RESUMEN
Visual evoked potential (VEP) is an established modality that allows safe brain tumor resection and preservation of optical function. We herein present a case of a pediatric craniopharyngioma with significant improvement in the VEP amplitude detected during endoscopic transsphenoidal surgery (ETS) and obvious postoperative improvement in visual acuity. A 13-year-old boy presented with visual acuity disturbance in his right eye and was followed up for 5 months by an ophthalmologist. His visual acuity rapidly worsened, and a suprasellar lesion with calcification was found on brain computed tomography. The patient underwent tumor resection during ETS with intraoperative transcranial VEP monitoring. Gross total tumor resection was achieved without injury to the perforators, including the superior hypophyseal arteries. The VEP amplitude was unstable, and significant waves were not detectable before tumor resection; however, a positive wave was detected after removing most of the tumor and exposing the bilateral optic nerves and optic chiasm. Subsequently, negative and positive VEP waves were continuously detected. Visual acuity improved remarkably on postoperative day 10. This case demonstrated both a significant increase in the intraoperative VEP amplitude and rapid postoperative improvement in visual acuity. We surmised that the preoperative rapid worsening of visual dysfunction, intraoperative increase in the VEP amplitude, and significant postoperative improvement in visual acuity were associated with the compression of the optic nerves by the internal carotid artery, anterior cerebral artery, and tumor.
Asunto(s)
Craneofaringioma , Potenciales Evocados Visuales , Neoplasias Hipofisarias , Agudeza Visual , Humanos , Masculino , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/complicaciones , Adolescente , Potenciales Evocados Visuales/fisiología , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/complicaciones , Agudeza Visual/fisiología , Monitorización Neurofisiológica Intraoperatoria/métodosRESUMEN
Rathke's Cleft Cysts (RCCs) and Craniopharyngiomas (CPs) may represent disease entities on the same etio-pathological spectrum. We report the case of a 36-year-old female presenting with vision loss and menstrual irregularities, imaging shows a predominantly cystic lesion in the sellar region with suprasellar extension. She underwent a microscopic transnasal resection of the lesion. She later presented with recurrent symptoms and increased residual lesion size on imaging, a transcranial excision of the lesion was performed. Histopathology from the initial operative specimen revealed RCC with squamous metaplasia which was BRAF negative, while the specimen from the second surgery revealed BRAF positive papillary stratified squamous architecture suggestive of Papillary CP. This case adds to the evidence that both RCCs and papillary CPs may be the spectrum of the same disease. Further, papillary CPs may be an evolution from the RCCs.
Asunto(s)
Quistes del Sistema Nervioso Central , Craneofaringioma , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/patología , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/diagnóstico , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/patología , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Femenino , Adulto , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagenRESUMEN
In May 1968, Lars Leksell and Erik-Olof Backlund achieved a pioneering breakthrough by performing the first Gamma Knife radiosurgery (GKRS) on a craniopharyngioma (CP). Today, more than 50 years later, this patient remains under continuous monitoring, providing the longest documented follow-up of a GKRS-treated CP. This case report provides a complete record of the patient's preoperative presentation, surgical assessment, GKRS, and an extensive long-term follow-up with multiple interventions. The investigation involved analysis of archived and digitalized patient records and radiological images. The patient was a 21-year-old female who presented with amenorrhea and low levels of gonadotropins. Pneumoencephalography showed a calcified 2 × 2.5 cm mass located in the suprasellar region, indicative of a CP. Subsequent stereotactic puncture confirmed a predominantly solid nature of the CP. Given the size and composition of the tumor, the surgical team opted for GKRS. Dose planning was performed manually, with coordinates determined using Leksell's stereotactic frame. The tumor was targeted with a total dose of 50 Gy using 179 beams of 60 Co. Over the subsequent 55 years, the patient underwent radiological and clinical follow-ups. Throughout this period, 4 cystic tumor recurrences occurred and were managed by stereotactic puncture and Yttrium-90 instillation radiotherapy. The solid component remained stable without repeated GKRS. The patient suffered lateral quadrant anopsia and endocrinological deficits, necessitating pharmaceutical intervention. Despite these challenges, the patient is still living an active life at age 76 years. This case stands as historic evidence of long-term safety and efficacy of GKRS for CPs.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Radiocirugia , Humanos , Craneofaringioma/cirugía , Craneofaringioma/radioterapia , Craneofaringioma/diagnóstico por imagen , Radiocirugia/métodos , Femenino , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/radioterapia , Adulto Joven , Estudios de Seguimiento , Persona de Mediana Edad , Resultado del Tratamiento , AdultoRESUMEN
PURPOSE: Basal duct-like recess (DR) sign serves as a specific marker of papillary craniopharyngiomas (PCPs) of the strictly third-ventricular (3 V) topography. Origins of this sign are poorly understood with limited validation in external cohorts. METHODS: In this retrospective study, MRIs of pathologically proven PCPs were reviewed and evaluated for tumor topography, DR sign prevalence, and morphological subtypes. RESULTS: Twenty-three cases with 24 MRIs satisfied our inclusion criteria. Median age was 44.5 years with a predominant male distribution (M/F ratio 4.7:1). Overall, strictly 3 V was the commonest tumor topography (8/24, 33.3%), and tumors were most commonly solid-cystic (10/24, 41.7%). The prevalence of DR sign was 21.7% (5/23 cases), all with strictly 3 V topography and with a predominantly solid consistency. The sensitivity, specificity and positive and negative predictive value of the DR sign for strict 3 V topography was 62.5%, 100%, 100% and 84.2% respectively. New pertinent findings associated with the DR sign were observed in our cohort. This included development of the cleft-like variant of DR sign after a 9-year follow-up initially absent at baseline imaging. Additionally, cystic dilatation of the basal tumor cleft at the pituitary stalk-tumor junction and presence of a vascular structure overlapping the DR sign were noted. Relevant mechanisms, hypotheses, and implications were explored. CONCLUSION: We confirm the DR sign as a highly specific marker of the strictly 3 V topography in PCPs. While embryological and molecular factors remain pertinent in understanding origins of the DR sign, non-embryological mechanisms may play a role in development of the cleft-like variant.
Asunto(s)
Craneofaringioma , Imagen por Resonancia Magnética , Neoplasias Hipofisarias , Sensibilidad y Especificidad , Humanos , Masculino , Craneofaringioma/diagnóstico por imagen , Femenino , Neoplasias Hipofisarias/diagnóstico por imagen , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Imagen por Resonancia Magnética/métodos , Anciano , Prevalencia , Adolescente , Tercer Ventrículo/diagnóstico por imagen , Tercer Ventrículo/patologíaRESUMEN
PURPOSE: We studied a pediatric group of patients with sellar-suprasellar tumors, aiming to develop a convolutional deep learning algorithm for radiological assistance to classify them into their respective cohort. METHODS: T1w and T2w preoperative magnetic resonance images of 226 Chilean patients were collected at the Institute of Neurosurgery Dr. Alfonso Asenjo (INCA), which were divided into three classes: healthy control (68 subjects), craniopharyngioma (58 subjects) and differential sellar/suprasellar tumors (100 subjects). RESULTS: The PPV among classes was 0.828±0.039, and the NPV was 0.919±0.063. Also explainable artificial intelligence (XAI) was used, finding that structures that are relevant during diagnosis and radiological evaluation highly influence the decision-making process of the machine. CONCLUSION: This is the first experience of this kind of study in our institution, and it led to promising results on the task of radiological diagnostic support based on explainable artificial intelligence (AI) and deep learning models.
Asunto(s)
Craneofaringioma , Aprendizaje Profundo , Imagen por Resonancia Magnética , Neoplasias Hipofisarias , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/diagnóstico , Craneofaringioma/cirugía , Niño , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/diagnóstico , Masculino , Femenino , Imagen por Resonancia Magnética/métodos , Adolescente , PreescolarRESUMEN
BACKGROUND: Differential diagnosis of hypothalamic-optic chiasmatic gliomas (HOCGs) and craniopharyngiomas on magnetic resonance imaging (MRI) can be quite challenging. PURPOSE: To compare the MRI features of HOCGs and cranipharyngiomas. MATERIAL AND METHODS: Patients diagnosed with HOCG or craniopharyngioma in histopathological evaluation between 2012 and 2022 and who underwent preoperative contrast-enhanced brain MRI were included. Various MRI features were retrospectively evaluated for each lesion: T2-weighted imaging and fluid attenuation inversion recovery hyperintensity, calcification, cystic change, T1-weighted (T1W) imaging hyperintensity of the cystic component, hemorrhage, involvement of sellar, suprasellar or other adjacent structures, lobulated appearance, presence of hydrocephalus, and contrast enhancement pattern. Apparent diffusion coefficient (ADC) values were also evaluated and compared. RESULTS: Among 38 patients included, 13 (34%) had HOCG and 25 (66%) had craniopharyngioma. Craniopharyngiomas had a significantly higher rate of cystic changes, calcification, and T1W imaging hyperintensity of the cystic component than HOCGs (P <0.05). Of HOCGs, 92% had chiasm involvement, 23% had optic nerve involvement, and 31% had brain stem involvement. On the other hand, chiasm involvement was observed in 8% of craniopharyngiomas, but none had optic nerve and/or brain stem involvement (P <0.05). While 62% (8/13) of HOCGs had diffuse homogeneous enhancement, 80% (20/25) of craniopharyngiomas had a diffuse heterogeneous enhancement pattern. Mean ADC values were significantly higher in craniopharyngiomas compared to HOCGs (2.1 vs. 1.6 ×10-3mm2/s, P <0.05). CONCLUSION: Although some neuroimaging findings may overlap, features such as presence of cyst and calcification, brain stem and optic pathway involvement, different enhancement patterns, and ADC values may be helpful in the differential diagnosis of HOCGs and craniopharyngiomas.
Asunto(s)
Craneofaringioma , Glioma , Imagen por Resonancia Magnética , Quiasma Óptico , Neoplasias Hipofisarias , Humanos , Craneofaringioma/diagnóstico por imagen , Masculino , Femenino , Imagen por Resonancia Magnética/métodos , Adulto , Persona de Mediana Edad , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Estudios Retrospectivos , Diagnóstico Diferencial , Adolescente , Glioma/diagnóstico por imagen , Glioma/patología , Quiasma Óptico/diagnóstico por imagen , Quiasma Óptico/patología , Adulto Joven , Niño , Anciano , Neoplasias Hipotalámicas/diagnóstico por imagen , Preescolar , Medios de ContrasteRESUMEN
BACKGROUND: Craniopharyngiomas are benign tumors of the anterior skull base arising from epithelial remnants of Rathke pouch. They mainly occur in the suprasellar space, can be incredibly debilitating, and remain difficult to resect as they frequently involve critical neurovascular structures. Although it is embryologically possible for craniopharyngiomas to arise extracranially along the entire migrational path of Rathke pouch, these remain exceedingly rare, especially among adults, and can be mistaken for nasopharyngeal cancer. As such, minimal data exist evaluating the management and outcomes of such lesions. We evaluated our institutional experience with purely infrasellar nasopharyngeal craniopharyngiomas and obtained individual patient data reported in the contemporary literature to better characterize the demographics, presentation, surgical management, and long-term outcomes of these lesions. METHODS: A systematic review of the literature was performed to identify previously published cases of purely infrasellar nasopharyngeal craniopharyngioma in 3 electronic databases: MEDLINE (PubMed), Embase, and Scopus. Search terms were "infrasellar craniopharyngioma" and "nasopharyngeal craniopharyngioma." RESULTS: We identified 25 cases, in which 72% of patients presented with symptoms of nasal obstruction, epistaxis, or headache. An endoscopic approach was performed in 40% of cases; 83.3% of all patients had gross total resection, with 60% having no recurrence at a median follow-up of 13 months. No postoperative complications were reported. Tumor location involving the cavernous sinus was associated with incomplete resection (100%) compared with tumors not involving the cavernous sinus (87%) (P = 0.033). CONCLUSIONS: While uncommon, infrasellar nasopharyngeal craniopharyngiomas appear to have better perioperative and long-term surgical outcomes than their suprasellar counterparts.
Asunto(s)
Craneofaringioma , Neoplasias Nasofaríngeas , Neoplasias Hipofisarias , Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Neoplasias Nasofaríngeas/cirugía , Neoplasias Nasofaríngeas/diagnóstico por imagen , Procedimientos Neuroquirúrgicos/métodos , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagenRESUMEN
BACKGROUND: Traditional microsurgical approaches for addressing intraventricular craniopharyngioma provide limited access to the retrochiasmatic area and tumors with significant lateral or rostrocaudal extensions. Extended endoscopic endonasal approaches can effectively overcome many of limitations, yet they require a favorable working angle between the optic chiasm and pituitary gland, as well as the involvement of the third ventricle floor by the tumor. METHODS: Herein, the authors describe the surgical nuances of a keyhole technique for resecting third ventricle craniopharyngiomas via a fully endoscopic minimally invasive trans-eyebrow supraorbital translaminar approach (ESOTLA). A case description detailing the key surgical steps and application of the approach is provided, along with a series of cadaveric photographs to highlight the relevant anatomy and step-by-step dissection process. RESULTS: The patient is a 44-year-old man who presented with polyuria, low urine specific gravity, and panhypopituitarism. Brain magnetic resonance imaging revealed a solid-cystic heterogeneous-enhanced retrochiasmatic mass within the third ventricle, consistent with craniopharyngioma. A 1-stage ESOTLA was indicated based on the narrow pituitary-chiasm angle and the high functional status of the patient. Near-total resection was achieved, and no new postoperative neurologic or endocrine change was observed. Targeted therapy was implemented based on the histologic result, and the most recent surveillance magnetic resonance imaging showed no evidence of the residual tumor. CONCLUSIONS: By combining a keyhole approach with variable-angle endoscopic visualization through a smaller bony and soft tissue exposure, ESOTLA can provide enhanced illumination within the third ventricle, potentially addressing cosmetic concerns and limited exposure area/angle of freedom associated with its conventional microsurgical counterpart.
Asunto(s)
Craneofaringioma , Neuroendoscopía , Neoplasias Hipofisarias , Tercer Ventrículo , Humanos , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Masculino , Tercer Ventrículo/cirugía , Tercer Ventrículo/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Neuroendoscopía/métodos , Adulto , Neoplasias del Ventrículo Cerebral/cirugía , Neoplasias del Ventrículo Cerebral/diagnóstico por imagen , Neoplasias del Ventrículo Cerebral/patología , Cejas , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Procedimientos Neuroquirúrgicos/métodos , Imagen por Resonancia MagnéticaAsunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/patología , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Masculino , FemeninoRESUMEN
BACKGROUND: The management of craniopharyngiomas is challenging due to their high rate of recurrence following resection. Excision of recurrent tumors poses further surgical challenges due to loss of arachnoidal planes and adherence to anatomical structures. The endoscopic endonasal approach (EEA) offers a favorable alternative to transcranial approaches for primary craniopharyngiomas. However, the safety and efficacy of EEA for recurrent tumors, specifically after a prior transcranial approach, needs further investigation. METHODS: We performed a systematic review using PubMed to develop a database of cases of recurrent craniopharyngiomas previously treated with a transcranial approach. RESULTS: Fifteen articles were included in this review with a total of 75 cases. There were 50 males and 25 females with a mean age of 38 years (range 2-80). One prior transcranial surgery was done in 80.0% of cases, while 8.0% had two and 12.0% had more than two prior surgeries. Radiotherapy after transcranial resection was given in 18 cases (24.0%). Following EEA, vision improved in 60.0% of cases, and vision worsened in 8.6% of the cases. Of cases, 64.4% had pre-existing anterior hypopituitarism, and 43.8% had diabetes insipidus prior to EEA. New anterior hypopituitarism and diabetes insipidus developed in 24.6% and 21.9% of cases, respectively following EEA. Gross total resection (GTR) was achieved in 64.0%, subtotal resection in 32.0%, and partial resection in 4.0% revision EEA cases. GTR rate was higher in cases with no prior radiotherapy compared to cases with prior radiotherapy (72.0% vs 39.0%, p = 0.0372). The recurrence rate was 17.5% overall but was significantly lower at 10.0% following GTR (p = 0.0019). The average follow-up length was 41.2 months (range, 1-182 months). CONCLUSION: The EEA can be utilized for resection of recurrent or residual craniopharyngiomas previously managed by a transcranial approach.
Asunto(s)
Craneofaringioma , Diabetes Insípida , Hipopituitarismo , Neoplasias Hipofisarias , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Endoscopía , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patologíaRESUMEN
Preoperative simulation for endoscopic endonasal approach(EEA)using computed tomography and magnetic resonance imaging evaluates tumor extension and the relationship between adjacent structure(the pituitary stalk, major vessels, and cranial nerves); therefore, preoperative planning of nasal procedure, skull base bony removal, and cranial base reconstruction are possible. Additionally, three-dimensional(3D)fusion image aids surgeons to visualize intraoperative 3D findings. These preoperative simulations are critical to avoid complications and predict pitfalls perioperatively. However, tumor consistency or adhesion with adjacent structure cannot be predicted but is judged perioperatively, which affects the extent of tumor resection. This manuscript describes important points of preoperative simulation for EEA, especially the transplanum-transtuberculum approach for craniopharyngiomas or tuberculum sellae meningiomas, showing some examples in patients.
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Craneofaringioma , Neoplasias Meníngeas , Meningioma , Neuroendoscopía , Neoplasias Hipofisarias , Neoplasias de la Base del Cráneo , Humanos , Meningioma/diagnóstico por imagen , Meningioma/cirugía , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Neuroendoscopía/métodos , Silla Turca/diagnóstico por imagen , Silla Turca/cirugía , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/cirugía , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugíaRESUMEN
OBJECTIVE: Due to the underdeveloped skull base in children, it is crucial to predict whether a sufficient surgical window for an endoscopic endonasal approach can be achieved. This study aimed to analyze the presumed surgical window through measurement of the intersiphon distance (ISD) and the planum-sella height (PSH) on the basis of age and its correlation with the actual surgical window for the endoscopic transtuberculum approach. METHODS: Twenty patients of each age from 3 to 18 years were included as the normal skull base population. ISD and PSH were measured and compared among consecutive ages. Additionally, 42 children with craniopharyngiomas or Rathke's cleft cysts who underwent treatment via the endoscopic transtuberculum approach were included. ISD and PSH were measured on preoperative images and then correlated with the dimensions of the surgical window on postoperative CT scans. The intraoperative endoscopic view was classified as narrow, intermediate, or wide based on operative photographs or videos, and relevant clinical factors were analyzed. RESULTS: In the normal skull base population, both ISD and the estimated area of the surgical window increased with age, particularly at 8 and 11 years old. On the other hand, PSH did not show an incremental pattern with age. Among the 42 children who underwent surgery, 24 had craniopharyngioma and 18 had Rathke's cleft cysts. ISD showed the strongest correlation with the actual area of the surgical window [r(40) = 0.69, p < 0.001] rather than with age or PSH. The visual grade of the intraoperative endoscopic view was narrow in 17 patients, intermediate in 21, and wide in 4. Preoperative ISD was 14.58 ± 1.29 mm in the narrow group, 16.13 ± 2.30 mm in the intermediate group, and 18.09 ± 3.43 mm in the wide group (p < 0.01). There were no differences in terms of extent of resection (p = 0.41); however, 2 patients in the narrow group had postoperative complications. CONCLUSIONS: Normal skull base development exhibited age-related growth. However, in children with suprasellar lesions, the measurement of the ISD showed a better correlation than age for predicting the surgical window for the endoscopic transtuberculum approach. Children with a small ISD should be approached with caution due to the limited surgical window.
Asunto(s)
Craneofaringioma , Base del Cráneo , Humanos , Niño , Adolescente , Base del Cráneo/cirugía , Base del Cráneo/diagnóstico por imagen , Masculino , Femenino , Preescolar , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neuroendoscopía/métodos , Quistes del Sistema Nervioso Central/cirugía , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Endoscopía/métodos , Estudios Retrospectivos , Silla Turca/cirugía , Silla Turca/diagnóstico por imagenRESUMEN
OBJECTIVE: We report our early clinical experience with image-guided, pencil beam scanning proton beam therapy (PBS-PBT) for residual and recurrent craniopharyngioma. METHODS: Between September 2019 and January 2023, 19 consecutive patients with residual or recurrent craniopharyngioma, suitable for radiotherapy and treated with image-guided PBS-PBT were analyzed. We documented detailed dosimetric data, acute toxicities, early outcomes, and imaging response on follow-up magnetic resonance imaging scans. RESULTS: A total of 19 patients (11 males and 8 females) with residual or recurrent craniopharyngioma were treated during the study period. The median age of the cohort was 14 years (range, 3-33 years). The histology of most lesions was the adamantinomatous subtype (95%). The most common clinical presentation (before PBT) and most common endocrine deficit was visual disturbance (79%) and hypocortisolism (74%), respectively. Of the 19 patients, 13 had recurrent craniopharyngioma, and 5 had undergone radiotherapy previously. Five patients (26%) had undergone surgery ≥3 times before proton therapy. The median dose delivered was 54 GyE. The most common acute toxicity was grade 1 alopecia (63%). No patient experienced grade ≥3 acute toxicity. With a median follow-up of 18 months (range, 3-40 months), 12 patients showed shrinkage of the residual tumor and/or cyst, and 4 showed a dramatic cyst reduction at 3-9 months of follow-up. Two patients experienced a reduction in both solid and cystic components, with the remaining experiencing a reduction in the cystic component only. The remaining 8 patients had stable disease on magnetic resonance imaging, with 100% disease control and overall survival. Visual function remained stable after treatment. CONCLUSIONS: Our preliminary experience with modern PBS-PBT and image guidance for craniopharyngioma is encouraging. Proton therapy in our cohort was well tolerated, resulting in limited toxicity and promising early outcomes.
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Craneofaringioma , Neoplasias Hipofisarias , Terapia de Protones , Humanos , Craneofaringioma/radioterapia , Craneofaringioma/diagnóstico por imagen , Femenino , Masculino , Terapia de Protones/métodos , Adulto , Neoplasias Hipofisarias/radioterapia , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Adolescente , Niño , Adulto Joven , India , Preescolar , Recurrencia Local de Neoplasia/radioterapia , Radioterapia Guiada por Imagen/métodos , Resultado del Tratamiento , Imagen por Resonancia MagnéticaRESUMEN
Cystic craniopharyngiomas of the third ventricle can be challenging to treat because complete resection of the cyst wall can be associated with hypothalamic dysfunction and minimal rostral displacement of the optic chiasm leads to a small endonasal operative corridor. Various methods to overcome the frequent recurrences have been described, such as intracystic bleomycin or catheter placement, with mixed results.1-12 In Video 1, we describe a simple cystocisternal fenestration technique with preservation of the rostral cyst wall via an endoscopic endonasal approach where the solid portion of the tumor is resected, and the inferior wall of the cyst is opened into the prepontine cistern and the superior wall of the cyst and adjacent third ventricle are preserved. This allows for ventricular pressure to collapse the cyst cavity in the postoperative period. In select patients where safe complete resection of a cystic craniopharyngioma is prohibitive, this may provide a durable treatment and can be performed through a small endonasal corridor below a nondisplaced optic chiasm.
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Craneofaringioma , Neuroendoscopía , Neoplasias Hipofisarias , Tercer Ventrículo , Humanos , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Tercer Ventrículo/cirugía , Neuroendoscopía/métodos , Neoplasias del Ventrículo Cerebral/cirugía , Neoplasias del Ventrículo Cerebral/diagnóstico por imagen , Seno Esfenoidal/cirugía , MasculinoRESUMEN
Craniopharyngiomas are histologically benign tumors that originate from squamous rests along the pituitary stalk. They make up approximately 1.2% to 4.6% of all intracranial tumors and do not show significant differences in occurrence based on sex. Adamantinomatous craniopharyngiomas have 2 peaks of incidence, commonly observed in patients from ages 5 to 15 years and again from 45 to 60 years. In contrast, papillary craniopharyngiomas mainly affect adults in their fifth and sixth decades of life.1 The "malignancy" of craniopharyngiomas is attributed to their location and the challenges associated with achieving complete removal because they can manifest in the sellar, parachiasmatic, and intraventricular regions or a combination of these.2,3 Various approaches have been used to resect these tumors.4,5 Radical resection offers the most promising option for disease control, potential cure, and the ability to transform the disease from lethal to survivable in children, allowing for a functional adult life.2,3 Meticulous evaluation is crucial to determine the appropriate approach and side, with particular emphasis on closely examining the relationship between the tumor and optic pathways (nerve, chiasm, tract), which are frequently involved. This assessment should also include the tumor's relationship with other crucial structures, such as the hypothalamus and adjacent arteries, to ensure that the strategy is adjusted accordingly to further minimize the risk of postoperative morbidity. Video 1 demonstrates a left-sided pterional transsylvian approach to remove a parachiasmatic craniopharyngioma involving the left optic chiasm and tract.
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Craneofaringioma , Neoplasias Hipofisarias , Adulto , Niño , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Hipófisis/patología , Hipotálamo/patología , Quiasma Óptico/diagnóstico por imagen , Quiasma Óptico/cirugía , Quiasma Óptico/patologíaRESUMEN
CONTEXT: Outcome of craniopharyngioma is related to its locoregional extension, which impacts resectability and the risk of surgical complications. To maximize resection and minimize complications, optic tract localization, temporal lobe extension, and hypothalamic involvement are essential factors for surgical management. OBJECTIVE: To assess the outcome of craniopharyngiomas depending on their relation to the hypothalamus location. METHODS: We conducted a retrospective analysis of 79 patients with a craniopharyngioma who underwent surgery from 2007 to 2022. Craniopharyngiomas were classified in 3 groups, depending on the type of hypothalamus involvement assessed by preoperative magnetic resonance imaging: infra-hypothalamic (type A, n = 33); perforating the hypothalamus (type B, n = 40); and supra-hypothalamic (type C, n = 6). Surgical strategy was guided by the type of hypothalamic involvement, favoring endonasal approaches for type A and type B, and transcranial approaches for type C. RESULTS: Long-term disease control was achieved in 33/33 (100%), 37/40 (92%), and 5/6 (83%) patients in type A, B, and C, respectively. In type B, vision was improved in 32/36 (89%) patients, while hypothalamic function was improved, stable, or worsened in 6/40 (15%), 32/40 (80%), and 2/40 (5%) patients, respectively. Papillary craniopharyngiomas were found in 5/33 (15%), 9/40 (22%), and 3/6 (50%) patients in types A, B, and C, respectively. In 4 patients, BRAF/MEK inhibitors were used, with significant tumor shrinkage in all cases. CONCLUSION: Craniopharyngiomas located below the hypothalamus or perforating it can be safely treated by transsphenoidal surgery. For supra-hypothalamic craniopharyngiomas, postoperative results are less favorable, and documenting a BRAF mutation may improve outcome, if targeted therapy was efficient enough to replace surgical debulking.
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Craneofaringioma , Hipotálamo , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/complicaciones , Craneofaringioma/diagnóstico por imagen , Masculino , Femenino , Estudios Retrospectivos , Adulto , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/diagnóstico por imagen , Hipotálamo/patología , Hipotálamo/cirugía , Hipotálamo/diagnóstico por imagen , Persona de Mediana Edad , Pronóstico , Adulto Joven , Anciano , Adolescente , Imagen por Resonancia Magnética , Procedimientos Neuroquirúrgicos , Resultado del Tratamiento , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/epidemiología , Estudios de Cohortes , Estudios de SeguimientoRESUMEN
BACKGROUND: In adamantinomatous craniopharyngiomas, tumor topographical categories, cystic component volume, and magnetic resonance signal intensity may impact prognosis. OBJECTIVE: To identify magnetic resonance imaging (MRI) variables associated with pituitary-hypothalamic axis dysfunction and predictive of outcome in children with cystic adamantinomatous craniopharyngiomas. MATERIALS AND METHODS: We evaluated 40 preoperative MRIs of adamantinomatous craniopharyngiomas to classify tumor topography, volume, and signal intensity of the cystic components and peritumoral edema. Volumes and normalized signal intensity minimum values were extracted from coronal T2-weighted images (nT2min). Radiological variables were compared to pituitary-hypothalamic axis dysfunction-related clinical data and surgical outcomes. RESULTS: Adamantinomatous craniopharyngiomas were categorized into five topographic classes (12 patients, sellar-suprasellar; seven patients, pseudo-intraventricular; six patients, strict intraventricular; 14 patients, secondary intraventricular; one patient, not strict intraventricular). All cases exhibited a predominant (30 patients, 80%) or total (10 patients, 20%) cystic tumor component and displayed low nT2min percentage values compared to cerebrospinal fluid (42.3% [interquartile range 28.4-54.6%]). Significant associations between tumor topographic classes and pituitary dysfunction (P<0.001), and between peritumoral edema and hypothalamic dysfunction (P<0.001) were found. Considering extent of surgical removal and tumor relapse, volume of the cystic tumor component displayed a positive correlation (P=0.002; r=0.48; P=0.02; r=0.36), while nT2min intensity values exhibited a negative correlation (P=0.01; r= - 0.40; P=0.028; r= - 0.34). CONCLUSION: Severe hypothalamic-pituitary axis dysfunction is associated with tumors along the pituitary stalk and peritumoral edema. Tumor invasion of the third ventricle, tight adherence to the hypothalamus, larger volumes, and lower nT2min intensity of the tumor cystic component are independent predictors of extent of adamantinomatous craniopharyngioma excision and recurrence.
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Craneofaringioma , Neoplasias Hipofisarias , Niño , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Recurrencia Local de Neoplasia/patología , Pronóstico , Imagen por Resonancia Magnética/métodos , Hipotálamo/diagnóstico por imagen , Hipotálamo/patología , EdemaRESUMEN
OBJECTIVE: The optimal surgical approach for pediatric craniopharyngiomas (CPs) remains a matter of debate, with selection bias classically precluding a fair comparison of outcomes between the transcranial approach (TCA) and endoscopic endonasal approach (EEA). The purpose of this systematic review was to analyze the current role of EEA in the treatment of pediatric CPs and to determine whether, upon expansion of its indications, a comparison with TCA is valid. METHODS: A systematic review of English-language articles published between February 2010 and June 2022 was performed to identify studies in the MEDLINE (PubMed) and Embase databases reporting on the resection of pediatric CPs. Included were articles reporting on pediatric CPs removed through TCA or EEA. Case reports, review articles, and earlier or less comprehensive series by the same center were excluded. Baseline characteristics and outcomes were analyzed. Prediction intervals (PIs), heterogeneity (Q, I2, and τ2 statistics), and publication bias (funnel plot analysis) were assessed. RESULTS: A total of 835 patients underwent TCA (18 articles) and 403 patients underwent EEA (19 articles). Preoperatively, the mean patient age (p = 0.055, PI = 5.05-15.11), visual impairment (p = 0.08, PI = 19.1-90.5, I2 = 80%), and hypothalamic syndrome (p = 0.17, PI = 6.5-52.2, I2 = 62%) did not significantly differ between the EEA and TCA groups. Endocrine deficit (anterior pituitary deficit [p < 0.001, PI = 16.5-92.9, I2 = 81%] and diabetes insipidus [p < 0.001, PI = 6.3-60.6, I2 = 43%]) was more frequent in the EEA group. Hydrocephalus and signs/symptoms of raised intracranial pressure were significantly higher (p < 0.001, PI = 5.2-73.3, I2 = 70% vs p < 0.001, PI = 4.6-73, I2 = 62%, respectively) in the TCA group. Recurrent lesions (p = 0.52, PI = 2.7-87.3, I2 = 13%), tumor size (p = 0.25, PI = 22.1-56.8), third ventricle involvement (p = 0.053, PI = 10.9-81.3, I2 = 69%), and hypothalamic involvement (p = 0.06, PI = 8.5-83.6, I2 = 79%) did not differ significantly between the approaches. EEA was preferred (p = 0.006, PI = 26.8-70.8, I2 = 40%) for sellar-suprasellar CPs, whereas TCA was preferred for purely suprasellar CPs (p = 0.007, PI = 13.5-81.1, I2 = 61%). There was no difference between the approaches for purely intrasellar lesions (p = 0.94, PI = 0-62.7, I2 = 26%). The breadth of PIs, I2 values, and analysis of publication bias showed substantial variability among the pooled data, hindering the possibility of outcome meta-analyses. CONCLUSIONS: With the adoption of extended approaches, the use of EEA became appropriate for a wider spectrum of pediatric CPs, with associated excellent outcomes. Although a fair comparison between outcomes in the EEA and TCA groups was hindered because of the differences in patient populations and tumor subtypes, given the increased versatility of EEA and improved expertise in its use, surgeons can now select the optimal surgical approach based on the unique benefits and drawbacks of each pediatric CP.
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Craneofaringioma , Neuroendoscopía , Neoplasias Hipofisarias , Niño , Humanos , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/complicaciones , Hidrocefalia/cirugía , Neuroendoscopía/efectos adversos , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/complicaciones , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
INTRODUCTION: Hypothalamic invasion in pediatric patients with craniopharyngioma negatively influences clinical outcomes. It has been shown that radiologic classification of hypothalamic invasion can effectively predict surgical strategies to minimize postoperative comorbidities in pediatric patients. However, no comparative analysis has been performed in adult patients with craniopharyngioma. This study implements the previously established radiologic classification to characterize postoperative morbidity, surgical outcome, and distress in adult patients with craniopharyngioma. METHODS: Electronic medical records of 22 adult patients with craniopharyngioma were used to analyze patient demographics, surgical data, endocrinologic and ophthalmologic status, and histopathology in a retrospective single-center study. Questionnaires regarding postoperative distress (National Comprehensive Cancer Network Distress Thermometer and Problem List), comorbidities (Charlson Comorbidity Index), employment status, and need for supportive care were distributed. Magnetic resonance imaging scans were categorized according to Puget et al. RESULTS: Patients with hypothalamic involvement show significantly higher rates of postoperative diabetes insipidus and higher scores on the National Comprehensive Cancer Network Distress Thermometer. This significant difference was lost when considering postoperative Puget grades. Puget grades 1 and 2 were found to be associated with the use of a subfrontal surgical approach (hazard ratio, 4.080; confidence interval, 1.153-14.431; P = 0.029). CONCLUSIONS: Our results point toward a possible predictive role of preoperative hypothalamic invasion for postoperative diabetes insipidus as well as higher perceived levels of distress after surgery, which may be established in larger patient cohorts. Furthermore, a subfrontal surgical approach seems to be predicted by tumors with hypothalamic invasion. In this case, preoperative magnetic resonance imaging grading may help guide the planning of an optimal surgical strategy for adults with craniopharyngioma to reduce postoperative morbidity.
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Craneofaringioma , Diabetes Insípida , Neoplasias Hipotalámicas , Neoplasias Hipofisarias , Adulto , Humanos , Niño , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/cirugía , Craneofaringioma/patología , Estudios Retrospectivos , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Hipotálamo/diagnóstico por imagen , Hipotálamo/cirugía , Hipotálamo/patología , Neoplasias Hipotalámicas/patología , Resultado del TratamientoRESUMEN
OBJECTIVE: Craniopharyngiomas are locally invasive neoplasms, and they cause potential lifelong morbidity because of their tendency for local recurrence. Despite advancements in endoscopic techniques, gross-total resection (GTR) of tumors with invasion or adhesion to important surrounding anatomical structures is extremely difficult. The authors present a single-center study that evaluated the impact of the endoscopic endonasal approach (EEA) on the surgical outcomes of pediatric craniopharyngiomas, the factors affecting the resection rate, and recurrence. METHODS: A total of 44 pediatric patients (age ≤ 18 years) who were treated via the EEA for craniopharyngioma from August 1997 to June 2022, as well as their 53 operations, were included in this study. The preoperative radiological configuration and surgical data of these cases were assessed. Also, preoperative and postoperative clinical (endocrinologic, neurological, and ophthalmological), hypothalamic, physical and social development, and neurocognitive assessment data were described. RESULTS: In total, 37 cases (69.8%) had no history of operation beforehand. The most common symptoms at presentation were endocrine disturbances (98.1%), headache without vomiting (84.3%), and visual disturbance (51%). Cases were classified as infrasellar (1.9%), sellar (32.1%), sellar-suprasellar (52.8%), and suprasellar (13.2%) localization. GTR was achieved in 34/53 cases (64.1%). The rate of GTR was higher in infrasellar and sellar tumors compared with sellar-suprasellar and suprasellar tumors (p = 0.003), and preoperative hypothalamic involvement was associated with lower likelihood of GTR (p = 0.024). Moreover, with experience, the rate of GTR increased (p = 0.037). Postoperative complications, other than endocrine impairment, occurred in 10/53 cases (18.9%). The mean duration of follow-up was 53.57 months. At follow-up, 21/53 (39.6%) cases presented with tumor recurrence. The 5-year progression-free survival (PFS) rate was 48.5%. There was a statistically significant difference between the GTR and other-than-GTR groups in terms of PFS (p < 0.001). According to univariate analysis, smaller tumor (p = 0.017), infrasellar and sellar localization (p = 0.031), and GTR (p < 0.001) were significantly associated with decreased rate of recurrence. Also, there was a statistically significant association between the recurrence rate and adhesion strength of the tumor (p < 0.001). CONCLUSIONS: This retrospective cohort study revealed surgical indications for EEA, as well as factors affecting the resection rate, recurrence, and quality of life during the follow-up period of the included cases. The authors believe that GTR should be the goal for craniopharyngioma treatment, but the authors' treatment approach was to provide a balance between radical surgery with maximum safety and adjuvant treatment for long-term disease control.