. None of the patients with preoperative endocrine dysfunction had endocrine function improved. Diabetes insipidus was observed in six new cases postoperatively. Cerebrospinal fluid (CSF) leakage occurred in 1 patient. One patient had bacterial meningitis, which was cured with antibiotics and a lumbar drain. No serious morbidity and mortality occurred in all patients.ConclusionsFor recurrent craniopharyngiomas, a personalized treatment plan should be developed according to the tumor characteristics and the patient's situation. There is no omnipotent method to be used for all patients. The EETS still is a safe and effective way to treat recurrent craniopharyngiomas in appropriate patients.

A Comparison of Long-Term Treatment Outcomes Between Giant and Nongiant Craniopharyngiomas

There is limited literature on outcomes after surgical treatment of giant craniopharyngiomas in adult and pediatric patients. A retrospective review of 159 patients undergoing surgery for craniopharyngiomas at a single institution was performed. Patients with giant craniopharyngiomas (maximum dimension ≥4.5 cm) were compared with nongiant tumors in terms of various clinical and radiological parameters and long-term surgical outcomes. Extent of resection was determined by postoperative magnetic resonance imaging. Factors associated with post-treatment obesity were also analyzed. Giant craniopharyngiomas (n= 66) were characterized by higher rates of childhood presentation, visual impairment, neurological deficits, multicompartmental involvement, and hydrocephalus as compared with nongiant tumors (n= 139). Giant tumors also were less likely to undergo transsphenoidal resection and were associated with a higher rate of postoperative neurological morbidity. There were no significant differences between the 2 groups in terms of extent of resection, use of postoperative radiation therapy, and long-term endocrinological outcomes. Overall recurrence rates over a mean follow-up period of 4.1 years were similar between giant and nongiant tumors; however, recurrences after presumed gross total resection/near total resection were significantly higher in the former subgroup versus the latter (39.4% vs. 18.4%; P= 0.044). Risk factors for post-treatment obesity in giant craniopharyngiomas included adult age (P= 0.001), preoperative obesity (P= 0.003), and hypothalamic involvement (P= 0.012). Gross total resection/near total resection of giant craniopharyngiomas can be achieved at rates comparable to nongiant tumors. However, there remains a greater risk of postoperative neurological morbidity. Radiation therapy mitigates the risk of recurrence on long-term follow-up.

Central Diabetes Insipidus in Children and Adolescents: Twenty-Six Year Experience from a Single Centre.

Introduction Paediatric cohorts of central diabetes insipidus (CDI) have shown varying prevalence for different causes of CDI. The objective of this study was to determine the causes of CDI and long-term outcome in children and adolescents from a Tertiary Paediatric Endocrinology unit. Methods The clinic database was searched to identify patients with CDI managed between 1993 and 2019. Relevant clinical information was collected from patient records. Results A total of 138 CDI patients, median age 6 years (range <1–18) at presentation, were identified. Principal CDI aetiologies were craniopharyngioma (n = 44), acute central nervous system (CNS) insult (n = 33), germinoma (n = 15), postneurosurgery (indication other than craniopharyngioma and germinoma, n = 20), midline CNS malformation (n = 14), Langerhans cell histiocytosis (n = 5), and familial (n = 2). Idiopathic CDI in this cohort was infrequent (n = 5). Patients with CNS malformations/infections presented with CDI at a younger age compared to patients with CNS tumours (p < 0.0001). Five patients, initially presenting as idiopathic CDI, were subsequently diagnosed with germinoma after a median interval of 3.3 years. All patients with CDI related to craniopharyngioma and nearly all (87%) patients with CDI related to germinoma had concomitant GH, ACTH, and TSH deficiency. The majority of patients who manifested CDI due to acute CNS insult either deceased (30%) or had transient CDI (33.3%). Conclusion Surgery for craniopharyngioma was the most common underlying aetiology of CDI with ubiquitous occurrence of panhypopituitarism in these patients. Manifestation of CDI in patients with acute CNS insult carries poor prognosis. We affirm that neuroimaging assessment in idiopathic CDI should be continued beyond 3 years from diagnosis as a significant number of patients exhibited progression of infundibular thickening 3 years post-CDI diagnosis.

A propensity-adjusted comparison of endoscopic endonasal surgery versus transcranial microsurgery for pediatric craniopharyngioma: a single-center study.

When comparing endoscopic endonasal surgery (EES) and transcranial microsurgery (TCM) for adult and mixed-age population craniopharyngiomas, EES has become an alternative to TCM. To date, studies comparing EES and TCM for pediatric craniopharyngiomas are sparse. In this study, the authors aimed to compare postoperative complications and surgical outcomes between EES and TCM for pediatric craniopharyngiomas. The data of pediatric patients with craniopharyngiomas who underwent surgery between February 2009 and June 2021 at a single center were retrospectively reviewed. All included cases were divided into EES and TCM groups according to the treatment modality received. The baseline characteristics of patients were compared between the groups, as well as surgical results, perioperative complications, and long-term outcomes. To control for confounding factors, propensity-adjusted analysis was performed. Overall, 51 pediatric craniopharyngioma surgeries were identified in 49 patients, among which 35 were treated with EES and 16 were treated with TCM. The proportion of gross-total resection (GTR) was similar between the groups (94.3% for EES vs 75% for TCM, p = 0.130). TCM was associated with a lower rate of hypogonadism (33.3% vs 64.7%, p = 0.042) and a higher rate of growth hormone deficiency (73.3% vs 26.5%, p = 0.002), permanent diabetes insipidus (DI) (60.0% vs 29.4%, p = 0.043), and panhypopituitarism (80.0% vs 47.1%, p = 0.032) at the last follow-up. CSF leakage only occurred in the EES group, with no significant difference observed between the groups (p > 0.99). TCM significantly increased the risk of worsened visual outcomes (25.0% vs 0.0%, p = 0.012). However, TCM was associated with a significantly longer median duration of follow-up (66.0 vs 40.5 months, p = 0.007) and a significantly lower rate of preoperative hypogonadism (18.8% vs 60.0%, p = 0.006). The propensity-adjusted analysis revealed no difference in the rate of recurrence, hypogonadism, or permanent DI. Additionally, EES was associated with a lower median gain in BMI (1.5 kg/m2 vs 7.5 kg/m2, p = 0.046) and better hypothalamic function (58.3% vs 8.3%, p = 0.027) at the last follow-up. Compared with TCM, EES was associated with a superior visual outcome, better endocrinological and hypothalamic function, and less BMI gain, but comparable rates of GTR, recurrence, and perioperative complications. These findings have indicated that EES is a safe and effective surgical modality and can be a viable alternative to TCM for pediatric midline craniopharyngiomas.

The clinical application of intraoperative visual evoked potential in recurrent craniopharyngiomas resected by extended endoscopic endonasal surgery

ObjectiveThe protection of visual function is a major challenge for recurrent craniopharyngioma due to the interface between tumor and surrounding structure (esp. optic nerve) is more unclear. Intraoperative visual evoked potential (VEP) has been integrated into transsphenoidal surgery for pituitary adenomas and primary craniopharyngiomas in the prediction of postoperative visual outcome. In the present study, we firstly analyzed the clinical value of the VEP in the prediction of postoperative visual outcome for recurrent craniopharyngiomas by extended endoscopic endonasal surgery (EEES). MethodsThirty-six patients with recurrent craniopharyngiomas who underwent EEES with intraoperative VEP monitoring were retrospectively reviewed. The association between the changes in the VEP amplitude and postoperative visual outcome was determined. In addition, other potential prognostic factors with regard to postoperative visual outcomes were also included in the analysis. ResultsGross total resection (GTR) was achieved in 32 patients (88.9%). Reproducible and stable VEP was recorded in 72 eyes. We recorded that there were 29 eyes remained stable VEP amplitude intraoperation, 4 (13.8%) of which suffered new visual acuity (VA) and 5 (17.2%) suffered new visual field (VF) defects after surgery. A transient VEP decrease was recorded in 41 eyes, 8 (19.5%) of which suffered from visual deterioration. Of 2 eyes with a permanent VEP decrease, 2 (100%) experienced postoperative visual impairment. Clinical variable analyses revealed a significant correlation between recurrent craniopharyngioma and age, adhesion with optic nerve, postoperative VA, and intraoperative VEP amplitude. Unstable VEP amplitude during operation and tight adhesion were independent risk factors for postoperative VA. Tight adhesion and a larger tumor volume were independent risk factors for postoperative VF defects. ConclusionsEEES provides a unique view for the lesions under and behind the chiasm and achieve higher rates of GTR with improved visual outcomes. Tight adhesion and unstable VEP amplitude were strong predictors of postoperative visual impairment. Recurrent craniopharyngiomas are more likely to adhere to the optic nerve, VEP is more likely to be unstable during operation, and the improvement rate of postoperative visual function is lower than primary craniopharyngiomas. So intraoperative VEP is more necessary for recurrent craniopharyngiomas.

Prognostic Utility of Optical Coherence Tomography for Visual Outcome After Extended Endoscopic Endonasal Surgery for Adult Craniopharyngiomas.

IntroductionOwing to the close vicinity of the optic chiasma, visual dysfunction is known as one of the most common surgical indications and postoperative complications in adult patients with craniopharyngiomas, probably leading to poor quality of life. Historically, very few consistent predictive factors associated with the visual outcome are identified, which may not be helpful for patient counseling and preoperative decision making. Recently, optical coherence tomography (OCT) serving as a novel high-resolution imaging technique can assess the retinal morphology by measuring the circumpapillary retinal nerve fiber layer (cpRNFL) and macular ganglion cell complex thickness. However, few studies have examined the prognostic utility of OCT parameters for visual outcome after surgery for craniopharyngiomas. This study aims to use the largest series to evaluate the association between OCT parameters and visual outcome after extended endoscopic endonasal surgery (EEES) for primary craniopharyngiomas in adults.Material and MethodsFrom October 2018 to October 2020, one hundred and seventy eyes in 88 adult patients with newly confirmed craniopharyngiomas were retrospectively reviewed and pertinent prognostic factors were analyzed.ResultsGross total resection was performed in 82 (93.2%) patients. The median postoperative follow-up time was 10.9 months. Multiple logistic regression analysis showed that increased temporal cpRNFL thickness was associated with higher odds of visual acuity (VA) improvement and maintenance (OR = 1.070; 95% CI, 1.005–1.140; p = 0.035), and greater inferior cpRNFL thickness was significantly associated with visual field (VF) improvement and maintenance (OR = 1.034; 95% CI, 1.001–1.068; p = 0.046). Furthermore, tight adhesion between optic nerves and craniopharyngiomas was demonstrated as an independent adverse factor for either postoperative VA or VF (p = 0.048, p = 0.030, respectively). The ROC results further verified the robustness of the prediction model either in VA (AUC = 0.843; 95% CI, 0.734–0.952; p < 0.001) or VF (AUC = 0.849; 95% CI, 0.741–0.958; p < 0.001).ConclusionPreoperative OCT can effectively predict visual outcome after EEES for adult craniopharyngiomas. It can also serve as a reliable alternative to evaluate preoperative visual field defects, especially for patients with lower compliance. Tight adhesion was confirmed as an independent risk factor for postoperative visual outcome. The OCT-based multivariable prediction models developed in the present study may contribute to patient counseling on visual prognosis.

Impact of Pituitary Stalk Preservation on Tumor Recurrence/Progression and Surgically Induced Endocrinopathy After Endoscopic Endonasal Resection of Suprasellar Craniopharyngiomas.

Objective: To investigate the factors associated with recurrence/progression after endoscopic endonasal resection of suprasellar craniopharyngiomas. Special attention was paid to assess the impact of pituitary stalk preservation on tumor recurrence/progression and endocrinological outcomes.Methods: We retrospectively recruited 73 patients with suprasellar craniopharyngiomas undergone endoscopic endonasal approach (EEA) surgery from September 2014 to May 2019 and assessed their clinical characteristics, surgical outcomes, and recurrence/progression. Stalk preservation or sacrifice was determined by reviewing operative records, videos, and post-operative magnetic resonance imaging.Results: Gross total resection (GTR) was achieved in 51 cases (69.9%). Tumor recurrence was seen in 5 cases (9.8%) and progression was seen in 8 cases (36.4%), respectively. GTR (OR = 0.248 CI 0.081–0.759; p = 0.015) was the only independent factor influencing recurrence/progression. Kaplan-Meier survival analysis showed that the mean recurrence/progression-free survival were 53 (95% CI 48–59) and 39 (95% CI 28–50) months, respectively, in patients with and without GTR (p = 0.011). Pituitary stalk preservation was more common in cases with peripheral type tumors (83% vs. 30%, p < 0.01). Preserving the pituitary stalk does not appear to decrease the percentage of GTR (75.5% vs. 55.0%, p = 0.089), or increase the rate of tumor recurrence (12.5% vs. 0%, p = 0.508) or progression (46.2% vs. 22.2%, p = 0.486). However, surgically induced hypothyroidism (60.5% vs. 100%, p = 0.041) and diabetes insipidus (35.1% vs. 81.8%, p = 0.017) were significantly lower in patients with stalk preservation. For patients who had hypopituitarism before EEA, there was no difference between those with and without stalk preservation regarding post-operative hypopituitarism (p > 0.05).Conclusion: GTR is the only independent predictor of recurrence/progression after EEA surgery for suprasellar craniopharyngiomas. Preserving the pituitary stalk does not appear to increase the risk of non-GTR and tumor recurrence/progression and might help reduce the risk of surgically induced hypothyroidism and diabetes insipidus. We recommend preserving the pituitary stalk in peripheral type suprasellar craniopharyngiomas with normal pituitary function, especially in cases without hypothyroidism or diabetes insipidus. On the other hand, stalk sacrifice could be considered in central type tumors with severe pre-operative endocrinopathy.

Proton Radiation Therapy for Pediatric Craniopharyngioma.

Radiation therapy (RT) is used for pediatric craniopharyngioma in the definitive, adjuvant, or salvage settings. Proton RT may be useful owing to tumor proximity to eloquent anatomy. We report clinical outcomes for a large cohort treated with proton therapy. We conducted a retrospective review of pediatric patients (≤21 years) treated with surgery and proton therapy for craniopharyngioma between August 2002 and October 2018. Clinical characteristics, treatment course, and outcomes were recorded. Acute toxicity was graded using Common Terminology Criteria for Adverse Events, version 5.0. Late toxicity was assessed using neuroendocrine, neuro-ophthalmologic, and neuropsychological testing. Among 77 patients, median age at diagnosis was 8.6 years (range, 1.3-20); median age at radiation was 9.6 years (range, 2.3-20.5). Most common presenting symptoms were headache (58%), visual impairment (55%), and endocrinopathy (40%). Patients underwent a median of 2 surgical interventions (range, 1-7) before protons. At initial surgery, 18% had gross total resection, 60% had subtotal resection, and 22% had biopsy/cyst decompression. Median RT dose was 52.2 Gy (relative biologic effectiveness). Common acute toxicities were headache (29%), fatigue (35%), and nausea/vomiting (12%). Only 4% developed any acute grade 3 toxicity. Nine patients experienced cyst growth requiring replanning or surgical decompression. At a median of 4.8 years from RT (range, 0.8-15.6), there were 6 local failures and 3 deaths, 2 related to disease progression. Effect of tumor and treatment contributed to late toxicity including Moyamoya syndrome (13%), visual impairment (40%), and endocrine deficiency requiring hormone replacement (94%). Subclinical decline in functional independence and adaptive skills in everyday life was detected at follow-up. Surgery and proton therapy results in excellent disease control for pediatric craniopharyngioma. Severe acute toxicity is rare. Late toxicities from tumor, surgery, and radiation remain prevalent. Endocrine and ophthalmology follow-up is necessary, and neuropsychological testing may identify patients at risk for treatment-related cognitive and adaptive functioning changes.

Risk Factors for Hypothalamic Obesity in Patients With Adult-Onset Craniopharyngioma: A Consecutive Series of 120 Cases.

ContextHypothalamic obesity (HO) is a severe complication following craniopharyngioma, but studies regarding the sequelae in adult-onset patients with craniopharyngioma are sparse.ObjectiveThe objective of the study was to describe weight changes after surgical treatment in adult-onset craniopharyngioma patients and to analyze risk factors for postoperative weight gain and HO.Subjects and MethodA retrospective analysis was conducted of 120 adult-onset patients who underwent surgery for craniopharyngioma and follow-up at the institution of the authors between January 2018 and September 2020. Clinical characteristics, anthropometric data, image features, treatment modalities, and endocrine indices were collected. Multivariable logistic regression analysis was used to identify independent risk factors for postoperative weight gain and HO.ResultsForty-nine (40.8%) patients had clinically meaningful weight gain (≥5%) in a median follow-up time of 12.0 months (range 1.0–41.0 months) after surgery. The mean postoperative weight gain in this subgroup was 17.59 ± 12.28 (%). Weight gain continued in the first year following surgery. Patients with lower preoperative BMI [OR 0.78, 95% CI (0.67–0.90), P = 0.001] and the adamantinomatous subtype [OR 3.46, 95% CI (1.02–11.76), P = 0.047] were more likely to experience postoperative weight gain ≥5%. The prevalence of HO was 19.2% preoperatively and increased to 29.2% at last follow-up postoperatively. Only preoperative BMI [OR 2.51, 95% CI (1.64–3.85), P < 0.001] was identified as an independent risk factor for postoperative HO.ConclusionsHO is a common complication in patients with adult-onset craniopharyngioma. Patients with higher preoperative BMI had a greater risk for developing HO postoperatively.

Comparison between extended transsphenoidal and transcranial surgery for craniopharyngioma: focus on hypothalamic function and obesity.

Patients suffering from craniopharyngiomas currently have good survival rates, but long-term sequelae, such as development of obesity, worsen their quality of life. Optimal treatment is still controversial and changed during the decades, becoming less aggressive. Transcranial (TC) surgery was the first approach to be used, followed by extended transsphenoidal (eTNS) access. This study aims to compare the two approaches in terms of risk of hypothalamic damage leading to obesity. This is a monocentric retrospective analysis of post-puberal patients treated for primary craniopharyngioma. Postoperative obesity and percentual postsurgical BMI variation were considered proxy for hypothalamic function and used to fit regression models with basal BMI, type of surgery, tumor volume and hypothalamic involvement (anterior vs. anteroposterior). No difference in radicality was observed between the two approaches; eTNS was more effective in ameliorating visual function but was significantly associated with CSF leaks. The TC approach was associated with a higher incidence of diabetes insipidus. Regression analysis showed only tumor volume and basal BMI resulted as independent predictors for both postoperative obesity (respectively, OR 1.15, P = 0.041, and OR 1.57, P < 0.001) and percentual BMI variation (respectively, + 0.92%, P = 0.005, and - 1.49%, P = 0.001). Larger lesions portend a higher risk to develop postoperative obesity, independently of hypothalamic involvement. Interestingly, basal BMI is independent of lesional volume and is associated with postoperative obesity, but lesser postoperative BMI variation. The surgical approach does not influence the obesity risk. However, eTNS proves valid in managing large tumors with important hypothalamic invasion.