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1.
Front Endocrinol (Lausanne) ; 13: 958295, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36120435

RESUMO

Objective: To investigate the relationship between postoperative hypothalamo-hypophyseal injury (HHI) and postoperative water and sodium disturbances in patients with craniopharyngioma. Methods: The medical records, radiological data, and laboratory results of 178 patients (44 children and 134 adults) who underwent microsurgery for craniopharyngioma in a single center were reviewed. Postoperative HHI was assessed using magnetic resonance imaging. Structural defects of the hypothalamo-hypophyseal system (pituitary, pituitary stalk, floor and lateral wall of the third ventricle) were assessed in four standard T1-weighted images. The defect of each structure was assigned 1 score (0.5 for the unilateral injury of the third ventricle wall), and a HHI score was calculated. Results: The number of patients with HHI scores of 0-1, 2, 2.5-3, and >3 was 35, 49, 61, and 33, respectively. Diabetes insipidus (DI) worsened in 56 (31.5%) patients with preoperative DI, while 119 (66.9%) patients were diagnosed with new-onset DI. Hypernatremia and hyponatremia developed in 127 (71.3%) and 128 (71.9%) patients after surgery, respectively. Syndrome of inappropriate antidiuresis occurred in 97(54.5%) patients. During hospitalization, hypernatremia recurred in 33 (18.5%) patients and in 54 (35.7%) during follow-up, of which 18 (11.9%) were severe. DI persisted in 140 (78.7%) patients before discharge. No relationship was found between the HHI score and incidence of early DI, hyponatremia, syndrome of inappropriate diuretic hormone, or prolonged DI. Compared with patients with a score of 0-1, those with scores =2.5-3 (OR = 5.289, 95% CI:1.098-25.477, P = 0.038) and >3 (OR = 10.815, 95% CI:2.148-54.457, P = 0.004) had higher risk of developing recurrent hypernatremia. Patients with a score >3 had higher risk of developing severe hypernatremia during hospitalization (OR = 15.487, 95% CI:1.852-129.539, P = 0.011) and at follow-up (OR = 28.637, 95% CI:3.060-267.981, P = 0.003). Conclusions: The neuroimaging scoring scale is a simple tool to semi-quantify HHI after surgery. Recurrent and severe hypernatremia should be considered in patients with a high HHI score (>2.5). An HHI score >3 is a potential predictor of adipsic DI development. Preventive efforts should be implemented in the perioperative period to reduce the incidence of potentially catastrophic complications.


Assuntos
Lesões Encefálicas Traumáticas , Craniofaringioma , Diabetes Insípido , Hipernatremia , Hiponatremia , Neoplasias Hipofisárias , Adulto , Lesões Encefálicas Traumáticas/complicações , Criança , Craniofaringioma/complicações , Craniofaringioma/cirurgia , Diabetes Insípido/complicações , Diuréticos , Hormônios , Humanos , Hipernatremia/epidemiologia , Hipernatremia/etiologia , Hiponatremia/epidemiologia , Hiponatremia/etiologia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Sódio , Água
2.
Medicine (Baltimore) ; 101(37): e30278, 2022 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-36123899

RESUMO

BACKGROUND: Adamantinomatous craniopharyngioma (ACP) is a subtype of craniopharyngioma, a neoplastic disease with a benign pathological phenotype but a poor prognosis in the sellar region. The disease has been considered the most common congenital tumor in the skull. Therefore, this article aims to identify hub genes that might serve as genetic markers of diagnosis, treatment, and prognosis of ACP. METHODS: The procedure of this research includes the acquisition of public data, identification and functional annotation of differentially expressed genes (DEGs), construction and analysis of protein-protein interaction network, and the mining and analysis of hub genes by Spearman-rho test, multivariable linear regression, and receiver operator characteristic curve analysis. Quantitative real-time polymerase chain reaction was used to detect the level of mRNA of relative genes. RESULTS: Among 2 datasets, a total of 703 DEGs were identified, mainly enriched in chemical synaptic transmission, cell adhesion, odontogenesis of the dentin-containing tooth, cell junction, extracellular region, extracellular space, structural molecule activity, and structural constituent of cytoskeleton. The protein-protein interaction network was composed of 4379 edges and 589 nodes. Its significant module had 10 hub genes, and SYN1, SYP, and GRIA2 were significantly down-regulated with ACP. CONCLUSION: In a word, we find out the DEGs between ACP patients and standard samples, which are likely to play an essential role in the development of ACP. At the same time, these DEGs are of great value in tumors' diagnosis and targeted therapy and could even be mined as biological molecular targets for diagnosing and treating ACP patients.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Biologia Computacional/métodos , Craniofaringioma/diagnóstico , Craniofaringioma/genética , Craniofaringioma/terapia , Diagnóstico Precoce , Perfilação da Expressão Gênica/métodos , Redes Reguladoras de Genes , Marcadores Genéticos , Humanos , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/terapia , RNA Mensageiro
3.
Probl Endokrinol (Mosk) ; 68(4): 40-45, 2022 Jun 06.
Artigo em Russo | MEDLINE | ID: mdl-36104965

RESUMO

Presented case demonstrates a rare diencephalic pathology - adipsic diabetes insipidus (ADI) with severe hypernatremia in a 58-year-old woman after ttranssphenoidal removal of stalk intraventricular craniopharyngioma. ADI was diagnosed because of hypernatremia (150-155 mmol/L), polyuria (up to 4 liters per day) and absence of thirst. Normalization of water-electrolyte balance occurred on the background of desmopressin therapy and sufficient hydration in postoperative period. After release from the hospital, the patient independently stopped desmopressin therapy and did not consume an adequate amount of fluid of the background of polyuria. This led to severe hypernatremia (155-160 mmol/L) and rough mental disorders.Patients with ADI need closely monitoring of medical condition and water-electrolyte parameters, appointment of fixed doses of desmopressin and adequate hydration.


Assuntos
Diabetes Insípido , Hipernatremia , Procedimentos Neurocirúrgicos , Complicações Pós-Operatórias , Cistos do Sistema Nervoso Central/cirurgia , Craniofaringioma/cirurgia , Desamino Arginina Vasopressina , Diabetes Insípido/diagnóstico , Diabetes Insípido/tratamento farmacológico , Diabetes Insípido/etiologia , Diabetes Mellitus , Feminino , Humanos , Hipernatremia/complicações , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/efeitos adversos , Procedimentos Neurocirúrgicos/métodos , Poliúria/etiologia
4.
Front Immunol ; 13: 949509, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36091021

RESUMO

Craniopharyngiomas (CPs) are histologically benign tumors located in the sellar-suprasellar region. Although the transcriptome development in recent years have deepened our knowledge to the tumorigenesis process of adamantinomatous craniopharyngioma (ACP), the peritumoral immune infiltration of tumor is still not well understood. In this study, weighted gene coexpression network analysis (WGCNA) was applied to identify different gene modules based on clinical characteristics and gene expression, and then, the protein-protein interaction (PPI) network with the Cytohubba plug-in were performed to screen pivotal genes. In addition, immune cell infiltration (ICI) analysis was used to evaluate the immune microenvironment of ACP patients. In total, 8,568 differential expression genes were identified based on our datasets and two microarray profiles from the public database. The functional enrichment analysis revealed that upregulated genes were mainly enriched in immune-related pathways while downregulated genes were shown in the hormone and transduction of signaling pathways. The WGCNA investigated the most relevant modules, and 1,858 hub genes was detected, from which the PPI network identified 14 pivotal genes, and the Hypoxia-inducible factor 1-alpha (HIF-1α) pathway including four critical genes may be involved in the development of ACP. Moreover, naïve CD4+ and CD8+ T cells were decreased while specific subtypes of T cells were significantly increased in ACP patients according to ICI analysis. Validation by immunofluorescence staining revealed a higher expression of HIF-1α in ACP (ACP vs. control) and adult-subtype (adult vs. children), suggesting a possible state of immune system activation. Notably, children with low HIF-1α scores were related to the hypothalamus involvement and hydrocephalus symptoms. In this study, we successfully identified HIF-1α as a key role in the tumorigenesis and development of ACP through comprehensive integrated analyses and systematically investigated the potential relationship with immune cells in ACP. The results may provide valuable resources for understanding the underlying mechanisms of ACP and strengthen HIF-1α as a potential immunotherapeutic target in clinical application.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Carcinogênese , Criança , Craniofaringioma/diagnóstico , Craniofaringioma/genética , Craniofaringioma/metabolismo , Redes Reguladoras de Genes , Humanos , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/metabolismo , Mapas de Interação de Proteínas , Microambiente Tumoral/genética
5.
J Neurooncol ; 159(3): 597-607, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35925530

RESUMO

INTRODUCTION: Children with craniopharyngiomas (CP) can experience significant morbidities caused by extensive surgery and/or radiation. Ommaya reservoir insertion (ORI) into cystic CP represents a minimally invasive approach allowing immediate decompression and aims to avoid additional injuries. The purpose of this study was to determine the surgical outcome and relevance of upfront ORI (± intracystic treatment) for preservation of endocrine function. METHODS: We performed a retrospective chart review of children with CP treated at the Hospital for Sick Children between 01/01/2000 and 15/01/2020. Endocrine function was reviewed at the time of initial surgery and throughout follow-up. New endocrinological deficits related to the index procedure were defined as immediate failure (IF), whereas postoperative duration of endocrinological stability (ES) was analyzed using the Kaplan-Meier method. The rate of IF and ES was compared between the treatment groups. RESULTS: Seventy-nine patients were included and had a median age of 8.3 years (range 2.1-18.0 years); 31 were males. Fifty-three patients with upfront surgical treatment, including 29 ORI and 24 gross total or partial resections had sufficient endocrinological follow-up data. Endocrine dysfunction occurring immediately after the index procedure (IF) was observed in 15 patients (62.5%) in the resection group compared to two patients (6.8%) in the ORI group, odds ratio: 0.05 (CI: 0.01-0.26, p < 0.0001). Excluding those with immediate endocrinological deficits, mean ES after ORI was 19.4 months (CI: 11.6-34.2), compared to 13.4 months (CI:10.6-NA) after surgical resection. CONCLUSIONS: Endocrine function was preserved in patients with upfront ORI (± intracystic treatment), which was confirmed as a minimally invasive procedure with an overall low morbidity profile.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Adolescente , Criança , Pré-Escolar , Craniofaringioma/cirurgia , Feminino , Humanos , Masculino , Neoplasias Hipofisárias/radioterapia , Neoplasias Hipofisárias/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
6.
J Neurooncol ; 159(3): 713-723, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35987949

RESUMO

PURPOSE: Children diagnosed with craniopharyngioma are vulnerable to adverse health outcomes. Characterization of body mass index (BMI), physical function, and cardiopulmonary fitness in those treated with proton radiotherapy (PRT) will serve to design interventions to improve outcomes. METHODS: Ninety-four children with craniopharyngioma completed physical function testing prior to PRT and annually for 5 years. For each outcome, age- and sex-specific z-scores were calculated using normative values. Participants with z-scores > 1.5 or < - 1.5 were classified as impaired. Those with z-scores > 2.0 or < - 2.0 were classified as significantly impaired. Descriptive statistics were used to describe study outcomes and change in prevalence of impairments from 2 to 5 years after treatment. RESULTS: Nearly half of participants [45.2%, 95% confidence interval (CI) 39.4, 51.0] had mean BMI z-scores > 1.5 at baseline, with prevalence increasing to 66.7% (95% CI 61.5, 71.9) at 5 years. More than half of participants (54.2%, 95% CI 48.4, 60.0) had knee extension strength z-scores < - 1.5 at baseline, with prevalence increasing to 81.3% (95% CI 77.7, 84.9) at 5 years. BMI and knee extension strength had the largest proportion of participants impaired at both 2 and 5 years (53.2% and 62.3%, respectively). Resting heart rate had the highest proportion of participants not impaired at 2 years but became impaired at 5 years (26.6%). CONCLUSIONS: Children with craniopharyngioma have BMI and fitness abnormalities at diagnosis and continue 5 years after treatment. This cohort may benefit from interventions designed to improve BMI, strength, and resting indicators of cardiopulmonary fitness.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Terapia com Prótons , Adolescente , Índice de Massa Corporal , Criança , Craniofaringioma/radioterapia , Feminino , Seguimentos , Humanos , Masculino , Neoplasias Hipofisárias/radioterapia , Prótons
7.
Neurosurg Rev ; 45(5): 3361-3379, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35982344

RESUMO

This study describes and characterizes a narrow, hollow tubular structure, termed as duct-like diverticulum (DV), found specifically at the basal midline of papillary craniopharyngiomas (PCPs) located within the third ventricle (3V). The presence of this structure was systematically investigated on autopsy studies and magnetic resonance imaging (MRI) scans of 3536 craniopharyngioma (CP) cases published in the medical literature from 1911 to 2021, as well as in other twelve 3V tumor categories (n = 1470 cases). A basal DV was observed in a total of 50 PCPs, including two of our own cases. This DV corresponds to a tubular-shaped recess invaginated at the midline bottom of the tumor, following the same angled trajectory as the pituitary stalk. It can be easily seen as a hypointense linear structure on T1- and T2-weighted MRI scans, with two main length types: long DVs (74%), which reach the tumor center, and short DVs (26%), which penetrate the tumor only a few millimeters. The DV sign identifies the papillary CP type with a specificity of 100% and a sensitivity of 33% in the overall CP population. This finding also serves to establish the strictly intra-3V location of the lesion with a 95% specificity and 42% sensitivity among papillary CPs. No similar basal DV was found in adamantinomatous CPs nor among other categories of strictly 3V tumors. Consequently, the presence of a diverticulum in a 3V tumor represents a morphological signature pathognomonic of the papillary type and a valuable sign to reliably define the strictly 3V topography.


Assuntos
Craniofaringioma , Divertículo , Neoplasias Hipofisárias , Terceiro Ventrículo , Craniofaringioma/diagnóstico por imagem , Craniofaringioma/cirurgia , Divertículo/diagnóstico por imagem , Divertículo/cirurgia , Humanos , Hipófise , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/cirurgia , Terceiro Ventrículo/diagnóstico por imagem , Terceiro Ventrículo/patologia , Terceiro Ventrículo/cirurgia
8.
J Neurooncol ; 159(2): 485-497, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35939144

RESUMO

PURPOSE: Identifying relationships between craniopharyngiomas (CPs) and contiguous structures, and tumor origin are crucial for treatments. This study attempted to explore the relationships and tumor origin. METHODS: CPs that underwent endoscopic surgeries were enrolled. The interfacial specimens of CPs attaching the hypothalamus, pituitary stalk (PS), pituitary grand (PG), optic chiasma (OC) and brain tissue (BT) were pathologically examined. Boundaries between CPs and these structures were observed during operations. Expression of ß-catenin and stem cell markers were analyzed to explore the tumor origin. Outcomes of patients were assessed. RESULTS: A total of 34 CPs were categorized into two groups based on the locations of finger-like protrusions (FP). Group A comprised 18 CPs with FP only present in the specimens attaching to hypothalamus. The surface of these CPs was fused with hypothalamus under endoscopic videos. However, the specimens attaching to the PS, PG, OC, and BT showed no FP. Clear boundaries was observed between these CPs and these structures. Group B comprised 16 CPs with FP only present in the specimens attaching to PS. The tumor surface was fused with PS. Specimens attaching to the hypothalamus, PG, OC and BT showed no FP. Clear boundary was observed among these CPs with these structures. These results implied CPs only invaded a certain part of hypothalamic-pituitary axis. ß-catenin and stem cells markers mainly distributed in the FP tissues of both groups. Patients in group B achieved better outcomes than group A. CONCLUSIONS: CPs only invade the hypothalamic-pituitary axis with FP and the FP would be the tumor origin.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Humanos , Hipotálamo , Hipófise , beta Catenina
9.
Anticancer Res ; 42(9): 4189-4197, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36039438

RESUMO

BACKGROUND/AIM: Craniopharyngiomas involving the ventricular system are rare but pose significant surgical challenges. We systematically reviewed the literature on craniopharyngiomas invading the ventricles (CP-V). MATERIALS AND METHODS: PubMed, EMBASE, Scopus, Web of Science, and Cochrane were searched to include studies reporting clinical data of patients with CP-Vs. Clinico-radiological features, management, and treatment outcomes were analyzed. RESULTS: We included 73 studies encompassing 407 patients. Patients were mostly male (61.5%), presenting with headache (57.9%) and/or endocrine disorders (52.1%). CP-Vs mostly involved the third ventricle (96.3%), followed by the lateral ventricles (2.9%), and the fourth ventricle (1%). Tumors had cystic components in 59% of cases and were mostly adamantinomatous (70.8%). Open resection was performed in 232 cases (57%), mostly with trans-lamina terminalis (36.6%) and trans-callosal (31.9%) approaches. Endoscopic resection was performed in 169 cases (41.5%), mostly with trans-sphenoidal (74.6%) and transventricular (24.9%) approaches. Gross-total tumor resection was obtained in most cases (62.9%). Adjuvant radiotherapy was delivered in 22.8% cases. A total of 178 patients experienced persistent complications, mostly including diabetes insipidus (47.1%) and panhypopituitarism (12.7%), not significantly different after open versus endoscopic resection (p=0.117). Symptom improvement was obtained in 88% of cases. CP-Vs recurrences were reported in 94 patients (23.1%), with median progression-free survival of 13.5 months (range=0.5-252.0 months). Fifty-nine patients died (14.5%), with median overall survival of 32.0 months (range=0.5-252.0 months), significantly longer after endoscopic resection than open resection (p=0.019). CONCLUSION: CP-Vs are uncommon and challenging entities. Surgical resection is feasible, but patient-tailored selection of open/endoscopic approaches is necessary to achieve optimal outcomes and minimize complication risks.


Assuntos
Craniofaringioma , Hipopituitarismo , Neoplasias Hipofisárias , Craniofaringioma/cirurgia , Feminino , Humanos , Hipopituitarismo/complicações , Masculino , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
10.
Adv Tech Stand Neurosurg ; 45: 97-137, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35976448

RESUMO

Taking into account the benign nature of craniopharyngiomas, the main method of treatment is the resection of the tumor. However, the tendency of these tumors to invade critical structures (such as optic pathways, the hypothalamic-pituitary system, the Willis circle vessels) often limits the possibility of a radical surgery.Craniopharyngiomas of the third ventricle represent the greatest challenge for surgery. After radical surgery, hypothalamic disorders often occur, including not only obesity but also cognitive, emotional, mental, and metabolic disturbances. Metabolic disorders associated with damage to the hypothalamus progress after surgery and lead to impaired functions of the internal organs. This process is irreversible and, in many cases, becomes the direct cause of mortality. The life expectancy of patients with the surgically affected hypothalamus is significantly shorter than in patients with preserved diencephalic function. The incidence of hypothalamic disorders after surgery can reach 40%.Even with macroscopically total resection, craniopharyngiomas can recur in 10-30% of cases, and in the presence of tumor remnants and with no further radiation treatment, the risk of recurrence significantly increases to up to 50-85% according to various studies. For this reason, the observation of patients with residual tumors after surgery is an incorrect strategy.Radiation therapy significantly improves progression-free survival (PFS), and the use of stereotactic irradiation techniques ensures conformity of irradiation of tumor remnants with a complicated shape and location (Iwata H et al., J Neurooncol 106(3):571-577, 2012; Aggarwal et al., Pituitary 16(1):26-33, 2013; Savateev et al., Zh Vopr Neirokhir Im N N Burdenko 81(3):94-106; 2017), which potentially reduces the risk of undesirable postradiation effects. Therefore, the quality of life in patients with craniopharyngiomas infiltrating the hypothalamus is significantly higher after non-radical operations with subsequent stereotactic radiation than after a total or subtotal removal.


Assuntos
Craniofaringioma , Doenças Hipotalâmicas , Neoplasias Hipofisárias , Radiocirurgia , Craniofaringioma/radioterapia , Humanos , Doenças Hipotalâmicas/complicações , Neoplasias Hipofisárias/radioterapia , Qualidade de Vida , Radiocirurgia/métodos , Resultado do Tratamento
11.
Adv Tech Stand Neurosurg ; 45: 139-176, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35976449

RESUMO

In spite of the significant technical and technological progress in neurosurgery and the continuous discoveries by the basic research, adamantinomatous craniopharyngioma remains a significant clinical challenge. Actually, the huge size of the tumor, its multiple cystic components, the encasement of Willis' circle and optic pathways, and the invasion of the hypothalamus often prevent its safe surgical resection. Moreover, the local aggressiveness of the tumor accounts for a high risk of recurrence even after a gross total resection. For these reasons, more and more efforts are being dedicated to enhance the knowledge about AC and improve the tools for its treatment.This paper is dedicated to the most recent advances concerning the AC management. Promising, new insights come for the basic research, thanks to the updates on the role of the WNT-ß-catenin pathway (important for the tumor genesis and progression, not yet developed enough for a safe target therapy in children but useful for determining the prognosis) and the inflammatory mediators (widely overexpressed, especially by the cyst of the tumor, and for which target therapies are being developed). Moreover, further factors and pathways are under investigation.Also the development of new treatment strategies accounts for the improvement of the prognosis and the quality of life of AC patients. The enhancement of the experience with the endoscopic techniques (both transsphenoidal and transventricular approaches) actually allows to perform a less invasive but effective surgery that can be coupled with new modalities of radiation therapy aiming at obtaining a reliable control of the disease and protecting the endocrinological, ophthalmological, and neurological functions. A special mention is finally deserved by the techniques specifically designed for the intracystic therapy (as cyst fenestration alone or in combination with administration of radionuclides or bleomycin or interferon-α) that are here analyzed together with the aforementioned advances.


Assuntos
Craniofaringioma , Cistos , Neoplasias Hipofisárias , Bleomicina , Criança , Craniofaringioma/terapia , Humanos , Neoplasias Hipofisárias/diagnóstico , Qualidade de Vida , Resultado do Tratamento
12.
Endocrine ; 78(1): 123-134, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35869972

RESUMO

PURPOSE: Craniopharyngiomas are nonmalignant sellar and parasellar tumors exhibiting a bimodal age distribution. While the outcomes following treatment in patients with childhood-onset craniopharyngiomas are well characterized, similar information in adult-onset craniopharyngiomas is limited. We aimed to describe the long-term outcomes (weight and metabolic parameters, mortality) in patients with adult-onset craniopharyngioma following treatment. METHODS: Patients with adult-onset craniopharyngioma with initial treatment (1993-2017) and >6 months of follow-up at our institution were retrospectively identified. Body mass index (BMI) categories included obese (BMI ≥ 30 kg/m2), overweight (BMI 25-29.9 kg/m2), and normal weight (BMI < 25 kg/m2). RESULTS: For the 91 patients with adult-onset craniopharyngioma (44% women, mean diagnosis age 48.2 ± 18 years) over a mean follow-up of 100.3 ± 69.5 months, weight at last follow-up was significantly higher than before surgery (mean difference 9.5 ± 14.8 kg, P < 0.001) with a higher percentage increase in weight seen in those with lower preoperative BMI (normal weight (20.7 ± 18%) vs. overweight (13.3 ± 18.0%) vs. obese (6.4 ± 15%), P = 0.012). At last follow-up, the prevalence of obesity (62 vs. 40.5%, P = 0.0042) and impaired glucose metabolism (17.4% vs. 34%, P = 0.017) increased significantly. All-cause mortality was 12%, with the average age of death 71.9 ± 19.7 years (average U.S. life expectancy 77.7 years, CDC 2020). CONCLUSION: Patients with adult-onset craniopharyngioma following treatment may experience weight gain, increased prevalence of obesity, impaired glucose metabolism, and early mortality. Lower preoperative BMI is associated with a greater percentage increase in postoperative weight.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Adulto , Idoso , Idoso de 80 Anos ou mais , Índice de Massa Corporal , Criança , Craniofaringioma/complicações , Craniofaringioma/epidemiologia , Craniofaringioma/cirurgia , Feminino , Glucose , Humanos , Masculino , Pessoa de Meia-Idade , Obesidade/complicações , Obesidade/diagnóstico , Obesidade/epidemiologia , Sobrepeso/complicações , Sobrepeso/epidemiologia , Neoplasias Hipofisárias/cirurgia , Estudos Retrospectivos
13.
Lancet Diabetes Endocrinol ; 10(8): 581-588, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35780804

RESUMO

BACKGROUND: Radiotherapy is a valuable treatment in the management algorithm of pituitary adenomas and craniopharyngiomas. However, the risk of second brain tumour following radiotherapy is a major concern. We assessed this risk using non-irradiated patients with the same primary pathology and imaging surveillance as controls. METHODS: In this multicentre, retrospective cohort study, 4292 patients with pituitary adenoma or craniopharyngioma were identified from departmental registries at six adult endocrine centres (Birmingham, Oxford, Leeds, Leicester, and Bristol, UK and Ferrara, Italy). Patients with insufficient clinical data, known genetic predisposition to or history of brain tumour before study entry (n=532), and recipients of proton beam or stereotactic radiotherapy (n=81) were excluded. Data were analysed for 996 patients exposed to 2-dimensional radiotherapy, 3-dimensional conformal radiotherapy, or intensity-modulated radiotherapy, and compared with 2683 controls. FINDINGS: Over 45 246 patient-years, second brain tumours were reported in 61 patients (seven malignant [five radiotherapy, two controls], 54 benign [25 radiotherapy, 29 controls]). Radiotherapy exposure and older age at pituitary tumour detection were associated with increased risk of second brain tumour. Rate ratio for irradiated patients was 2·18 (95% CI 1·31-3·62, p<0·0001). Cumulative probability of second brain tumour was 4% for the irradiated and 2·1% for the controls at 20 years. INTERPRETATION: Irradiated adults with pituitary adenoma or craniopharyngioma are at increased risk of second brain tumours, although this risk is considerably lower than previously reported in studies using general population controls with no imaging surveillance. Our data clarify an important clinical question and guide clinicians when counselling patients with pituitary adenoma or craniopharyngioma on the risks and benefits of radiotherapy. FUNDING: Pfizer.


Assuntos
Adenoma , Neoplasias Encefálicas , Craniofaringioma , Neoplasias Hipofisárias , Adenoma/diagnóstico por imagem , Adenoma/epidemiologia , Adenoma/radioterapia , Adulto , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/epidemiologia , Neoplasias Encefálicas/radioterapia , Estudos de Coortes , Craniofaringioma/complicações , Craniofaringioma/diagnóstico por imagem , Craniofaringioma/radioterapia , Humanos , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/radioterapia , Estudos Retrospectivos
16.
Medicine (Baltimore) ; 101(26): e29777, 2022 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-35777048

RESUMO

Studies with relatively large sample size as well as long-term follow-up focusing on adult craniopharyngioma (CP) patients are still lacking. We attempted to identify independent prognostic factors and establish a nomogram model to estimate survival rates for adult CP patients. The Surveillance, Epidemiology, and End Results database was used to obtain data on patients with CP. Univariable and multivariable Cox analyses were utilized to identify the prognostic factors of adult CP patients. A survival prediction model was constructed and its predictive performance was also assessed. A total of 991 patients (695 in training group and 296 in validation group) were eligible for final inclusion. Multivariate Cox analysis presented that age at diagnosis, marital status, race, tumor size, and surgery type were statistically significant prognostic factors for overall survival (all P < .05). A graphical predicting nomogram model was developed to calculate the predicted patients' survival probabilities at 1, 2, 5, and 10 years. The concordance indexes were 0.708 ± 0.019 and 0.750 ± 0.025 for the training and validation samples, respectively, demonstrating favorable discrimination abilities. Similarly, the time-dependent area under curve also showed overall satisfactory discrimination ability. Favorable consistencies between the predicted and actual survival were presented according to the calibration curves. An easy-to-use nomogram, being proven to be with reliable discrimination ability and accuracy, was established to help predict overall survival for adult patients with CP using the identified significant prognostic factors.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Adulto , Humanos , Nomogramas , Fatores de Risco , Programa de SEER
18.
World Neurosurg ; 165: e148-e158, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35660482

RESUMO

OBJECTIVE: Papillary craniopharyngioma (PCP) was previously believed to occur only in adults. Sporadic pediatric PCP (PPCP) confirmed by detection of BRAF V600E mutation has been reported since 2018, but is often misdiagnosed before being diagnosed definitively. We aimed to evaluate PPCP characteristics and propose diagnostic criteria for prompt diagnosis, seeking to reduce patient morbidity and mortality and reduce costs linked to misdiagnosis. METHODS: This study included 5 patients with PPCPs whose data were retrieved retrospectively from among 1032 patients with craniopharyngiomas admitted to Sanbo Brain Hospital Management Group from March 2017 to May 2021. Patients' demographics, clinical presentation, tumor imaging characteristics, histopathologic results, surgical approaches, and postoperative outcomes were analyzed. RESULTS: PPCP was misdiagnosed intraoperatively as sellar abscess (n = 4) or Rathke cleft cyst (n = 1). Preoperative magnetic resonance imaging showed that all tumors were under the saddle diaphragm, and the cyst wall was enhanced (n = 5). Computed tomography scans showed scattered high-density signs (n = 4). No recurrence was noted after complete resection. Postoperative hypothalamic dysfunction was mild. BRAF V600E mutation was confirmed in all cases by sequencing and immunohistochemistry. Immunohistochemistry showed granulation and inflammation and MPO, CD3, CD20, CD38, CD68, and CD163 were positively expressed. CONCLUSIONS: Misdiagnosis of PPCP is responsible for failed surgical treatment. We propose that prompt diagnosis of PPCP can be achieved if preoperative magnetic resonance images show the tumor under saddle diaphragm with tumor wall enhancement and computed tomography scans show high-density signs scattered in the tumor, which leads to safe, effective tumor resection. Our proposed diagnosis and treatment strategy for PPCP reduces morbidity and mortality.


Assuntos
Cistos do Sistema Nervoso Central , Craniofaringioma , Neoplasias Hipofisárias , Adulto , Cistos do Sistema Nervoso Central/diagnóstico por imagem , Cistos do Sistema Nervoso Central/genética , Criança , Craniofaringioma/diagnóstico por imagem , Craniofaringioma/genética , Humanos , Imageamento por Ressonância Magnética , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/genética , Proteínas Proto-Oncogênicas B-raf/genética , Estudos Retrospectivos
19.
J Craniofac Surg ; 33(5): e537-e538, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35762626

RESUMO

ABSTRACT: Craniopharyngioma is rare epithelial tumor that develops along the craniopharyngeal duct, and most of these tumors occur in the sellar and suprasellar regions. Although it rarely occurs in the extracranial region, sphenoid solitary lesions were not reported in previous literature. In this study, we report a case of infrasellar craniopharyngioma within the sphenoid sinus without intracranial lesion. A patient with intermittent headache visited a private clinic and presented with sphenoid lesions based on the Magnetic resonance imaging scan results. The mass was completely removed using endoscopic endonasal transsphenoidal approach without any complications and showed characteristic pathologic findings, which lead to the diagnosis of craniopharyngioma.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Craniofaringioma/diagnóstico por imagem , Craniofaringioma/cirurgia , Humanos , Imageamento por Ressonância Magnética , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/cirurgia , Osso Esfenoide/patologia , Seio Esfenoidal/diagnóstico por imagem , Seio Esfenoidal/patologia , Seio Esfenoidal/cirurgia
20.
Front Endocrinol (Lausanne) ; 13: 859381, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35707464

RESUMO

We collected 61 craniopharyngioma (CP) specimens to investigate the expression of TrkA, ß-catenin, BRAF gene mutation, and NTRK1 fusion in CP. There were 37 male and 24 female individuals with a median age of 34 years (range, 4-75 years). Histologically, there were 46 cases of adamantinomatous craniopharyngioma (ACP), 14 cases of papillary craniopharyngioma (PCP), and 1 case with a mixed adamantinomatous and papillary pattern. By immunohistochemistry, we found that moderate/high TrkA expression was detected in 47% (28/60) CP and was significantly higher in adult patients (p = 0.018). Interestingly, TrkA is more expressed in "whorled epithelium" cells in ACP, similar to the localization of abnormal ß-catenin. The abnormal expression rate of ß-catenin was 70% (43/61), and the medium/high cyclin D1 expression rate was 73% (44/60), both of which were significantly higher in ACP than in PCP. Of the CP, 41% (21/51) had a moderate/strong P16-positive signal; 58% (34/59) showed a high Ki-67 expression, and there was a significant correlation between high Ki-67 L.I. and high tumor recurrence (p = 0.021). NTRK1 fusion was not found in CP by fluorescence in situ hybridization (FISH). By PCR, 26% (15/58) CP showed BRAF V600E gene mutation, which mainly occurred in PCP (100%, 14/14) except one case of mixed CP. Moreover, TrkA expression was negatively correlated with Ki-67 index and positively correlated with P16 expression. There was a significantly negative correlation between BRAF V600E mutation and abnormal ß-catenin expression. Our results demonstrate for the first time that TrkA expression might occur in CP, especially in adult CP patients, and suggest that cyclin D1 could be used for ACP histological classification in addition to ß-catenin and BRAF V600E mutation, while Ki-67 could be used as a marker to predict CP recurrence.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Proteínas Proto-Oncogênicas B-raf , Receptor trkA , beta Catenina , Adolescente , Adulto , Idoso , Ciclo Celular , Criança , Pré-Escolar , Craniofaringioma/genética , Ciclina D1/genética , Feminino , Humanos , Hibridização in Situ Fluorescente , Antígeno Ki-67/genética , Masculino , Pessoa de Meia-Idade , Mutação , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/metabolismo , Prognóstico , Proteínas Proto-Oncogênicas B-raf/genética , Receptor trkA/genética , Adulto Jovem , beta Catenina/genética
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