RESUMEN
We report the case of a 63-year-old man with a midline posterior fossa tumor and peculiar imaging features where we were unsure of the nature of the lesion preoperatively. Histopathology revealed it to be a craniopharyngioma. It appeared to arise from the inferior medullary velum, a site not described before in the literature. The previous four cases mentioned in literature and speculations on the origins in this uncommon site are discussed.
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Craneofaringioma , Imagen por Resonancia Magnética , Humanos , Craneofaringioma/cirugía , Craneofaringioma/patología , Craneofaringioma/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Neoplasias Infratentoriales/cirugía , Neoplasias Infratentoriales/patología , Neoplasias Infratentoriales/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Fosa Craneal Posterior/patología , Fosa Craneal Posterior/diagnóstico por imagen , Fosa Craneal Posterior/cirugía , Bulbo Raquídeo/patología , Bulbo Raquídeo/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
A female, in her 60s, presented with pain and swelling of the right eye for 3 years. The radiological work-up revealed an extraconal solid-cystic orbital tumour suggestive of an epidermoid cyst. The patient underwent supraorbital craniotomy with a gross total excision of the tumour. An intraoperative diagnosis was sought, which on both squash smear and frozen section showed features of craniopharyngioma (CP), later confirmed on paraffin sections and immunohistochemistry. The orbit is a very rare site for ectopic CP, with only two cases reported in the literature. Many theories have been proposed to explain the occurrence of CP at ectopic sites. This report aims to provide insight into the different hypotheses of the pathogenesis of ectopic CP through a review of the literature.
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Craneofaringioma , Neoplasias Orbitales , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/diagnóstico , Femenino , Neoplasias Orbitales/cirugía , Neoplasias Orbitales/diagnóstico por imagen , Neoplasias Orbitales/diagnóstico , Neoplasias Orbitales/patología , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/diagnóstico , Persona de Mediana Edad , Craneotomía , Imagen por Resonancia Magnética , Diagnóstico DiferencialRESUMEN
Introduction: Craniopharyngiomas (CPs) are benign brain tumors accounting for 5 - 11% of intracranial tumors in children. These tumors often recur and can cause severe morbidity. Postoperative radiotherapy efficiently controls and prevents progression and recurrence. Despite advancements in neurosurgery, endocrinological, visual, and neuropsychological complications are common and significantly lower the quality of life of patients. Methods: We performed a retrospective study, including all patients younger than sixteen diagnosed with CP between July 1989 and August 2022 and followed up in Hôpital Universitaire de Bruxelles. Results: Nineteen children with CP were included, with median age of 7 years at first symptoms and 7.5 at diagnosis. Common symptoms at diagnosis were increased intracranial pressure (63%), visual impairment (47%), growth failure (26%), polyuria/polydipsia (16%), and weight gain (10.5%). As clinical signs at diagnosis, growth failure was observed in 11/18 patients, starting with a median lag of 1 year and 4 months before diagnosis. On ophthalmological examination, 27% of patients had papillary edema and 79% had visual impairment. When visual disturbances were found, the average preoperative volume was higher (p=0.039). Only 6/19 patients had gross total surgical resection. After the first neurosurgery, 83% experienced tumor recurrence or progression at a median time of 22 months. Eleven patients (73%) underwent postsurgical radiotherapy. At diagnosis, growth hormone deficiency (GHD) was the most frequent endocrine deficit (8/17) and one year post surgery, AVP deficiency was the most frequent deficit (14/17). Obesity was present in 13% of patients at diagnosis, and in 40% six months after surgery. There was no significant change in body mass index over time (p=0.273) after the first six months post-surgery. Conclusion: CP is a challenging brain tumor that requires multimodal therapy and lifelong multidisciplinary follow-up including hormonal substitution therapy. Early recognition of symptoms is crucial for prompt surgical management. The management of long-term sequelae and morbidity are crucial parts of the clinical path of the patients. The results of this study highlight the fundamental importance of carrying out a complete assessment (ophthalmological, endocrinological, neurocognitive) at the time of diagnosis and during follow-up so that patients can benefit from the best possible care.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Niño , Estudios Retrospectivos , Femenino , Masculino , Preescolar , Adolescente , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Estudios de Seguimiento , Calidad de VidaRESUMEN
Surgical selection for third ventricle tumors demands meticulous planning, given the complex anatomic milieu. Traditional open microsurgical approaches may be limited in their access to certain tumors, prompting the exploration of alternative techniques. The endoscopic supraorbital translaminar approach (ESOTLA) has emerged as a promising alternative for managing these tumors. By combining a minimally invasive keyhole approach with endoscopic visualization, the ESOTLA provides enhanced illumination and a wider angle of view within the third ventricle. This unique advantage allows for improved access to retrochiasmatic tumors and reduces the need for frontal lobe and optic chiasm retraction required of microscopic techniques, decreasing the risk of neurocognitive and visual deficits. Complications related to the ESOTLA are rare and primarily pertain to cosmetic issues and potential compromise of the hypothalamus or optic apparatus, which can be minimized through careful subarachnoid dissection. This chapter offers a comprehensive description of the technical aspects of the ESOTLA, providing insights into its application, advantages, and potential limitations. Additionally, a case description highlights the successful surgical extirpation of an intraventricular papillary craniopharyngioma via the ESOTLA followed by targeted therapy. To better illustrate the stepwise dissection through this novel approach, a series of cadaveric and intraoperative photographs are included.
Asunto(s)
Neuroendoscopía , Humanos , Neoplasias del Ventrículo Cerebral/cirugía , Craneofaringioma/cirugía , Neuroendoscopía/métodos , Procedimientos Neuroquirúrgicos/métodos , Órbita/cirugía , Neoplasias Hipofisarias/cirugía , Tercer Ventrículo/cirugíaRESUMEN
OBJECTIVE: Central diabetes insipidus or vasopressin deficiency (AVP-D) is the most frequent water balance disorder after transsphenoidal surgery (TSS) with variable prevalence amongst studies. We aimed to determine rates of newly developed transient or permanent AVP-D in patients with pituitary tumours treated with TSS. DESIGN AND METHODS: We performed systematic review of Medline, Embase, and Cochrane Library between January 1, 2000 and January 31, 2021 for studies reporting on outcomes for pituitary adenoma, craniopharyngioma, and Rathke's cleft cyst (RCC) after TSS and providing definition of post-operative AVP-D. We pooled the results as proportions with 95% confidence intervals (CIs) using Freeman-Tukey transformation random effects meta-analysis. RESULTS: From 11 694 studies, 51 were included. Rates of transient or permanent AVP-D were: 17% (95% CI, 13-21) and 3% (95% CI, 2-5) in total group, 16% (95% CI, 12-21) and 2% (95% CI, 2-3) in pituitary adenomas, 31% (95% CI, 24-39) and 30% (95% CI, 22-39) in craniopharyngiomas, and 35% (95% CI, 16-57) and 14% (95% CI, 6-23) in RCCs, respectively. Based on diagnostic criteria, rates of transient or permanent AVP-D were: For hypotonic polyuria, 14% (95% CI, 8-22) and 3% (95% CI, 1-4), for hypotonic polyuria and hypernatraemia, 21% (95% CI, 13-29) and 5% (95% CI, 2-11), and for desmopressin administration, 22% (95% CI, 15-29) and 9% (95% CI, 0-30), respectively. CONCLUSIONS: Following TSS, a small proportion of patients with pituitary adenoma have permanent AVP-D (2%), but prevalence reaches 30% in ones with craniopharyngioma and 14% in those with RCC. Diagnostic criteria for post-operative AVP-D remain variable affecting reported rates of this condition.
Asunto(s)
Diabetes Insípida Neurogénica , Neoplasias Hipofisarias , Complicaciones Posoperatorias , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/epidemiología , Humanos , Diabetes Insípida Neurogénica/epidemiología , Diabetes Insípida Neurogénica/etiología , Diabetes Insípida Neurogénica/diagnóstico , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Craneofaringioma/cirugía , Vasopresinas/deficiencia , Adenoma/cirugía , Adenoma/epidemiología , Procedimientos Neuroquirúrgicos/efectos adversosRESUMEN
BACKGROUND: Various clinical classifications of craniopharyngiomas (CRPs) have been proposed to suggest optimal surgical planning. We aimed to evaluate the clinical outcomes of pediatric CRPs and the clinical significance of anatomical classification in relation to the diaphragm sellae. METHODS: A retrospective review was conducted on patients below 18 years of age who underwent surgery for CRPs from July 1998 to August 2022. The patients were divided into transcranial approach (TCA), and transsphenoidal approach (TSA) groups, which included microscopic TSA and endoscopic endonasal approach (EEA) groups. EEA has been adopted at our institute since 2011. CRPs were classified by their origin and relationship with the diaphragm sellae. RESULTS: A total of 132 pediatric CRP patients were included in this study, 117 of whom underwent surgery for primary CRP and 15 for recurrent CRP. Among them, 89 (67.4%) underwent TCA, 9 (6.8%) had microscopic TSA, and 34 (25.8%) had EEA. In subdiaphragmatic CRPs with competent diaphragm sellae, TSA tended to yield better outcomes than TCA did in terms of stalk preservation and ophthalmologic outcomes. After the introduction of EEA, the proportion of supradiaphragmatic CRPs treated via the TSA increased from 0% to 50% (P<0.001). Gross total resection (HR=0.194; 95% CI=0.102-0.367, P<0.001) and adjuvant therapy (HR=0.208; 95% CI=0.048-0.897, P=0.035) were found to be positive prognostic factors for long-term tumor control. CONCLUSIONS: Over time, with the adoption of EEA at our institute, the impact of anatomical classification on the surgical approach has decreased. Nevertheless, an individualized surgical approach should be employed to improve long-term outcomes and minimize complications for pediatric CRPs.
Asunto(s)
Craneofaringioma , Procedimientos Neuroquirúrgicos , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/diagnóstico por imagen , Craneofaringioma/patología , Femenino , Niño , Masculino , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Estudios Retrospectivos , Adolescente , Preescolar , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Neuroendoscopía/métodos , LactanteRESUMEN
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
BACKGROUND Craniopharyngioma is a rare, partly cystic embryonic malformation of the sellar and parasellar region and is usually benign. This report is of a 55-year-old woman presenting with a second diagnosis of craniopharyngioma following diagnosis and successful treatment of craniopharyngioma as a 5-year-old child. CASE REPORT Our patient was diagnosed with craniopharyngioma at age 5 when she presented with headaches accompanied by nausea and vomiting, decreased visual acuity, polyurea, and polydipsia for 6 months. She was found to have diplopia and grade II papilledema. A skull X-ray showed separation of the sutures and a calcified mass in the suprasellar region. A pneumoencephalogram showed extension of the tumor into the third ventricle. Surgery was performed via transcallosal approach followed by radiotherapy at 5000 rays. She was followed up clinically and radiologically and had been disease-free until age 55, when she presented with headache and facial numbness. On examination, she had right-eye Horner syndrome, decreased sensation in the right side of the face, diplopia, and grade 2 facial palsy. An MRI revealed interval significant recurrence of the craniopharyngioma at the sellar/suprasellar mass with extension to the right Meckel's cave and the right posterior fossa. On April 6, 2023, she underwent surgical resection through a right-sided craniotomy and Kawase approach. This was followed by CyberKnife radiation therapy. CONCLUSIONS This report has presented a rare recurrence of craniopharyngioma with a 50-year interval and has highlighted the challenges in the diagnosis and the multidisciplinary approach to patient diagnosis and management.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Humanos , Femenino , Craneofaringioma/diagnóstico , Craneofaringioma/cirugía , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/cirugía , Persona de Mediana Edad , Preescolar , Recurrencia Local de Neoplasia , Imagen por Resonancia MagnéticaRESUMEN
BACKGROUND: Cerebrospinal fluid leak after endoscopic skull base surgery remains a significant complication. Several investigators have suggested Hydroset cranioplasty to reduce leak rates. We investigated our early experience with Hydroset and compared the rate of nasal complications and CSF leak rates with case-controlled historic controls. METHODS: We queried a prospective database of patients undergoing first time endoscopic, endonasal resection of suprasellar meningiomas and craniopharyngiomas from 2015 to 2023. We compared cases closed with a gasket seal, Hydroset, and a nasoseptal flap with those closed with only a gasket seal and nasoseptal flap. Demographics, technical considerations and postoperative outcomes (SNOT-22) were compared. RESULTS: Seventy patients met inclusion criteria, twenty patients in the Hydroset group (meningioma n = 12; craniopharyngioma n = 8) and 50 control patients (meningioma n = 25; craniopharyngioma n = 25). CSF diversion was used in fewer Hydroset patients (75%, 15/20) compared with control group (94%, 47/50; p = 0.02). CSF leak was less frequent in the Hydroset than the control group (5% versus 12%, p = 0.38). One Hydroset patient required delayed nasal debridement. SNOT-22 responses demonstrated no significant difference in sinonasal complaints between groups (Hydroset average SNOT-22 score 22.45, control average SNOT-22 score 25.90; p = 0.58). CONCLUSIONS: We demonstrate that hydroxyapatite reconstruction leads to improved CSF leak control above that provided by the gasket-seal and nasoseptal flap, without significant associated morbidity as long as the cement is fully covered with vascularized tissue.
Asunto(s)
Pérdida de Líquido Cefalorraquídeo , Craneofaringioma , Meningioma , Base del Cráneo , Colgajos Quirúrgicos , Humanos , Masculino , Femenino , Persona de Mediana Edad , Pérdida de Líquido Cefalorraquídeo/prevención & control , Pérdida de Líquido Cefalorraquídeo/etiología , Pérdida de Líquido Cefalorraquídeo/cirugía , Estudios de Casos y Controles , Base del Cráneo/cirugía , Craneofaringioma/cirugía , Anciano , Meningioma/cirugía , Adulto , Neoplasias Hipofisarias/cirugía , Complicaciones Posoperatorias/prevención & control , Complicaciones Posoperatorias/etiología , Resultado del Tratamiento , Neoplasias de la Base del Cráneo/cirugía , Procedimientos de Cirugía Plástica/métodos , Neoplasias Meníngeas/cirugía , Tabique Nasal/cirugíaRESUMEN
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.
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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
INTRODUCTION: Disturbances in plasma sodium levels are a major complication following recent resections of craniopharyngiomas in children. They must be properly managed to avoid neurological sequelae. We aimed to describe the variations and characteristics of postoperative natremia in children who had undergone a first craniopharyngioma resection with a particular focus on the frequency of triphasic syndrome in these patients. METHODS: Paediatric patients with craniopharyngiomas who underwent a first surgical resection in the neurosurgery department of the Hôpital Femme Mère Enfant (Lyon, France) between January 2010 and September 2021 were included in the present study and the medical records were analysed retrospectively. RESULTS: A total of 26 patients were included. Of these, 17 (65.4%) had a postoperative course characterised by the occurrence of both initial diabetes insipidus (DI) and hyponatremia a few days later. Eight patients (30.8%) presented then with isolated and persistent DI. Patients with the triphasic syndrome had a significantly higher grade of Puget classification on MRI (1 and 2), compared to the other patients. CONCLUSION: Dysnatremia is common after craniopharyngioma resections in children. This immediate postoperative complication is particularly difficult to manage and requires rapid diagnosis and prompt initiation of medical treatment to minimize fluctuations in sodium levels and avoid neurological sequelae.
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Craneofaringioma , Neoplasias Hipofisarias , Complicaciones Posoperatorias , Sodio , Humanos , Craneofaringioma/cirugía , Craneofaringioma/sangre , Femenino , Masculino , Niño , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/sangre , Sodio/sangre , Francia/epidemiología , Complicaciones Posoperatorias/sangre , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/epidemiología , Adolescente , Preescolar , Estudios Retrospectivos , Estudios de Cohortes , Hiponatremia/etiología , Hiponatremia/sangre , Procedimientos Neuroquirúrgicos/efectos adversos , Diabetes Insípida/etiología , Diabetes Insípida/sangre , Diabetes Insípida/epidemiologíaRESUMEN
BACKGROUND: Postoperative central diabetes insipidus (CDI) is commonly observed in craniopharyngioma (CP) patients, and the inflammatory response plays an important role in CPs. We aimed to evaluate the predictive value of preoperative peripheral inflammatory markers and their combinations regarding CDI occurrence in CPs. METHODS: The clinical data including preoperative peripheral inflammatory markers of 208 CP patients who underwent surgical treatment were retrospectively collected and analyzed. The preoperative peripheral white blood cells (WBC), neutrophils, lymphocytes, monocytes, platelet (PLT), neutrophil-to-lymphocyte ratio (NLR), derived-NLR (dNLR), monocyte-to-lymphocyte ratio (MLR) and PLT-to-lymphocyte ratio (PLR) were assessed in total 208 CP patients and different age and surgical approach CP patient subgroups. Their predictive values were evaluated by the receiver operator characteristic curve analysis. RESULTS: Preoperative peripheral WBC, neutrophils, NLR, dNLR, MLR, and PLR were positively correlated and lymphocyte was negatively associated with postoperative CDI occurrence in CP patients, especially when WBC ≥ 6.66 × 109/L or lymphocyte ≤ 1.86 × 109/L. Meanwhile, multiple logistic regression analysis showed that WBC > 6.39 × 109/L in the > 18 yrs age patients, WBC > 6.88 × 109/L or lymphocytes ≤ 1.85 × 109/L in the transcranial approach patients were closely associated with the elevated incidence of postoperative CDI. Furthermore, the area under the curve obtained from the receiver operator characteristic curve analysis showed that the best predictors of inflammatory markers were the NLR in total CP patients, the MLR in the ≤ 18 yrs age group and the transsphenoidal group, the NLR in the > 18 yrs age group and the dNLR in the transcranial group. Notably, the combination index NLR + dNLR demonstrated the most valuable predictor in all groups. CONCLUSIONS: Preoperative peripheral inflammatory markers, especially WBC, lymphocytes and NLR + dNLR, are promising predictors of postoperative CDI in CPs.
Asunto(s)
Craneofaringioma , Diabetes Insípida Neurogénica , Neoplasias Hipofisarias , Complicaciones Posoperatorias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/sangre , Craneofaringioma/complicaciones , Femenino , Masculino , Estudios Retrospectivos , Adulto , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/sangre , Neoplasias Hipofisarias/complicaciones , Complicaciones Posoperatorias/sangre , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/diagnóstico , Adolescente , Persona de Mediana Edad , Niño , Adulto Joven , Diabetes Insípida Neurogénica/sangre , Diabetes Insípida Neurogénica/etiología , Neutrófilos , Biomarcadores/sangre , Linfocitos , Inflamación/sangre , Recuento de Leucocitos , Periodo Preoperatorio , Preescolar , Pronóstico , Curva ROCRESUMEN
BACKGROUND: Craniopharyngiomas (CPs) are generally derived from the craniopharyngeal duct epithelium, accounting for 38% and 24.5% of mortality in pediatric and adult patients, respectively. At present, the widespread application of the endoscopic endonasal transsphenoidal approach (EEA) has led to controversy between the traditional microscopic transcranial approach (TCA) and EEA in relation to the surgical management of CPs. OBJECT AND METHOD: We performed a systematic review and meta-analysis comparing the complications, surgical outcomes, and endocrine functions of patients with CPs to provide evidence-based decision-making in their surgical management. RESULT: Overall, 11 observational studies with 12,212 participants were included in the meta-analysis, in which five of them only included an adult population, three of them only included a child population, and the other three studies included a mixed population (adult and child). In pediatric patients, the EEA achieved a higher gross total resection (GTR) rate (odds ratio (OR) = 5.25, 95%CI: 1.21-22.74), lower recurrence rate (OR = 0.54, 95%CI: 0.31-0.94, p = 0.030), and less hypopituitarism (OR = 0.34, 95%CI: 0.12-0.97, p = 0.043). In adult patients, EEA significantly improved mortality (OR = 0.09, 95%CI: 0.06-0.15, p < 0.001) and visual outcomes (visual improvement: OR = 3.42, 95%CI: 1.24-9.40, p = 0.017; visual deficit: OR = 0.30, 95%CI: 0.26-0.35) with decreases in postoperative stroke (OR = 0.58, 95%CI: 0.51-0.66, p < 0.001), hydrocephalus, and infections (OR = 0.32, 95%CI: 0.24-0.42, p < 0.001). CONCLUSION: Compared with the traditional TCA in primary CP resection, the development and wide application of EEA optimistically decreased the recurrence rate of CP, alleviated hypopituitarism with improvement in the GTR rate of pediatric patients, and significantly improved the visual outcomes, hydrocephalus, postoperative stroke, survival, and infection rates of the patients. Therefore, EEA is an optimal approach for primary CP resection.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/patología , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Complicaciones Posoperatorias/etiología , Pronóstico , Resultado del Tratamiento , Recurrencia Local de Neoplasia/cirugía , Recurrencia Local de Neoplasia/patología , Endoscopía/métodosRESUMEN
PURPOSE: The aim of this study was to evaluate the treatment outcomes of neuroendoscopic cyst partial resection (ECPR) combined with stereotactic radiotherapy (SRT) for cystic craniopharyngiomas. METHODS: In this retrospective study, 22 craniopharyngioma patients undergoing ECPR combined with SRT were included. This combination therapy was indicated for suprasellar cystic craniopharyngiomas in patients whose pituitary function was preserved but would be difficult to preserve in direct surgery. The outcomes of combination therapy, including tumor control and postoperative visual and pituitary functions, were investigated. RESULTS: ECPR was safely performed, and cyst shrinkage was accomplished in all cases. After ECPR, visual function improved in 12 of 13 patients (92%) with visual field disturbance and did not deteriorate in any patients. Pituitary function was preserved in 14 patients (64%) and deteriorated in eight patients (36%) after ECPR. As a complication of ECPR, meningitis occurred because of a wound infection in one patient. In 18 of 22 patients (82%), the tumor was controlled without further treatment 19 - 87 months (median, 33 months) after SRT. Hypopituitarism was an adverse event after SRT in two of the 18 patients who achieved tumor control. Four patients (18%) had enlarged cysts after SRT. Postoperative pituitary function was significantly more likely to deteriorate in cases of extensive detachment from the ventricular wall, and retreatment was significantly more common in cases with hypothalamic extension. CONCLUSION: Although limited to some cases, ECPR combined with SRT is a less invasive and useful therapeutic option for suprasellar cystic craniopharyngiomas. However, its long-term prognosis requires further evaluation.
Asunto(s)
Craneofaringioma , Neuroendoscopía , Neoplasias Hipofisarias , Radiocirugia , Humanos , Craneofaringioma/cirugía , Craneofaringioma/radioterapia , Masculino , Femenino , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/radioterapia , Adulto , Persona de Mediana Edad , Radiocirugia/métodos , Radiocirugia/efectos adversos , Neuroendoscopía/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven , Adolescente , Niño , Quistes/cirugía , Anciano , Terapia Combinada/métodosRESUMEN
OBJECTIVE: Treatment of craniopharyngioma typically entails gross total resection (GTR) or subtotal resection with adjuvant radiation (STR-RT). We analyzed outcomes in adults with craniopharyngioma undergoing GTR versus STR-RT. METHODS: This retrospective study enrolled 115 patients with craniopharyngioma in 5 institutions. Patients with STR received postoperative RT with stereotactic radiosurgery or fractionated radiation therapy per institutional preference and ability to spare optic structures. RESULTS: Median age was 44 years (range, 19-79 years). GTR was performed in 34 patients and STR-RT was performed in 81 patients with median follow-up of 78.9 months (range, 1-268 months). For GTR, local control was 90.5% at 2 years, 87.2% at 3 years, and 71.9% at 5 years. For STR-RT, local control was 93.6% at 2 years, 90.3% at 3 years, and 88.4% at 5 years. At 5 years following resection, there was no difference in local control (P = 0.08). Differences in rates of visual deterioration or panhypopituitarism were not observed between GTR and STR-RT groups. There was no difference in local control in adamantinomatous and papillary craniopharyngioma regardless of treatment. Additionally, worse local control was found in patients receiving STR-RT who were underdosed with fractionated radiation therapy (P = 0.03) or stereotactic radiosurgery (P = 0.04). CONCLUSIONS: Good long-term control was achieved in adults with craniopharyngioma who underwent STR-RT or GTR with no significant difference in local control. First-line treatment for craniopharyngioma should continue to be maximal safe resection followed by RT as needed to balance optimal local control with long-term morbidity.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Radiocirugia , Humanos , Craneofaringioma/radioterapia , Craneofaringioma/cirugía , Adulto , Persona de Mediana Edad , Neoplasias Hipofisarias/radioterapia , Neoplasias Hipofisarias/cirugía , Femenino , Masculino , Estudios Retrospectivos , Anciano , Adulto Joven , Resultado del Tratamiento , Radiocirugia/métodos , Radioterapia Adyuvante/métodos , Procedimientos Neuroquirúrgicos/métodos , Estudios de SeguimientoRESUMEN
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
OBJECTIVE: Tumors located in the retrochiasmatic region with extension to the third ventricle might be difficult to access when the pituitary-chiasmatic corridor is narrow. Similarly, tumor extension into the interpeduncular and retrosellar space poses a major surgical challenge. Pituitary transposition techniques have been developed to gain additional access. However, when preoperative pituitary function is already impaired or the risk of postoperative panhypopituitarism (PH) is considered to be particularly high, removal of the pituitary gland (PG) might be the preferred option to increase the working corridor. The aim of this study was to describe the relevant surgical anatomy, operative steps, and clinical experience with the endoscopic endonasal pituitary sacrifice (EEPS) and transsellar approach. METHODS: This study comprised anatomical dissections to highlight the relevant surgical steps and a retrospective case series reporting clinical characteristics, indications, and outcomes of patients who underwent EEPS. The surgical technique is as follows: both lateral opticocarotid recesses are exposed laterally, the limbus sphenoidale superiorly, and the sellar floor inferiorly. After opening the dura, the PG is detached circumferentially and mobilized off the medial walls of the cavernous sinuses. The descending branches of the superior hypophyseal artery are coagulated, and the stalk is transected. After removal of the PG, drilling of the dorsum sellae and bilateral posterior clinoidectomies are performed to gain access to the hypothalamic region, interpeduncular, and prepontine cisterns. RESULTS: From 2018 to 2023, 11 patients underwent EEPS. The cohort comprised mostly tuberoinfundibular craniopharyngiomas (n = 8, 73%). Seven (64%) patients had partial or complete anterior PG dysfunction preoperatively, while 4 (36%) had preoperative diabetes insipidus. Because of the specific tumor configuration, the chance of preserving endocrine function was estimated to be very low in patients with intact function. The main reasons for pituitary sacrifice were impaired visibility and surgical accessibility to the retrochiasmatic and retrosellar spaces. Gross-total tumor resection was achieved in 10 (91%) patients and near-total resection in 1 (9%) patient. Two (18%) patients experienced a postoperative CSF leak, requiring surgical revision. CONCLUSIONS: When preoperative pituitary function is already impaired or the risk for postoperative PH is considered particularly high, the EEPS and transsellar approach appears to be a feasible surgical option to improve visibility and accessibility to the retrochiasmatic hypothalamic and retrosellar spaces, thus increasing tumor resectability.
Asunto(s)
Hipófisis , Neoplasias Hipofisarias , Humanos , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Masculino , Femenino , Persona de Mediana Edad , Adulto , Estudios Retrospectivos , Hipófisis/cirugía , Hipófisis/patología , Hipófisis/anatomía & histología , Anciano , Neuroendoscopía/métodos , Procedimientos Neuroquirúrgicos/métodos , Quiasma Óptico/cirugía , Quiasma Óptico/patología , Adulto Joven , Craneofaringioma/cirugía , Craneofaringioma/patología , Silla Turca/cirugía , Silla Turca/patología , Cirugía Endoscópica por Orificios Naturales/métodos , Resultado del Tratamiento , Adenoma/cirugía , Adenoma/patologíaRESUMEN
Background: Craniopharyngioma (CP), although slow growing and histologically benign, has high morbidity, mostly related to hypothalamus-pituitary dysfunction and electrolyte imbalance. Increased risk of vascular complications has been described. However, data are still poor, especially in the paediatric population. The aim of our study was to evaluate the occurrence, timing, and predisposing factors of deep venous thrombosis (DVT) and other vascular alterations in neurosurgical paediatric CP patients. Materials and Methods: In a single-centre, retrospective study, we investigated 19 CP patients (11 males, 8 females, mean age 10.5 ± 4.3 years), who underwent neurosurgery between December 2016 and August 2022, referred to Meyer Children's Hospital IRCCS in Florence. Results: Five patients (26.3%) presented vascular events, which all occurred in connection with sodium imbalances. Three DVT (two with associated pulmonary embolism, in one case leading to death) developed in the post-operative period, most frequently at 7-10 days. Elevated D-dimers, a reduced partial activated thrombin time and a prolonged C-reactive protein increase were highly related to thrombotic vascular events. One case of posterior cerebral artery pseudoaneurysm was described soon after neurosurgery, requiring vascular stenting. Superficial vein thrombophlebitis was a late complication in one patient with other predisposing factors. Conclusion: CP patients undergoing neurosurgery are at risk of developing DVT and vascular alterations, thus careful follow-up is mandatory. In our study, we found that the phase of transition from central diabetes insipidus to a syndrome of inappropriate antidiuretic hormone secretion may be a period of significant risk for DVT occurrence. Careful vascular follow-up is mandatory in CP-operated patients.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Complicaciones Posoperatorias , Humanos , Craneofaringioma/cirugía , Craneofaringioma/complicaciones , Femenino , Masculino , Niño , Estudios Retrospectivos , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/complicaciones , Adolescente , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/epidemiología , Trombosis de la Vena/etiología , Trombosis de la Vena/epidemiología , Procedimientos Neuroquirúrgicos/efectos adversos , Preescolar , Enfermedades Vasculares/etiología , Enfermedades Vasculares/epidemiología , Enfermedades Vasculares/patología , Estudios de SeguimientoRESUMEN
BACKGROUND: Craniopharyngioma is a common intracranial tumour in children. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Defects in pituitary function cause shortages of growth hormone, gonadotropin, corticotropin, thyrotropin, and vasopressin, resulting in short stature, delayed puberty, feebleness, lethargy, polyuria, etc. However, manifestations involving precocious puberty (PP) are rare. CASE REPORT: In both patients, surgical resection was performed after the diagnosis of craniopharyngioma, and breast development occurred postoperatively at one month in one patient and at one year and three months in the other patient. Central precocious puberty (CPP) was diagnosed via relevant examinations. Leuprorelin was injected subcutaneously every 28 days, and changes in height, weight, bone age, gonadal ultrasound and sex hormones were recorded. During the follow-up of the two children, the sex hormone levels were significantly reduced, and significant acceleration in bone age was not observed. CONCLUSIONS: CPP was induced by craniopharyngioma surgery, and treatment with gonadotropin-releasing hormone analogues (GnRHa) inhibited sexual development and bone age progression. More attention should be given to monitoring for CPP during long-term follow-up of craniopharyngiomas in the clinic.
Asunto(s)
Craneofaringioma , Neoplasias Hipofisarias , Pubertad Precoz , Humanos , Craneofaringioma/cirugía , Craneofaringioma/complicaciones , Leuprolida/uso terapéutico , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/complicaciones , Complicaciones Posoperatorias/etiología , Pubertad Precoz/etiologíaRESUMEN
BACKGROUND: Surgical resection is the most effective method for craniopharyngioma, with complex operations and a high incidence of complications, especially for complex craniopharyngioma. The study focuses on selecting a proper surgical method to treat complex craniopharyngioma. A clinical study was conducted in this direction to explore the efficacy of expanded endoscopic endonasal transsphenoidal approach (EETS) and transcranial approach (TCA) in the treatment of complex craniopharyngioma and their effects on pituitary function and complications of patients. METHODS: The clinical data of 73 patients with complex craniopharyngioma in Baoding No.2 Central Hospital from December 2017 to December 2022 were retrospectively analyzed. 13 patients who did not meet the admission criteria were excluded, and 60 patients were finally included. The included patients were divided into the TCA and EETS groups according to the treatment method. The surgical conditions, total tumor resection rate, clinical remission rate, and complications of patients in two groups were compared. The pituitary function of all patients, including thyroid stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), and human growth hormone (hGH), was compared. RESULTS: A total of 60 patients were finally included in the study, including 30 cases in the EETS group and 30 cases in the TCA group. The two groups had no statistical difference in baseline demographic characteristics and pathological types (p > 0.05). Compared with the TCA group, the EETS group had less intraoperative blood loss, operation time, tumor resection time, and hospitalization time (p < 0.001) and had a significantly higher total tumor resection rate and clinical remission rate (p < 0.05). The EETS group had higher ACTH and hGH levels than the TCA group (p < 0.05). There was no statistical difference between the two groups incidence of complications and disease recurrence rate (p > 0.05). CONCLUSION: The craniopharyngioma resection via EETS has a significant therapeutic effect in complex craniopharyngioma with a high total tumor resection rate and clinical remission rate, which can protect the pituitary function of patients and provide more benefits for patients.