Recurrence Of Craniopharyngioma Research Articles

The CD44s Isoform is a Potential Biomarker for Predicting Craniopharyngioma Recurrence in Children

Adamantinomatous craniopharyngioma (ACP) is an intracranial tumor considered partly malignant due to its ability to infiltrate surrounding structures and tendency to relapse despite radical resection. CD44 is a known stem cell marker in ACP and is upregulated in cell clusters of invasive ACP protrusions; however, the functions of its alternative splicing isoform variants, CD44s and CD44v1-10, have not yet been studied in terms of ACP recurrence, despite their confirmed roles in cancer development and progression. In this study, we first confirmed the difference in total CD44 expression between samples from patients who experienced relapse and those from patients who did not. Moreover, our findings showed that, in recurrent samples, the predominant isoform expressed was CD44s, which might indicate its significance in predicting ACP recurrence. The association between increased CD44 expression and recurrence may lead to the development of prognostic markers of ACP aggressiveness and relapse potential; however, further studies are needed to clarify the exact mechanism of CD44 expression.

Topography and Radiological Variables as Ancillary Parameters for Evaluating Tissue Adherence, Hypothalamic-Pituitary Dysfunction, and Recurrence in Craniopharyngioma: An Integrated Multidisciplinary Overview.

Craniopharyngiomas continue to present a challenge in clinical practice due to their heterogeneity and unpredictable adherence to vital neurovascular structures, particularly the hypothalamus. This results in different degrees of hypothalamus-pituitary axis dysfunction and a lack of uniform consensus and treatment guidelines regarding optimal management. MRI and CT are complementary techniques in the preoperative diagnostic phase, enabling the precise definition of craniopharyngioma size, shape, and consistency, as well as guiding classification into histopathological subtypes and topographical categories. Meanwhile, MRI plays a crucial role in the immediate postoperative period and follow-up stages by identifying treatment-related changes and residual tumors. This pictorial essay aims to provide an overview of the role of imaging in identifying variables indicative of the adherence degree to the hypothalamus, hypothalamic-pituitary dysfunction, the extent of surgical excision, and prognosis. For a more comprehensive assessment, we choose to distinguish the following two scenarios: (1) the initial diagnosis phase, where we primarily discuss the role of radiological variables predictive of adhesions to the surrounding neurovascular structures and axis dysfunction which may influence the choice of surgical resection; (2) the early post-treatment follow-up phase, where we discuss the interpretation of treatment-related changes that impact outcomes.

Complication of the use of bleomycin in the management of cystic craniopharyngioma: case report

Complication of the use of bleomycin in the management of cystic craniopharyngioma: case report

Growth Hormone treatment in children with Growth Hormone deficiency, idiopathic short stature, SHOX gene mutation, small for gestational age and Turner syndrome

To describe the evolution of a group of children who received GH in a tertiary center between 2012-2022. Descriptive, retrospective study. We analyzed the impact on height after GH use with Z-score according to etiology, age at onset and bone age. Patients under 15 years old at baseline and receiving GH for at least 12 months, with diagnoses of GH deficiency (GHD), idiopathic short stature (ISS), small for gestational age (SGA), SHOX Haploinsufficiency (SHOX) and Turner syndrome (TS) were included. Height was expressed as Z-score for age and sex, according to NCHS curves. 145 children received GH. Sixty patients were excluded due to irregular administration, incomplete data, less than 12 months of GH, change of hospital, and associated comorbidities. Seventy-three patients were analyzed, 23 GHD, 15 ISS, 20 SGA, 9 SHOX and 6 TS patients. Significant improvement in height (Z-score for age and sex) was observed in SGA (1.4 ± 0.8 gain; p < 0.001), GHD (1.1 ± 1.0; p < 0.001), ISS (1.1 ± 0.8; p < 0.001) and SHOX (0.8 ± 0.7; p = 0.007) patients. In TS, a non-statistically significant improvement was observed (0.7 ± 0.8; p = 0.085). In GHD, onset before 3 years showed a gain of 1.9 ± 1.1, vs 0.7 ± 0.6 (p = 0.083) and in ISS onset with bone age less than 9 years increased it by 1.7 ± 0.5 vs 0.5 ± 0.5 (p < 0.001). 27/73 (37%) headache, 18/73 (24%) lower extremity pain, 1/73 (1.5%) dizziness, 1/73 (1.5%) scoliosis, 1/73 (1.5%) epiphysiolysis and 1/73 (1.5%) craniopharyngioma recurrence. Children with GHD, ISS, SHOX mutation and SGA significantly improved their height, highlighting in GHD and ISS the importance of early treatment. Treatment was well tolerated in the 5 groups analyzed.

CSIG-29. CLINICAL AND TRANSCRIPTOMIC DATASET IDENTIFIES LARGE CHANGES IN IMMUNE AND HORMONAL SIGNALING AMONGST RECURRENT PATIENTS WITH CRANIOPHARYNGIOMA

Abstract INTRODUCTION Craniopharyngioma is a low-grade suprasellar brain tumor that often produces deficiencies of pituitary hormones, including growth hormone. Although epidemiological data are reassuring that growth hormone does not lead to craniopharyngioma recurrence, the theoretical risk is cause for concern. To address this and other questions related to the care of individuals with recurrent craniopharyngioma, we analyzed gene expression in craniopharyngiomas in the context of clinical data. We envision insights that could guide decisions about hormone replacement and tumor-focused treatment alternatives. METHODS Clinical information was abstracted for a cohort of individuals diagnosed with craniopharyngioma at the Children’s Hospital of Philadelphia between 2016 - 2020. In parallel, tumor transcriptomic data for patients with craniopharyngioma were collected from the Children’s Brain Tumor Network (CBTN) and matched to clinical data where applicable. Bulk RNA-sequencing analysis, pathway analysis, and immune deconvolution (xCell) of samples was performed in R to identify transcript-level changes that distinguish recurrent from initial craniopharyngioma. RESULTS CBTN data was available for 54 initial tumor samples and 17 recurrent tumor samples, with matched clinical data available for 33 samples (26 initial, 7 recurrent). We did not find differences in clinical characteristics between individuals with versus without recurrence. Pathway analysis notably suggested altered levels of antigen presentation (NES: 2.97), Programmed cell death (NES: 2.34), WNT signaling (NES: 1.97), and Growth Hormone Signaling (NES: -1.277) amongst recurrent samples when compared against initial samples. Deconvolution of samples showed altered levels of M2-like macrophages (39.2% increased) and CD4+ T-helper-2 cells (-54.7% decreased) in recurrent samples compared against initial samples. Preliminary work is being completed to frame these results in the context of available clinical neuroendocrine data. CONCLUSION This work highlights marked changes in immune and hormonal signaling in samples from recurrent craniopharyngioma tumors. These insights have potential therapeutic implications that deserve further investigation.

The influence of tumor topography on the surgical outcome of craniopharyngiomas.

Various topographical classifications for craniopharyngioma have been proposed based on their relationship with optic chiasm and the third ventricular floor. There is a paucity of literature evaluating the surgical outcome based on tumor topography. This study aims to compare the surgical outcomes of retrochiasmatic craniopharyngiomas (RCPs) and nonretrochiasmatic craniopharyngiomas (non-RCPs). This retrospective study includes newly diagnosed patients with craniopharyngioma who underwent surgery between January 2000 and December 2015. Clinical features, the extent of resection (EOR), surgical outcomes, tumor recurrence, and progression-free survival (PFS) of craniopharyngiomas were compared with respect to their relationship to the optic chiasm and third ventricular floor. The authors identified RCPs in 104 and non-RCPs in 33 patients. RCPs were significantly larger and more associated with hydrocephalus than were non-RCPs (p < 0.001) at the time of diagnosis. Puget grade 2 hypothalamic involvement was more frequent with RCPs. EOR and PFS following either subtotal resection (p = 0.07) or gross-total resection (p = 0.7) were comparable between RCPs and non-RCPs. There was no significant difference in the postoperative visual outcome. Resection of RCPs resulted in higher postoperative hypopituitarism (64% vs 42%, p = 0.01) and hypothalamic dysfunction (18% vs 3%, p = 0.02). Location of the tumor, either retrochiasmatic (HR 0.5; 95% CI 0.14-2.2; p = 0.4) or nonretrochiasmatic (HR 1.3; 95% CI 0.3-5.5; p = 0.6), did not show association with recurrence. RCPs with extra- and intraventricular components (type 3b) had a higher incidence of postoperative hypothalamic morbidities (p = 0.01) and tumor recurrence (36% vs 19%; p = 0.05) during follow-up than the extraventricular (type 3a) RCP. Between prechiasmatic and infrachiasmatic/intrasellar craniopharyngiomas, EOR (p = 0.7), postoperative diabetes insipidus (p = 0.4), endocrinological outcome (p = 0.7), and recurrence (p = 0.1) were comparable. The patients with complex multicompartmental tumors had a lower rate of gross-total resection (25%, p = 0.02) and a higher incidence of tumor recurrence (75%, p = 0.004) than the rest. The tumor topography can influence the postoperative outcome. RCPs can be associated with a higher incidence of hypopituitarism and hypothalamic morbidities postoperatively. The influence of topography on EOR and tumor recurrence is controversial. However, this study did not find a significant difference in EOR and tumor recurrence between RCPs and non-RCPs. PFS and overall mortality are also comparable.

THU085 Can Genetic Mutations And Molecular Markers Predict Recurrence In Adamantinomatous Or Papillary Craniopharyngiomas? A Systemic Review.

Abstract Disclosure: M. Nicholas: None. U. Mahfuza: None. A. De Ieva: None. M. Rodriguez: None. V.A. Preda: None. Craniopharyngiomas are rare, but clinically significant intracranial tumors. According to the WHO criteria, two clinicopathological subtypes, adamantinomatous (aCP) and papillary (pCP) (WHO 2021) are distinct entities[1]. They may have a poor prognosis due to their high recurrence rate and anatomical location. The association between surgical procedures and recurrence is well reported, however, a definitive predictive factor for recurrence based on tumor molecular markers has yet to be elucidated. The genetic mutations BRAF V600E and CTNNB1 are mutually exclusive to aCP and pCP respectively and their presence has been well documented (WHO). The expression of molecular markers ki67, p53, VEGF, PD-L1, pATM, RARγ, RARβ, CD166, CD133, E-Cadherins, FABP5, CRAPBII, Vimentins, CXCL12, CXCR4, MCM6 and DNA Topo II alpha have also all been found in populations of craniopharyngiomas, but the level of their expression and proliferation indices have not previously been clearly summarized in the literature as markers of recurrence. Aim: To perform a systematic review of the common genetic and molecular markers found in either adamantinomatous or papillary craniopharyngiomas and assessment of their impact on recurrence rate. Methods: MEDLINE, Embase, PubMed, Cochrane Library and Scopus electronic were searched in December 2022 using a search string with key words “craniopharyngioma”, “adamantinomatous”, “papillary”, “genetic mutation”, “molecular markers” and “recurrence”. The initial search resulted in 1105 studies. These studies were assessed against inclusion and exclusion criteria. Results: The final review included 26 studies that included 905 patients. Ki67, p53, PD-L1, CXCL12, CXCR4, RARγ FABP5, CRABPII, MCM6 and DNA Topo II alpha molecular markers and CTNNB1 and PTTG-1 gene mutations were found to have an association with an increase in recurrence of adamantinomatous craniopharyngiomas, while VEGF, TERTp, pATM, RARβ, CD166, CD133, E-Cadherins and Vimentins were not. Ki67, p53, PD-L1, FABP5, CRABPII, MCM6 and DNA Topo II alpha molecular markers and BRAF and PTTG-1 gene mutations were found to have an association with an increase in recurrence of papillary craniopharyngiomas, while TERTp, pATM were not. The association of other molecular markers and tumour recurrence, despite large scale studies, gave conflicting results. Conclusion: Our systematic review demonstrates that a multitude of molecular markers and genetic mutations are markers of recurrence or worsening clinicopathology. The identification of genetic mutations and expression of molecular markers and their role in the likelihood of recurrence in either adamantinomatous or papillary craniopharyngiomas provides insight into the potential for novel targets in future therapies.

Predictive Factors for Pediatric Craniopharyngioma Recurrence: An Extensive Narrative Review.

Despite being classified as benign tumors, craniopharyngiomas (CPs) are associated with significant morbidity and mortality due to their location, growth pattern, and tendency to recur. Two types can be identified depending on age distribution, morphology, and growth pattern, adamantinomatous and papillary. The adamantinomatous CP is one of the most frequently encountered central nervous system tumors in childhood. Our aim was to review the relevant literature to identify clinical, morphological, and immunohistochemical prognostic factors that have been implicated in childhood-onset CP recurrence. Lack of radical surgical removal of the primary tumor by an experienced neurosurgical team and radiotherapy after a subtotal excision has been proven to significantly increase the recurrence rate of CP. Other risk factors that have been consistently recognized in the literature include younger age at diagnosis (especially <5 years), larger tumor size at presentation, cystic appearance, difficult tumor location, and tight adherence to surrounding structures, as well as the histological presence of whorl-like arrays. In addition, several other risk factors have been studied, albeit with conflicting results, especially in the pediatric population. Identifying risk factors for CP recurrence is of utmost importance for the successful management of these patients in order to ultimately ensure the best prognosis.

Ultrasound-guided endoscopic endonasal resection of sellar and suprasellar craniopharyngiomas.

Optimal planning and minimally invasive surgical approach are essential to complete craniopharyngiomas (CP) resection with limited postoperative morbidity. Given the nature of craniopharyngioma recurrence, complete resection of the neoplasm is crucial. Since CP arise from the pituitary stalk and may grow anteriorly or laterally, some cases require an extended endonasal craniotomy. The extension of the craniotomy is crucial to expose the whole tumor and to make its dissection from the surrounding structures feasible. In order to guide the extension of the approach, the intraoperative use of ultrasound is helpful for the surgeons. The objective of this paper is to describe and to demonstrate the applicability of the utilization of intraoperative ultrasound (US) guidance for planning and confirmation of craniopharyngioma resection in EES. The authors selected one operative video of a sellar-suprassellar craniopharyngioma gross-totally resected by EES. The authors demonstrate the extended sellar craniotomy, the anatomic landmarks that guide bone drilling and dural opening, the aspect of the intraoperative real time US, tumor resection and dissection from the surrounding structures. The solid component of the tumor was mostly isoechogenic in texture compared to the anterior pituitary gland, with several wide spread hyperechogenic images corresponding to calcifications and hypoechogenic vesicles corresponding to cysts inside the CF ("salt-and-pepper" pattern). The intraoperative endonasal US is a new surgical tool that allows for real-time active imaging for skull base procedures, such as sellar region tumors. Besides tumor evaluation, the intraoperative US helps the neurosurgeon to determine the size of craniotomy, to anticipate the relation between the tumor and vascular structures and to guide the best strategy for gross-total resection of the tumor. The EES allows a straight access to the craniopharyngiomas located in the sellar region or that grow anteriorly or superiorly. This approach allows the surgeon to dissect the tumor with minimal manipulation of the surrounding structures, when compared to craniotomy approaches. In order to accomplish that, the use of intraoperative endonasal ultrasound helps the neurosurgeon to perform the most suitable strategy, optimizing the rate of success.

Safety and Efficacy of Pediatric Growth Hormone Therapy: Results From the Full KIGS Cohort.

The Kabi/Pfizer International Growth Database (KIGS) is a large, international database (1987-2012) of children treated with recombinant human growth hormone (rhGH) in real-world settings. This work aimed to evaluate the safety and efficacy of rhGH from the full KIGS cohort. Data were collected by investigators from children with growth disorders treated with rhGH (Genotropin [somatropin]; Pfizer). Safety was evaluated in all treated patients, and efficacy in those treated for 1 year or more. A subgroup included patients treated for 5 years or more (≥ 2 years prepubertal) who had reached near-adult height (NAH). Main outcomes included adverse events (AEs), serious AEs (SAEs), and height growth. The full KIGS cohort (N = 83 803 [58% male]) was treated for idiopathic GH deficiency (IGHD; 46.9%), organic GHD (10.0%), small for gestational age (SGA; 9.5%), Turner syndrome (TS; 9.2%), idiopathic short stature (ISS; 8.2%), and others (16.2%). Median rhGH treatment duration was 2.7 years and observation 3.1 years. SAEs occurred in 3.7% of patients and death in 0.4%. The most common SAEs were recurrence of craniopharyngioma (n = 151), neoplasm (n = 99), and cancer (n = 91); and scoliosis (n = 91). Median first-year delta height-SD score (SDS) (Prader) in prepubertal patients was 0.66 (IGHD), 0.55 (ISS), 0.58 (TS), and 0.71 (SGA). Median gains in NAH-SDS were 1.79 (IGHD), 1.37 (ISS), and 1.34 (SGA) for boys, and 2.07 (IGHD), 1.62 (ISS), 1.07 (TS), and 1.57 (SGA) for girls. Data from KIGS, the largest and longest running international database of rhGH-treated children, show that rhGH is safe and increases short-term height gain and adult height across GHD and non-GHD conditions.

RARE-13. Clinical management and functional and survival outcomes in pediatric craniopharyngioma, a patient and family perspective

Craniopharyngiomas are rare, histologically benign, sellar/parasellar tumors with significant tumor and therapy related morbidity and impairment in quality of life (QOL). We report survey results from patients/families affected by childhood-onset craniopharyngioma to identify opportunities for improvement in management. An anonymous REDCap survey was distributed via social media and clinic visits to patients/families of craniopharyngioma survivors. Survey questions investigated perspectives on clinical management and functional and survival outcomes at initial diagnosis and recurrence. A total of 159 patients/families completed the survey, 40% (n=64) reported craniopharyngioma recurrence. For primary craniopharyngioma, maximal safe resection was the most frequent treatment reported (n=84), followed by partial resection (n=40), radiation (n=8), biopsy (n=5), and chemotherapy (n=3). Most patients (n=120) decided on a treatment plan within one week, 63 (40%) decided in one day. For recurrent craniopharyngioma, maximal safe resection and radiation were the most frequent interventions (n=33 each), followed by partial resection (n=13), chemotherapy (n=4) and biopsy (n=2). Multiple treatment options and/or participation in a clinical trial were offered to similar numbers of patients across primary and recurrent diagnoses (~21% for each). Most recurrent craniopharyngioma patients decided on management within one week (n=43). Long term effects related to tumor and treatment were identified as the primary concern in all respondents. The most common deficits for all patients were neuro-endocrine followed by vision and neurocognition problems. Neuro-endocrine complications were self-reported as the biggest impact on QOL. Families reported that they would prefer treatment options with the potential for improved QOL, even if these options also carried an increased risk of recurrence. Craniopharyngioma continues to be predominantly treated with surgery and radiation initially and with recurrence. Survivors have multiple comorbidities, with an interest in targeted therapies that preserve QOL. Novel therapies to prevent co-morbidities and provide long term benefits are necessary and upcoming.

Comparison of Outcomes following Primary and Repeat Resection of Craniopharyngioma.

Introduction The management of recurrent craniopharyngioma is complex with limited data to guide decision-making. Some reports suggest reoperation should be avoided due to an increased complication profile, while others have demonstrated that safe reoperation can be performed. For other types of skull base lesions, maximal safe resection followed by adjuvant therapy has replaced radical gross total resection due to the favorable morbidity profiles. Methods Seventy-one patients underwent resection over a 9-year period for craniopharyngioma and were retrospectively reviewed. Patients were separated into primary resection and reoperation cohorts and stratified by surgical approach (endonasal vs. cranial) and survival analyses were performed based on cohort and surgical approach. Results Fifty patients underwent primary resection, while 21 underwent reoperation for recurrence. Fifty endonasal transsphenoidal surgeries and 21 craniotomies were performed. Surgical approaches were similarly distributed across cohorts. Subtotal resection was achieved in 83% of all cases. There were no differences in extent of resection, visual outcomes, subsequent neuroendocrine function, and complications across cohorts and surgical approaches. The median time to recurrence was 87 months overall, and there were no differences by cohort and approach. The 5-year survival rate was 81.1% after reoperation versus 93.2% after primary resection. Conclusion Compared with primary resection, reoperation for craniopharyngioma recurrence is associated with similar functional and survival outcomes in light of individualized surgical approaches. Maximal safe resection followed by adjuvant radiotherapy for residual tumor likely preserves vision and endocrine function without sacrificing overall patient survival.

Management strategies for recurrent pediatric craniopharyngioma: new recommendations.

The goal of this study was to identify the independent risk factors for recurrence or progression of pediatric craniopharyngioma and to establish predictors of the appropriate timing of intervention and best management strategy in the setting of recurrence/progression, with the aim of optimizing tumor control. This is a retrospective cohort study of all pediatric patients with craniopharyngioma who were diagnosed and treated at Boston Children's Hospital between 1990 and 2017. This study was approved by the institutional review board at Boston Children's Hospital. All statistical analyses were performed using Stata software. Eighty patients (43 males and 37 females) fulfilled the inclusion criteria. The mean age at the time of diagnosis was 8.6 ± 4.4 years (range 1.2-19.7 years). The mean follow-up was 10.9 ± 6.5 years (range 1.3-24.6 years). Overall, 30/80 (37.5%) patients developed recurrence/progression. The median latency to recurrence/progression was 12.75 months (range 3-108 months). Subtotal resection with no adjuvant radiotherapy (p < 0.001) and fine calcifications (p = 0.008) are independent risk factors for recurrence/progression. An increase (%) in the maximum dimension of the tumor at the time of recurrence/progression was considered a statistically significant predictor of the appropriate timing of intervention. Based on the identified independent risk factors for tumor recurrence/progression and the predictors of appropriate timing of intervention in the setting of recurrence/progression, the authors propose an algorithm for optimal management of recurrent pediatric craniopharyngioma to increase the likelihood of tumor control.

Functional Neurologic and Surgical Outcomes Among Patients Undergoing Reoperation for Craniopharyngioma Recurrence

Aabedi, Alexander A BS; Young, Jacob S MD; Phelps, Ryan R.L BA; Winkler, Ethan A MD, PhD; Theodosopoulos, Philip V MD Author Information

Characteristics of ectopic recurrence of craniopharyngioma: Case report and literature review

Craniopharyngiomas makes up between 5 and 15% of brain tumors in children and 1% in adults. While these lesions are considered to be benign, they are characteristically infiltrative and have a high rate of local recurrence. Although rare, ectopic recurrences have been documented in case reports with a total of 61 cases available. Here we analyze the available literature for trends in patients with ectopic recurrence as well as present a case of an ectopic recurrence which was resected 11 years after its initial diagnosis. Ectopic recurrence is thought to occur due to seeding along the surgical tract or spread along the CSF pathway. These lesions occur at a later time point after initial treatment compared to local recurrences. All pediatric cases were of adamantinomatous histology, while 27% of adult cases showed papillary histology, matching the distribution of general adult craniopharyngioma. Half of all cases recurred in the frontal lobes with an even distribution in the rest of the cited locations, likely reflecting the surgical approach. We found a significantly longer time period to ectopic recurrence in patients who underwent radiotherapy for subtotal tumor resection compared to those who either had a gross total resection or subtotal resection with no further treatment. Further, the Ki-67 labeling indices, which can serve as a marker of the proliferative potential of tumors was lower in the ectopic recurrences when compared to available data on local recurrences, possibly explaining the longer time between initial diagnosis and ectopic recurrence. In some of the cases, local recurrence preceded ectopic recurrence and we looked at how and if the local recurrences are different from those reported in the literature, which we did not find to be significantly different. In light of longer time to recurrence in these cases, prolonged follow-up is warranted in patients post craniopharyngioma resection.

Malignant Craniopharyngioma: A Report of Seven Cases and Review of the Literature

Malignant craniopharyngioma is a rare clinical entity; most patients with this disease show transformation from an initially benign craniopharyngioma. The rare prevalence of the disease, nonspecific presenting symptoms, and imaging features that overlap with benign craniopharyngiomas make preoperative identification challenging. In this study, we retrospectively evaluated the data of 7 patients diagnosed with malignant craniopharyngioma within a 5-year period in a single center. A thorough review of patient records from Beijing Tiantan Hospital, Capital Medical University, between 2013 and 2018 was performed. After identifying these 7 patients with malignant craniopharyngioma, we evaluated their demographic and clinical characteristics, including symptoms at presentation, imaging characteristics, treatment, and outcomes. Five patients showed transformation from benign craniopharyngiomas, and 2 had de novo malignancy. Two patients were children at diagnosis of malignancy. Patients' average age was 22 years. Headache was the most common presenting symptom, followed by visual field defects and adenohypophyseal dysfunction. Imaging characteristics showed that all patients had giant mass in the sellar, suprasellar, and/or parasellar regions that had invaded surrounding anatomic structures. Of the 5 patients who showed transformation from benign craniopharyngioma, 3 had undergone radiologic treatment before the diagnosis of malignant transformation. Malignant craniopharyngioma should not be overlooked in patients with a giant sellar mass, especially in young patients who have experienced multiple recurrences of craniopharyngioma and patients who received radiologic therapy.

A novel clinical risk score that accurately predicts recurrence of craniopharyngioma - a multicentre cohort study

A novel clinical risk score that accurately predicts recurrence of craniopharyngioma - a multicentre cohort study

Recurrence Rate and Prognostic Factors for the Adult Craniopharyngiomas in Long-Term Follow-Up

Craniopharyngiomas show a high recurrence rate despite their pathologically benign nature. Thus, we analyzed the clinical features to elucidate the prognostic factors for the recurrence of craniopharyngiomas in adults with long-term follow-up. This retrospective study reviewed and analyzed the preoperative features, surgical results, and tumor recurrence of patients who underwent an operation at a single institution from 2004 to2013. This study analyzed the results of 64 consecutive adult patients, and the median follow-up period was 83.5 months (range 9-163 months). Ten patients had a history of surgery, whereas 4 had a history of adjuvant radiation. Retrochiasmatic tumors (n= 51, 79.7%) were more common than prechiasmatic tumors. Operations were performed via the transcranial approach in 31 (48.4%) patients and transsphenoidal approach in 33. Gross total removal was achieved in 44 (68.8%) patients, and the transsphenoidal approach showed a greater gross total removal rate than the transcranial approach did (97.0% vs. 38.7%, P < 0.001). Adjuvant radiotherapy was performed in 8 patients, and radiosurgery was performed in 2. Recurrence was identified in 25 (39.1%) patients in 45.0 months of the median time to recurrence. The overall actuarial 5- and 7-year progression-free survival rates were 71.8% and 63.6%, respectively. Multivariate analysis revealed that supra- and subdiaphragmatic tumor locations and subtotal removal were risk factors for long-term tumor recurrence. The long-term recurrence rate of craniopharyngiomas was high; therefore, long-term regular follow-up is mandatory. Adjuvant irradiation for remnant tumors showed a long-term tumor control rate comparable to that of completely removed tumors.

Excision of pediatric craniopharyngioma: pattern of recurrence in 35 patients at a tertiary care hospital in Saudi Arabia.

Craniopharyngiomas are benign tumors of central nervous system which are known to affect both adults and children. Despite their benign origin, the recurrence is still one of the main postoperative challenges. The aim of this study was to investigate in retrospect factors related to recurrence of craniopharyngioma in a tertiary center in Riyadh, Saudi Arabia. We conducted a review of charts of all craniopharyngioma patients operated in neurosurgery department at King Faisal Specialist Hospital & Research Center in Riyadh (KFSH-RC). Age at surgery, gender, body mass index, symptoms at presentation, hormonal data, tumor characteristics and location, presence of hydrocephalus, previous treatments, neuroimaging features, surgical results, and recurrence were abstracted from the medical charts of the patients retrospectively. In all, 70.6% of patients had gross total resection (GTR). The recurrence after GTR in our series was 25% which considered low when compared to most surgical series. From all above studied variables, VP shunt insertion at presentation was constantly significant in both uni- and multi-variable analysis. In this study, we analyzed several factors to determine if they had any significant correlation with recurrence. Only VP shunt insertion was found significant. Further researches are needed to verify these factors and to discover others.

Clinical, Pathological and Surgical Risk Factors Associated with Craniopharyngioma Recurrence: A Literature Review

Objective: This review article attempts to examine and provide an overview of the risk factors associated with craniopharyngioma recurrence. Methods: A literature review of articles relating to the recurrences of craniopharyngioma and the clinical, molecular prognostic indicators of recurrence and treatment outcomes was performed retrospectively. Results: A total of 107 studies which described specific risk factors related to craniopharyngioma recurrence were identified which included but not limited to 54 retrospective case series, 7 systematic reviews, 21 laboratory reports, 13 case reports and 12 literature reviews. Conclusion: Based on the evidence identified in this review, the risk factors for recurrence in craniopharyngioma management are interrelated in a complex way, and surgery with or without adjuvant radiotherapy is reported to be of long-term benefit, but a disparity in findings suggests no definitive consensus on the risk factors of craniopharyngioma recurrence. More high-quality research is needed.