Tumor epitelial benigno, parcialmente cístico, da região selar, presumivelmente derivado do epitélio da bolsa de Rathke. Afeta principalmente crianças e adultos jovens. Existem duas formas clinicopatológicas: craniofaringioma adamantinomatoso e craniofaringioma papilar. O fator mais significativo associado à recorrência é a extensão da ressecção cirúrgica, com lesões maiores que 5 cm de diâmetro apresentando um prognóstico marcadamente pior. (Adaptado da OMS)
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Tumor epitelial benigno, parcialmente cístico, da região selar, presumivelmente derivado do epitélio da bolsa de Rathke. Afeta principalmente crianças e adultos jovens. Existem duas formas clinicopatológicas: craniofaringioma adamantinomatoso e craniofaringioma papilar. O fator mais significativo associado à recorrência é a extensão da ressecção cirúrgica, com lesões maiores que 5 cm de diâmetro apresentando um prognóstico marcadamente pior. (Adaptado da OMS)
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 11 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 46 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
Key downstream component of the canonical Wnt signaling pathway (PubMed:17524503, PubMed:18077326, PubMed:18086858, PubMed:18957423, PubMed:21262353, PubMed:22155184, PubMed:22647378, PubMed:22699938). In the absence of Wnt, forms a complex with AXIN1, AXIN2, APC, CSNK1A1 and GSK3B that promotes phosphorylation on N-terminal Ser and Thr residues and ubiquitination of CTNNB1 via BTRC and its subsequent degradation by the proteasome (PubMed:17524503, PubMed:18077326, PubMed:18086858, PubMed:189574
CytoplasmNucleusCytoplasm, cytoskeletonCell junction, adherens junctionCell junctionCell membraneCytoplasm, cytoskeleton, microtubule organizing center, centrosomeCytoplasm, cytoskeleton, spindle poleSynapseCytoplasm, cytoskeleton, cilium basal body
Colorectal cancer
A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history.
Protein kinase involved in the transduction of mitogenic signals from the cell membrane to the nucleus (Probable). Phosphorylates MAP2K1, and thereby activates the MAP kinase signal transduction pathway (PubMed:21441910, PubMed:29433126). Phosphorylates PFKFB2 (PubMed:36402789). May play a role in the postsynaptic responses of hippocampal neurons (PubMed:1508179)
NucleusCytoplasmCell membrane
Medicamentos aprovados (FDA)
1 medicamento encontrado nos registros da FDA americana.
Variantes genéticas (ClinVar)
702 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 18 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
46 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
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Remédios, cuidados de apoio e o que precisa acompanhar
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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119 ensaios clínicos encontrados, 20 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 2.156
Diagnostic yield from minimally invasive pediatric brain tumor biopsies in the molecular era.
This study aimed to evaluate the diagnostic yield from minimally invasive biopsies of pediatric brain tumors using endoscopic, robot-guided, and optical image-guided techniques. A single-center retrospective review was conducted on the data of children (< 18 years of age) who underwent minimally invasive brain tumor biopsies using optical image-guided, endoscopic, or robot-assisted needle biopsies. Data collected consisted of demographic details, tumor location, simultaneous CSF diversion, and macroscopic details of collected specimens. Primary outcomes were histological and molecular diagnostic yield, histological accuracy, need for repeat biopsy, and reasons for biopsy failure. Adverse events were prospectively recorded. Statistical analysis was conducted in SPSS and Prism using Pearson's chi-square and Fisher's exact tests. Between January 2004 and February 2023, 188 biopsies were performed using endoscopic (41.5%), optical image-guided (37.2%), and robot-guided (21.3%) techniques. Tumor location was classified as suprasellar (28.2%), pineal region (20.2%), brainstem (19.1%), basal ganglia/thalamic (16.5%), supratentorial lobar (10.1%), intraventricular (5.3%), and cerebellar (0.5%). Histological diagnosis was achieved in 88.3% (166/188) and consisted of low-grade glioma (33.5%), high-grade glioma (31.9%), germ cell tumor (10.6%), embryonal tumor (5.3%), craniopharyngioma (2.1%), lymphoma (1.6%), pineoblastoma (1.6%), choroid plexus papilloma (0.5%), meningioma (0.5%), and pineal cyst (0.5%). Overall, 11.7% (22/188) of biopsies were nondiagnostic: 19.2% for endoscopic, 8.6% for optical image-guided, and 2.5% for robot-guided techniques. Thirteen (6.9%) patients required further biopsy. A molecular diagnosis was achieved in 82.0% (109/133) of eligible cases. All tumors with a molecular diagnosis had a histological diagnosis; of 24 patients for whom molecular diagnosis was not possible, 79.2% (19/24) had a histological diagnosis. Larger-sized biopsies were more likely to result in a positive diagnosis for histology and molecular analysis. Hemorrhage in the specimen did not affect results. Endoscopic biopsies were most likely to be small and yield a lower volume of core specimens. Histological diagnostic accuracy for glioma was more likely for high-grade tumors, larger-sized biopsies, and tumors in which a molecular diagnosis was achieved. Through the use of contemporary minimally invasive techniques, pediatric brain tumor biopsy has a histological diagnostic yield of 88.3% and molecular diagnostic yield of 82.0%. Robot-guided and optical image-guided techniques provided the highest diagnostic yield (97.5% and 91.4%, respectively, for histological analysis), while endoscopic biopsies had the highest rate of nondiagnostic results (19.2%), likely due to smaller specimen volumes.
Surgical outcomes and longitudinal quality of life after endoscopic endonasal resection of craniopharyngioma.
Craniopharyngiomas present a complex challenge for the neurosurgeon. The imperative is finding the balance between optimizing the extent of resection, decompressing critical structures and limiting the morbidity to the patient. The endoscopic endonasal approach is increasingly utilised for the management of craniopharyngioma, offering improved access to the suprasellar region while minimizing morbidity to the optic apparatus and neurovascular structures. Data regarding post operative quality of life outcomes remains limited in the literature. This mutli-centered prospective study evaluated surgical morbidity and patient reported outcomes measures following endoscopic endonasal resection within the Australian context. Patients undergoing endoscopic endonasal resection for craniopharyngioma at three tertiary skull base units in Melbourne, Australia were prospectively enrolled between 2016 and 2024. Pre-operative and serial post operative assessments included gross total resection, visual outcomes, endocrine outcomes and quality of life measures. Quality of life measures were reported using the Sinonasal Outcome Test (SNOT-22) and Anterior Skull Base Questionnaire (ASBQ-35). Patients' questionnaires were collected at baseline and sequentially in the first 12 months post operatively. A total of 32 patients underwent endoscopic endonasal resection of craniopharyngioma over the study period. Sino-nasal specific quality of life initially worsened following surgery. As expected, day 1 SNOT-22 scores were significantly worse compared to baseline (mean difference -23.8 (95%CI: -41.4 to -6.17, p = 0.004). Based on the minimum clinically important difference (MCID) of 8.9, SNOT-22 scores remained worse at day 1, 3 and 7 before falling below MCID at week 3. This trend continued out to 6 and 12 months post-operatively with further reduction in mean difference to near zero at 12 months post-operatively (mean difference -0.1 (95%CI: -20.6 to 20.3). In the nasal subdomain, there was a significant increase in symptoms at Day 1 (p = 0.034) and 3 (p = 0.001) while no significant difference was found beginning at day 7 (p = 0.099). Overall quality of life, as measured by the ASBQ-35, was worse at 6 weeks post-operatively compared to baseline (107.0 ± 20.5 v 119.3 ± 18.5; mean change 12.1 (95%CI: 0.5-23.7, p = 0.042). This had returned to normal by 3 months and was maintained at 12 months post-operatively. The endoscopic endonasal is a safe technique with good tumour control and acceptable visual and endocrine outcomes. Post-operative quality of life based on SNOT-22 and ASBQ appears to expediently return to baseline for patients with no evidence of long-term QOL detriment. It is the preferred technique in appropriately selected cases amongst our surgeons.
Ventriculo-gallbladder shunt reflux: A case study and review of the literature.
Ventriculo-gallbladder (VGB) shunt represents an uncommon yet well-documented surgical intervention for the treatment of hydrocephalus through cerebrospinal fluid (CSF) diversion into the gastrointestinal tract through the gallbladder. When other ventricular shunt types have failed or are contraindicated, VGB shunts are effective. In exceptionally rare circumstances, biliary reflux can occur and cause accumulation of bile in the ventricular system. The authors report a case of bile reflux through a ventricular gallbladder shunt with eventual patient recovery after surgical intervention. Prior reports of VGB shunt complications are discussed. The authors present the case of a 3-year-old girl with a history of craniopharyngioma causing obstructive hydrocephalus. At an outside facility, she underwent ventriculo-peritoneal (VP) shunt placement, which failed due to abdominal pseudocyst after 6 months. The patient then underwent a subtotal resection of her tumor and placement of a right VGB shunt system with a subdural and ventricular catheter connected distally to a Certas valve. Her postoperative course was complicated by the left-sided tonic-clonic seizures and hyponatremia. Three months later, the patient presented to our emergency department with persistent hyponatremia, encephalopathy, and seizure activity in the setting of COVID-19. Imaging demonstrated a right hypodense subdural fluid collection. Right frontal craniotomy demonstrated accumulation of bile in the subdural space secondary to retrograde flow from the gallbladder, which was evacuated. After externalized subdural drainage evolved from biliary fluid to normal CSF, the externalized ventricular shunt and subdural drain were replaced with a ventriculo-atrial shunt. The patient gradually improved with each follow-up visit over the course of several years. This case demonstrates a potentially life-threatening complication of VGB shunt in the form of biliary reflux into the subdural space. Valveless VGB shunts should be avoided to prevent this rare but potentially fatal complication.
Intraoperative MRI for endoscopic endonasal transsphenoidal surgery in children.
The use of intraoperative MRI (iMRI) has been well described for endoscopic endonasal transsphenoidal surgery (EETS) in adults. However, literature on its use for EETS in pediatric patients is lacking. The objective of this study was to evaluate the usefulness, efficacy, and safety of iMRI for EETS in the pediatric population. A retrospective review of patient medical records and imaging was performed for all patients younger than 18 years of age who underwent EETS at a single institution from January 2005 to December 2024. Based on whether iMRI was used, cases were divided into two groups and compared. Collected data included the preoperative goal of the surgery (biopsy, debulking/decompression, subtotal resection [STR], and gross-total resection [GTR]) and the iMRI timing (predissection, intradissection, and postdissection), purpose, and findings, as well as how these findings impacted intraoperative surgical management and whether the preoperative goal of the surgery was met. Additional demographic and clinical variables were assessed, including age at surgery, surgery duration, hospital length of stay (LOS), whether postoperative MRI was performed during the same admission, repeat surgery, histopathological diagnosis, complications, long-term tumor progression/recurrence, and use of adjuvant therapy. Twenty-two EETS procedures were performed among 21 patients with sellar/suprasellar pathologies; iMRI was used in 13 of 22 cases (12 patients, age range 6-17 years) including 4 predissection, 8 pre- and intradissection, and 1 postdissection iMRI evaluations. Pathologies included mostly craniopharyngiomas (46.2%) and pituitary adenomas (38.5%). Surgical goals included biopsy (n = 2), debulking/decompression (n = 3), STR (n = 1), and GTR (n = 7) and were achieved in 12 of 13 cases (92.3%). The mean operative duration was 6.1 hours, mean LOS was 2.9 days, and mean follow-up was 4.3 years. The rate of complications was 38.5% (4 pituitary insufficiency/diabetes insipidus [DI] and 1 CSF leak). Two adenomas recurred and required repeat surgery. For the 9 patients (age range 5-17 years) in the non-iMRI EETS group, the most common pathologies were Rathke cleft cyst (33.3%), craniopharyngioma (22.2%), and adenoma (22.2%). The surgical goal was achieved in 66.6%, with no statistically significant difference compared with the iMRI group (p = 0.264). The operative time (mean 5.8 hours), LOS (mean 4.3 days), and complication rate (22.2%) were similar between the non-iMRI and iMRI groups. Three patients in the non-iMRI group (2 with craniopharyngiomas and 1 with a Rathke cyst) required reoperation for recurrence. For pediatric EETS, iMRI showed promise as an effective and safe surgical adjunct to help verify, in the same setting, that the goals of surgery had been met. However, the outcome measures studied herein were not better than those achieved without the use of iMRI.
Secondary narcolepsy and cognitive dysfunction related to craniopharyngioma: a case study.
Craniopharyngiomas, benign tumors of the sellar region, frequently disrupt hypothalamic function, leading to endocrine and neurological sequelae, including sleep disorders. This case highlights the diagnostic and therapeutic challenges of secondary narcolepsy in a 19-year-old woman with craniopharyngioma. The patient presented with excessive daytime sleepiness, prolonged sleep duration, no cataplexy, and cognitive dysfunction following tumor treatment. However, CSF level of orexin/hypocretin was not measured. Diagnosis of secondary narcolepsy was confirmed through overnight polysomnography and multiple sleep latency tests. Combining methylphenidate for wakefulness and donepezil for cognitive support led to symptomatic improvement. This case underscores the necessity of recognizing secondary narcolepsy in patients with hypothalamic dysfunction and tailoring multidisciplinary management strategies to optimize outcomes. The online version contains supplementary material available at 10.1186/s12883-026-04629-7.
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Acta neurochirurgicaChopsticks mononostril approach with sphenoid sinus cranialization and septal mucosa suturing: a new minimally invasive concept and closure technique for endoscopic endonasal approaches.
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CureusEvaluating temozolomide for pediatric adamantinomatous craniopharyngiomas using microspheroid-based drug screening.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryTwo-Week Interval Hypofractionated Stereotactic Radiosurgery for Benign Intracranial Tumors: Volumetric Kinetics and Radiobiological Rationale.
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Radiology case reportsMinimally Invasive Management of Adamantinomatous Intraventricular Craniopharyngiomas: A Two-Case Series.
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Journal of neurosurgeryLong-term outcomes of paediatric craniopharyngiomas: a comparison of two large international series.
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The Journal of clinical endocrinology and metabolismSurgical outcomes and longitudinal quality of life after endoscopic endonasal resection of craniopharyngioma.
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Clinical endocrinologyCorrelation Between Endocrine and Other Clinical Factors with Peripapillary Retinal Nerve Fiber Layer Thickness After Surgical Treatment of Pediatric Craniopharyngioma.
BiomedicinesEndonasal endoscopic surgery for residual or recurrent craniopharyngiomas: A propensity score-matched cohort study.
Neurosurgical reviewContemporary series of transsphenoidal microsurgery in pediatric patients.
Neurosurgical reviewSurgical management of giant craniopharyngiomas: expanded endoscopic endonasal or transcranial approach?
Journal of neurosurgeryEndoscopic endonasal approach for craniopharyngioma resection in 604 cases: tumor classification, treatment strategies, and surgical outcomes.
Journal of neurosurgeryIntraoperative MRI for endoscopic endonasal transsphenoidal surgery in children.
Journal of neurosurgery. PediatricsMalignant Craniopharyngioma With Features Resembling Ghost Cell Odontogenic Carcinoma: A Case Report and Histopathology Literature Review.
Neuropathology : official journal of the Japanese Society of NeuropathologyCurrent Approaches and Therapeutic Strategies for Hypothalamic Syndrome in Patients with Childhood-onset Craniopharyngioma.
Journal of clinical research in pediatric endocrinologySecondary narcolepsy and cognitive dysfunction related to craniopharyngioma: a case study.
BMC neurologyEndoscopic repair of large dural defects in transsphenoidal surgery by suturing acellular dermal matrix graft with the dura: a technical note.
Acta neurochirurgicaGiant Craniopharyngioma Complicated by Cerebrospinal Fluid Ascites in a 7-Year-Old Boy: A Case Report and Review of Literature.
Pakistan journal of medical sciencesClinical Management Considerations for Craniopharyngioma in Geriatric Populations: An Assessment of the Surveillance, Epidemiology, and End Results Database.
Journal of clinical medicineEpidemiologic characteristics and postoperative complication analysis of patients with craniopharyngioma in China.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyGanglion cell complex and retinal nerve fiber layer thickness in predicting visual outcome in brain tumors after surgery.
World journal of experimental medicineBone flap osteomyelitis following craniotomy in children: A 20-year audit.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryMalignant craniopharyngiomas: Institutional experience and literature review.
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Journal of endocrinological investigationMultiomics reveals the mechanism of KNG1 in promoting tumor progression in adamantinomatous craniopharyngioma.
Journal of neuro-oncologySpatially Resolved Multiomics Reveals Metabolic Remodeling and Autophagy Activation in Adamantinomatous Craniopharyngiomas.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Trapped in Silence: Severe Catatonia From Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis in a Neurofibromatosis Type 1 Patient With Craniopharyngioma.
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Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryA Causal Relationship Between the Lipidome and Central Nervous System Tumors.
World neurosurgeryEarly Experience of Endoscopic Endonasal Transphenoidal Surgery in the Democratic Republic of Congo: Expanding Access to Skull Base Oncology.
CureusTwo-stage surgery for cystic craniopharyngiomas: a purely endoscopic strategy and outcomes.
PituitaryAcute postoperative parotitis following frontal interhemispheric craniotomy: A case report.
Surgical neurology internationalRadiological Differentiation of Pituitary Adenomas From Other Sellar Masses: A Systematic Review.
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Current oncology reportsEfficacy and safety of BRAF-MEK inhibitors in treatment of Chinese patients with papillary craniopharyngioma: A retrospective cohort study.
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Hormones (Athens, Greece)Critical shift phase in deep vein thrombosis development after major sellar region tumor surgeries: a systematic review.
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Journal of neurological surgery reportsComparative analysis of the bone age of wrist bones in Chinese children with different causes of short stature and central precocious puberty.
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Translational pediatrics[Endocrine Complications and Their Management Following Treatment of Pediatric Brain Tumors].
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Neurosurgical reviewPediatric Oncology Patients With Germline Pathogenic Variants in Adult-Onset Cancer Predisposition Genes.
JCO precision oncologyTrends of hospital-based reporting of intracranial neoplasms in Nigeria from 1960 to 2024: A systematic review and pooled analysis of literatures.
Neuro-oncology advancesMapping the evolution of pediatric craniopharyngioma research: a bibliometric analysis.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryThe interdisciplinary management of craniopharyngioma - practice patterns, outcomes, and insights.
BMC cancerEnhancing Slice-Wise Brain MRI Tasks using Self-Supervised and Auxiliary Learning.
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Discover oncologySurgical Approaches to the Hypothalamus: A Systematic Review of Anatomical Corridors and Postoperative Outcomes.
World neurosurgeryTumors of the Sellar and Suprasellar Regions.
Neuroimaging clinics of North AmericaRadiotherapy of benign intracranial tumours: 2025 update.
Cancer radiotherapie : journal de la Societe francaise de radiotherapie oncologiqueExtended endoscopic transtuberculum approach for purely supradiaphragmatic pediatric craniopharyngiomas: outcomes from a single-center cohort study.
Neurosurgical reviewCraniopharyngioma in children. An age-based analysis on our 11 year experience with the endoscopic endonasal approach.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryComparative analysis of the outcomes of craniotomy and endoscopic resection for recurrent craniopharyngioma: a single institution study.
BMC cancerNovel autonomic dysregulation score in children with hypothalamic syndrome.
Endocrine connectionsA Patient-Derived Organoid Biobank of Adamantinomatous Craniopharyngioma as a Platform for Drug Discovery.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Neuro-ophthalmic clues affecting the surgical approach to a brain tumor: the case of a craniopharyngioma masquerader.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryStereotactic radiosurgery for craniopharyngioma: a systematic review and meta-analysis.
Neurosurgical reviewEpidemiology of acquired hypothalamic obesity following traumatic brain injury and nonspecific hypothalamic microinjury: A nationwide German claims data analysis.
Journal of neuroendocrinologyDiagnostic criteria for acquired hypothalamic obesity - international expert guidance document.
Endocrine journalCraniopharyngioma - What's next.
PituitaryCoexistence Tumor of Craniopharyngioma and Pituitary Neuroendocrine Tumor: A Case Report and Literature Review.
CureusEfficacy and safety of BRAF-MEK dual inhibition in BRAF-V600E mutated papillary craniopharyngioma: a systematic review.
Journal of neuro-oncologyInterrogation of the cellular hierarchies reveals neoplastic evolution and therapeutic vulnerability in craniopharyngioma.
Neuro-oncologySleep Disorders, Dysregulation of Circadian Rhythms, and Fatigue After Craniopharyngioma-A Narrative Review.
BiomedicinesEffectiveness and safety of GLP-1 receptor agonists in craniopharyngioma patients with obesity: A multicentre real-world study.
Diabetes, obesity & metabolismThe Craniopharyngioma Quality of Life (C-QOL) Index: a preliminary disease-specific, patient-centered tool for assessing patient reported outcomes in adult craniopharyngioma patients.
Journal of neuro-oncologyNasoseptal Flap Reuse for Reconstruction in Revision Endoscopic Skull Base Surgery.
Journal of neurological surgery. Part B, Skull baseThe Incidence of Calcified Pituitary Macroadenomas: Challenging the Craniopharyngioma Assumption.
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Oxford medical case reportsArtificial Intelligence in Pituitary Disease.
Endocrinology and metabolism clinics of North AmericaPathogenesis of Craniopharyngiomas and Targeted Approaches to Therapy.
Endocrinology and metabolism clinics of North AmericaA rare case of diffuse myelitis following intracystic bleomycin chemotherapy for craniopharyngioma.
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Best practice & research. Clinical endocrinology & metabolismMortality in craniopharyngiomas: Data from the last two decades.
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Georgian medical newsPostoperative weight gain following endoscopic endonasal surgery for craniopharyngiomas in children and adults: A retrospective cohort study.
PituitaryReturn to work and factors influencing recovery after endoscopic transsphenoidal surgery for hypothalamic and pituitary tumors.
Scientific reportsEctopic craniopharyngiomas.
Best practice & research. Clinical endocrinology & metabolismThe effect of growth hormone replacement therapy on craniopharyngioma recurrence: a systematic review and meta-analysis.
PituitaryHypothalamus-sparing surgery in craniopharyngiomas: Oncological and hypothalamic long-term outcomes.
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Surgical neurology internationalInsertion of a ventriculo-biliary shunt in a 3-year-old complicated by subdural biliary hygromas: a case report and review of the literature.
British journal of neurosurgeryCharacteristics and influencing factors of neuroendocrine dysfunction in patients with adult-onset craniopharyngioma.
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Neurosurgical reviewEfficacy and safety of proton therapy and photon therapy for patients with craniopharyngioma: a systematic review and meta‑analysis.
PituitaryAccuracy of Tumor Surveillance of Pediatric Adamantinomatous Craniopharyngioma Using Noncontrast Brain MRI.
AJNR. American journal of neuroradiologyVirtual Reality and Three-Dimensional Printing of Craniopharyngioma: A Case Report Proposing an Innovative Model of Pediatric Endocrine Care to Enhance Patient Engagement.
Clinical case reportsClinical characteristics, surgical and endocrinological outcomes of sellar and parasellar masses in pediatric patients: a single-center experience.
PituitaryCausal Effects of Exposure to Air Pollution on the Risk of Neurosurgical Multi-system Diseases: A Worldwide Study of Mendelian Randomization.
International journal of medical sciencesLimitations of MRI in differentiating solid and cystic components of craniopharyngiomas.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryLinear regression models predict survival and complication rates in patients undergoing gamma knife radiosurgery for recurrent and residual craniopharyngiomas: a meta-analysis of 743 tumors.
Journal of neuro-oncologyPituitary Macroadenoma and Adamantinomatous Craniopharyngioma: A Rare Case Report of Sellar Collision Tumors.
Case reports in neurological medicineHypothalamic Morbidities and Endocrinological Outcomes Following Transcranial and Endoscopic Endonasal Resection of Craniopharyngiomas: A Systematic Review and Meta-Analysis.
Neurology IndiaBrown tumor in the pterygopalatine fossa with maxillary and sphenoidal involvement: a rare case report and literature review.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryTrametinib-induced Hyponatremia in a Patient With Craniopharyngioma and Diabetes Insipidus.
Journal of pediatric hematology/oncologyEffect of Extent of Resection and Adjuvant Radiation on Recurrence of BRAF versus β-Catenin-Mutated Craniopharyngioma: A Single Institutional Case Series.
Journal of neurological surgery. Part B, Skull baseTargeted Therapy for Recurrent and Refractory Papillary Craniopharyngioma: A Systematic Narrative Review.
World neurosurgeryIntrauterine-diagnosed fetal intracranial adamantinomatous craniopharyngioma: A case report with histopathological and molecular characterization.
International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and ObstetricsAlterations of Brain Structural and Functional Connectivity Networks and Its Correlations With Cognitive Function in Patients With Hypothalamic Syndrome Following Craniopharyngioma Resection.
Brain and behavior[Anatomical variants of papillary craniopharyngiomas: analysis of surgical series].
Zhurnal voprosy neirokhirurgii imeni N. N. BurdenkoEpidemiology of Primary Intracranial Tumors at a Tertiary Care Hospital in Central India: A Retrospective Study.
CureusHepatopulmonary Syndrome as First Presentation of Liver Cirrhosis.
The Journal of the Association of Physicians of IndiaClinical characteristics of visual function, retinal ganglion cells, and nerve fiber layer in patients with adult craniopharyngioma.
Japanese journal of ophthalmologySemaglutide treatment in hypothalamic obesity: Two-Year outcomes on body composition, appetite, and quality of life.
PituitaryEvaluation of the Effect of Optic Nerve Compression by Craniopharyngioma on Retinal Nerve Fiber Layer Thickness in Pediatric Patients.
CancersComparative Outcomes of Gross Total Resection vs. Subtotal Resection Plus Radiotherapy for Preventing Craniopharyngioma Recurrence: A Meta-Analysis of the Endoscopic Endonasal Approach.
CancersB7-H3 in Cancer Immunotherapy-Prospects and Challenges: A Review of the Literature.
CellsEndoscopic Endonasal Approach for Craniopharyngiomas With Intraventricular Extension: Anatomic-Clinical Considerations and Surgical Outcomes in a Series of 61 Patients.
Operative neurosurgery (Hagerstown, Md.)Ectopic clival craniopharyngioma.
NeuroradiologyRE: "Craniopharyngiomas in children: the pendulum moves again for an aggressive surgery-late complications and considerations with a recent series of 26 patients treated in Lyon".
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryUse of integrated spatial transcriptomics and histopathological analysis in adamantinomatous craniopharyngiomas to identify stromal cells as a new cellular source of leukemia inhibitory factor.
Journal of neurosurgeryWhole exome sequencing study of adamantinomatous craniopharyngioma reveals the mutational characteristics of recurrent cases.
Journal of neuro-oncologyEctopic Sinonasal Adamantinomatous Craniopharyngioma in an Adolescent.
Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of IndiaComparative analysis of clinical features, methylation and immune microenvironment in pediatric and adult papillary craniopharyngiomas: results from a multicenter study.
Scientific reportsClinical integration and application of the 2022 WHO pituitary tumor classification.
Neuro-oncology advances'Un-thirsty' hypernatremia.
Endocrinology, diabetes & metabolism case reportsTargeted therapy of papillary craniopharyngioma.
Medical oncology (Northwood, London, England)Transsellar and Transplanum Endoscopic Endonasal Resection of a Sellar and Suprasellar Craniopharyngioma With Virtual Reality Modeling: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)The many different clinical faces of acquired hypothalamic dysfunction: a retrospective cohort study in the Netherlands.
EClinicalMedicineSuccessful Treatment of Hypothalamic Obesity with Tirzepatide.
AACE endocrinology and diabetesMinimally Invasive Cyst Drainage for Cystic Craniopharyngioma: Neuroendoscopic Fenestration or Stereotactic Cyst Drainage?
World neurosurgeryProtect the pituitary stalks in craniopharyngioma resection by endoscopic endonasal approach.
Journal of endocrinological investigationLetter to "Tumor features in adult papillary and adamantinomatous craniopharyngioma: neuroradiological evaluation of pituitary-hypothalamic-axis dysfunction and outcome prediction. Neuroradiology".
NeuroradiologyIntracranial Collision Tumor: Coexistence of Pituitary Adenoma and Craniopharyngioma.
The Journal of craniofacial surgeryRacemose neurocysticercosis mimicking craniopharyngioma.
Revista da Sociedade Brasileira de Medicina TropicalHigh-risk pregnancy complicated by craniopharyngioma: diagnosis in the context of visual impairment and tumor resection during pregnancy following IVF.
Endocrinology, diabetes & metabolism case reportsComprehensive molecular characterization of craniopharyngiomas using whole transcriptome and spatial transcriptomics approaches.
Brain tumor pathologyEndocrine and metabolic consequences of childhood-onset craniopharyngioma during the transition age: A literature review by the TALENT study group.
Reviews in endocrine & metabolic disordersSuccessful Pregnancy in Woman With Hyperprolactinemia Associated With Hemorrhagic Giant Craniopharyngioma.
Maternal-fetal medicine (Wolters Kluwer Health, Inc.)Primary pediatric brain tumors in Africa: a systematic review and comparative meta-analysis.
Journal of neurosurgery. PediatricsTargeted Treatment of Pediatric Craniopharyngioma: Lesson Learnt After 20-Year Experience with Intracystic Interferon Alpha.
Advances and technical standards in neurosurgeryPediatric Craniopharyngioma Survival Outcomes at the U.S.-Mexico Border: Results from a Cross-Border Neuro-Oncology Program.
World neurosurgeryRare secondary pituitary abscess arising in a craniopharyngioma: A case report and literature review.
Experimental and therapeutic medicineFAP as a prognostic biomarker and radiomics-based predictor of angiogenesis-associated recurrence in Adamantinomatous craniopharyngioma.
PituitarySafety Profile of Select Perioperative Lumbar Drain Use After Endoscopic Endonasal Surgery.
Operative neurosurgery (Hagerstown, Md.)Follow up assessment on the first local theranostic intra-cavitary Yttrium-90 citrate colloid irradiation for refractory cystic craniopharyngioma: Is it still a valuable treatment option?
Asia Oceania journal of nuclear medicine & biologyImpaired splicing machinery in craniopharyngiomas unveils PRPF8 and RAVER1 as novel biomarkers and therapeutic targets.
Acta neuropathologica communicationsMethylation and gene expression patterns in adamantinomatous craniopharyngioma highlight a panel of genes associated with disease progression-free survival.
Frontiers in endocrinologyEEA for sellar chodrosarcomas: case series with literature review.
Chinese neurosurgical journalLactate levels during anesthesia in patients undergoing craniopharyngioma surgery.
Frontiers in surgerySurgical strategies in pediatric craniopharyngioma-really a "pendulum" of surgical radicality or rather an evolution towards preserving the long-term quality of life?
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgerySkull base surgery via extended endoscopic endonasal approach: Predictors of ear-nose-throat complications.
Acta otorrinolaringologica espanolaQuality of life in pediatric patients treated with adjuvant proton radiation for craniopharyngiomas.
Journal of neuro-oncologyThe management of hypothalamic obesity in craniopharyngioma.
Best practice & research. Clinical endocrinology & metabolismRegarding "Neuroendocrine Deficits and Weight Development Before and After Proton Therapy in Children With Craniopharyngioma" by Bischoff et al.
Clinical oncology (Royal College of Radiologists (Great Britain))Prolactin serum concentrations in childhood-onset craniopharyngioma patients.
Journal of endocrinological investigationMachine learning method based on radiomics help differentiate posterior pituitary tumors from pituitary neuroendocrine tumors and craniopharyngioma.
Scientific reportsGlobal Trends of Craniopharyngioma Research: A Web of Science-Based Bibliometric Analysis from 1980 to 2024.
World neurosurgeryAssessment of Clinical and Neurological Alterations Before Radiation Therapy in Children With Malignant Brain Tumours.
Clinical oncology (Royal College of Radiologists (Great Britain))A rare case of giant infrasellar craniopharyngioma with extensive invasion of the pterygopalatine fossa: A case report and literature review.
Surgical neurology internationalAnaphylaxis associated with Tisseel fibrin sealant during re-resection of a craniopharyngioma: illustrative case.
Journal of neurosurgery. Case lessonsPituitary Blastoma: Expanding the Spectrum of Histopathological Findings in a Young Adult With DICER1 Mutation.
Neuropathology : official journal of the Japanese Society of Neuropathology[Sellar collision tumors: difficulties of preoperative neuroimaging and selection of surgical approach. Case reports and literature review].
Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko[Pituitary adenoma combined with craniopharyngioma. Case reports and literature review].
Zhurnal voprosy neirokhirurgii imeni N. N. BurdenkoA case-based review of adult-onset craniopharyngioma.
Frontiers in endocrinologyRecent advances in craniopharyngioma pathophysiology and emerging therapeutic approaches.
Frontiers in endocrinologyThe Efficacy of Semaglutide on Hypothalamic Obesity Caused by Craniopharyngioma Surgery.
Clinical endocrinologyManagement of Acquired Hypothalamic Obesity After Childhood-Onset Craniopharyngioma-A Narrative Review.
BiomedicinesImpact of AI Decision Support on Clinical Experts' Radiographic Interpretation of Adamantinomatous Craniopharyngioma.
AMIA ... Annual Symposium proceedings. AMIA SymposiumRevisiting the combined approach of Yaşargil for microsurgical removal of intra-extraventricular and pure intraventricular craniopharyngiomas.
Acta neurochirurgicaEndoscopic Super Extended Endonasal Approach: Transplanum Transtubercullum With Pituitary Hemitransposition and Tailored Transclival Approach for the Resection of a Giant Craniopharyngioma: 2-Dimensional Operative Video.
Operative neurosurgery (Hagerstown, Md.)Craniopharyngioma, Rathke Cleft Cyst, and Empty Sella Natural History and Endocrine Outcome in Pediatric Age: A Single Tertiary Center Experience over the 2000-2024 Period.
NeuroendocrinologyLetter: Commentary: Is It Justified to Sacrifice the Pituitary Stalk During Craniopharyngioma Surgery? A Systematic Review and Meta-Analysis.
NeurosurgeryGrowth Hormone Replacement in Craniopharyngioma: Analysis of the Hypopituitary Control and Complications Study (HypoCCS).
Journal of the Endocrine SocietyEndoscopic transorbital approach to posterior fossa recurrent craniopharyngioma.
Neurosurgical focus: VideoHypopituitarism in non-neuroendocrine pituitary tumors: a systematic review.
Reviews in endocrine & metabolic disordersAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Diagnostic yield from minimally invasive pediatric brain tumor biopsies in the molecular era.
- Surgical outcomes and longitudinal quality of life after endoscopic endonasal resection of craniopharyngioma.Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia· 2026· PMID 41690055mais citado
- Ventriculo-gallbladder shunt reflux: A case study and review of the literature.
- Intraoperative MRI for endoscopic endonasal transsphenoidal surgery in children.
- Secondary narcolepsy and cognitive dysfunction related to craniopharyngioma: a case study.
- Risk Factors for Postoperative Cerebrospinal Fluid Leak Following Endoscopic Transsphenoidal Surgery for Craniopharyngioma: A Multicenter Cohort Study with a Contemporary Surgeon Practice Survey.
- Gamma Knife Stereotactic Radiosurgery for Pediatric Meningiomas.
- Case report of transsphenoidal meningocele presenting as airway obstruction and panhypopituitarism: Radiological findings and the role of CSF flow analysis.
- [Hypothalamic obesity in adults with craniopharyngiomas: prevalence and risk factors before and after neurosurgical treatment].
- Postoperative Visual Outcomes in Pediatric Craniopharyngioma: A Comparison of Endoscopic Endonasal and Transcranial Approaches.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:54595(Orphanet)
- MONDO:0018907(MONDO)
- GARD:10486(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q1786513(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
