Assessment of quality of life in patients with craniopharyngioma and identification of risk factors for compromised overall wellness

ABSTRACT Objective: Quality of Life (QoL) has been a multifactorial concerning issue in oncology. We aimed to inspect the pre-operative QoL among patients with craniopharyngioma and to explore the potential correlations between parameters of QoL and clinical indices. Subjects and methods: We enrolled a total of 109 patients with craniopharyngioma. We utilized Short Form 36 (SF-36), Symptom Check List-90, Generalized Anxiety Disorder Questionnaire scale (GAD7), Patient Health Questionnaire Depression (PHQ9) and Pittsburgh Sleep Quality Index to prospectively evaluated their QoL. Parameters of QoL along with clinical indices were compared among sub-groups divided according to Puget classification. Correlation analyses and regression analyses were performed to detect influential determinants to self-reported wellness. Results: Patients presented impaired QoL compared with general population ( p < 0.001), as assessed by SF-36. Correlation analyses indicated the detrimental influence resulting from central diabetes insipidus (CDI). Multivariate linear regression unveiled the adverse effect of CDI on Mental Component Summary (coefficient = −13.869, p = 0.007), GAD7 total score (coefficient = 2.072, p = 0.049) as well as PHQ9 total score (coefficient = 3.721, p = 0.001). Multivariate logistic regression verified CDI as a risk factor of developing depressive symptoms (OR = 6.160, p = 0.001). Conclusion: QoL of patients with craniopharyngioma was remarkably compromised before operation. CDI exerted detrimental influences on patients' QoL and it might serve as a marker for early identification of patients at risk of depression.

Características clínicas e histopatológicas de niños peruanos con craneofaringioma

Introducción: Los craneofaringiomas son tumores benignos, de los cuales hasta el 50% ocurren en niños. Sin embargo, no hay estudios en niños peruanos. Objetivo: describir las características clínicas e histopatológicas de niños con craneofaringioma hospitalizados en el Hospital Nacional Edgardo Rebagliati Martins entre agosto de 2019 y mayo de 2021. El estudio: El diseño es transversal y la fuente de los datos fueron las historias clínicas. Se recolectó información sobre el sexo, edad, cirugías y características relacionadas a la primera cirugía de resección tumoral. Hallazgos: Incluimos 12 pacientes. El 83.3% fueron varones, al momento del diagnóstico la mediana de edad fue de 6 años y predominaron los síntomas visuales, todos tuvieron deficiencias hormonales luego de la primera cirugía de resección. Conclusión: tres recibieron radioterapia, uno desarrolló transformación maligna y otro falleció. Es necesario realizar a futuro estudios prospectivos.

Introduction: Craniopharyngiomas are benign tumors, of which up to 50% occur in children. However, there are no studies in Peruvian children. Objective : to describe the clinical and histopathological characteristics of children with craniopharyngioma hospitalized at the Edgardo Rebagliati Martins National Hospital between August 2019 and May 2021. The study: The design is cross-sectional and the data source were medical records. Information on sex, age, surgeries and characteristics related to the first tumor resection surgery were collected. Findings: We included 12 patients. 83.3% were male, at the time of diagnosis the median age was 6 years and visual symptoms predominated, all had hormonal deficiencies after the first resection surgery. Conclusions: three received radiotherapy, one developed malignant transformation and one died. Future prospective studies are necessary.

Study on the correlation between retinal microvascular density and damage to visual field in patients with sellar region tumor / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To evaluate the changes of retinal microvascular density in patients with sellar region tumor, and its correlation with the damage to visual field, and to explore its application value in evaluating optic nerve injury of those patients.METHODS: Cross-sectional study. A total of 157 patients(292 eyes)with sellar region tumor, including 82 cases(152 eyes)of pituitary adenoma and 75 cases(140 eyes)of craniopharyngioma, were selected from neurosurgery department and ophthalmology department of Beijing Tiantan Hospital, Capital Medical University between October 2018 and May 2022. A total of 90 people(180 eyes)during the same period, including the family members of patients, students and staff in Beijing Tiantan Hospital, Capital Medical University were collected as control group. All participants underwent optical coherence tomography angiography(OCTA)examination. The changes of retinal microvascular density and its correlation with visual field parameters were compared between the two groups.RESULTS: In patients with sellar region tumor, the radial peripapillary capillary(RPC)and superficial retinal capillary plexus(SRCP)density were significantly lower than that in the control group [50.81%(46.49%, 53.49%)vs. 52.78%(50.73%, 54.51%)and 50.57%(48.13%, 52.73%)vs. 51.63%(49.78%, 53.02%), all P<0.05]. The RPC density in the craniopharyngioma group was lower than that in the pituitary adenoma group [49.71%(44.33%, 53.14%)vs. 51.37%(47.42%, 53.95%), P<0.05]. The MD, PSD and VFI of the sellar region tumor group were -4.33(-12.22, -1.85)dB, 3.37(1.91, 8.82)dB and 92%(65%, 97%)respectively. RPC density of patients with sellar region tumor was positively correlated with MD and VFI, and was negatively correlated with PSD. The SRCP density of each quadrant was positively correlated with MD, and was positively correlated with VFI except Para-T and it was negatively correlated with PSD(all P<0.05).CONCLUSION: Retinal microvascular changes were present in patients with sellar region tumor. Lower vessel density indicates more severe damage to visual field. In the clinic, visual field examinations combined with OCTA were helpful to find the optic nerve injury of patients.

The molecular pathogenesis of craniopharyngiomas

ABSTRACT Research from the last 20 years has provided important insights into the molecular pathogenesis of craniopharyngiomas (CPs). Besides the well-known clinical and histological differences between the subtypes of CPs, adamantinomatous (ACP) and papillary (PCP) craniopharyngiomas, other molecular differences have been identified, further elucidating pathways related to the origin and development of such tumors. The present minireview assesses current knowledge on embryogenesis and the genetic, epigenetic, transcriptomic, and signaling pathways involved in the ACP and PCP subtypes, revealing the similarities and differences in their profiles. ACP and PCP subtypes can be identified by the presence of mutations in CTNNB1 and BRAF genes, with prevalence around 60% and 90%, respectively. Therefore, β-catenin accumulates in the nucleus-cytoplasm of cell clusters in ACPs and, in PCPs, cell immunostaining with specific antibody against the V600E-mutated protein can be seen. Distinct patterns of DNA methylation further differentiate ACPs and PCPs. In addition, research on genetic and epigenetic changes and tumor microenvironment specificities have further clarified the development and progression of the disease. No relevant transcriptional differences in ACPs have emerged between children and adults. In conclusion, ACPs and PCPs present diverse genetic signatures and each subtype is associated with specific signaling pathways. A better understanding of the pathways related to the growth of such tumors is paramount for the development of novel targeted therapeutic agents.

Microneurosurgery treatment for 41 recurrent craniopharyngioma cases / 中国医师进修杂志

Objective:To investigate the method and effect of microneurosurgery in the treatment of recurrent craniopharyngioma.Methods:The clinical data of 41 recurrent craniopharyngioma patients treated by microneurosurgery in Capital Medical University Sanbo Brain Hospitalfrom January 2018 to January 2022 were retrospectively analyzed.Results:Among the 41 patients, 38 cases were treated with the frontal basal interhemispheric approach and 3 cases with the translongitudinal fissure combined with the transSylvian fissure approach. Gross total resection was performed in 34 cases (82.9%) and subtotal resection in 7 cases (17.1%). One patient (2.4%) died of pulmonary embolism during perioperative period. All patients had transient electrolyte disorder after operation, and recovered within 3 months after treatment. All patients had endocrine dysfunction. After 3 months of hormone replacement therapy, 11 patients were cured. Visual acuity decreased in 3 cases after operation, and 2 cases improved after treatment.Conclusions:Surgical treatment of recurrent craniopharyngioma is very difficult, which is a great challenge for doctors and patients. However, through the individualized evaluation of the patients and the meticulous technique of the doctors during the operation, a satisfactory effect can be achieved post the operation of recurrent craniopharyngioma.

Craniopharyngioma and anterior central space of brain base / 中国医师进修杂志

Objective:To analyze the surgical (anatomical) approach of craniopharyngioma based on the concept of anterior central space of brain base.Methods:The clinical data of MRI images of 10 cases of craniopharyngioma who treated in Sanbo Brain Hospital, Capital Medical University from January 2021 to December 2021 were analyzed retrospectively. The brain cisterns involved in the growth of craniopharyngioma were analyzed from the perspective of anterior central space of brain base. Meanwhile, the surgical approach and the basis of selection were discussed.Results:Among all the surgical approaches that can reach the anterior central space of the brain base, the fronto-basal interhemispheric approach was the best.Conclusions:The concept of anterior central space of the brain base is a new observation and interpretation of the anatomy of the brain base from a new perspective, and then create a new concept of brain base surgery. As a new method to analyze the surgical anatomy of this area, its inclusion has important clinical significance.

The etiology and treatment of craniopharyngioma with aneurysm / 中国医师进修杂志

Objective:To explore the etiology and treatment of craniopharyngioma with aneurysm.Methods:Seven cases of craniopharyngioma with aneurysm from March 2014 to October 2019 treated in Sanbo Brain Hospital, Capital Medical University were retrospectively analyzed. Among the 7 patients, there were 5 males and 2 females. There were 4 cases of recurrent craniopharyngiomas, 1 case of primary tumor and 2 cases of non-recurrence tumor. Three patients with blood blister-like aneurysms were treated with microsurgical suture after craniopharyngioma resection. Among the three cases with internal carotid artery fusiform aneurysm, 1 case underwent craniopharyngioma resection after internal maxillary artery-radial artery-middle cerebral artery bypass and isolation of the aneurysm; 1 case only underwent internal maxillary artery-radial artery-middle cerebral artery bypass and isolation of the aneurysm for non-recurrence tumor; 1 case underwent craniopharyngioma resection and dynamic observation of aneurysm. One case with a cystic aneurysm of the middle cerebral artery was clipped and the craniopharyngioma did not relapse.Results:All patients had no serious postoperative complications. During the follow-up period, there was no recurrence of craniopharyngioma, no recurrence of treated aneurysms, and the stability of aneurysms was observed.Conclusions:Inflammatory stimulation of craniopharyngioma cystic fluid and operation itself are the important reasons for the occurrence of aneurysms after craniopharyngioma surgery. Choosing appropriate surgical methods can complete the removal of craniopharyngioma and the treatment of aneurysms at one time.

Imaging findings and clinical analysis of craniopharyngioma / 中国医师进修杂志

Objective:To retrospectively summarize the imaging and clinical features of craniopharyngioma in order to improve the preoperative diagnosis level.Methods:One hundred and twenty-seven patients with craniopharyngioma diagnosed by pathology in Sanbo Brain Hospital, Capital Medical University from March 2019 to June 2021 were selected and the pathological coincidence rate of imaging diagnosis were analyzed.Results:The coincidence rate of MRI diagnosis was 89.3%. The coincidence rate of CT diagnosis was 71.5%. On T 2WI and T 1WI enhanced sequences, the solid portion of the tumor may showed uneven hyperintensity, diffuse striation and spotty hyperintensity. MRI sagittal view was helpful in showing small tumors, but less sensitive to calcification than CT. MRI enhancement was very important, especially for patients with solid lesions. Conclusions:The imaging findings of craniopharyngioma are diverse. Some characteristic manifestations provide important information for the diagnosis and differential diagnosis of craniopharyngioma, which can improve the diagnostic accuracy combined with clinical data.

Resultados clínicos y quirúrgicos del tratamiento de craneofaringiomas en pediatría / Clinical and surgical results of the treatment of craniopharyngiomas in pediatrics

Introducción: Los craneofaringiomas son tumores benignos frecuentes en pediatría. La cirugía resectiva solía ser el tratamiento de elección. Sin embargo, en los últimos años se encuentra muy cuestionada debido a su elevada morbilidad. El objetivo primario de este trabajo es presentar los resultados clínicos y quirúrgicos de la cirugía resectiva de craneofaringiomas por vía transcraneal en nuestra institución. El objetivo secundario es presentar una serie de casos en los que se realizó un tratamiento quirúrgico conservador utilizando un reservorio de Ommaya para tratamiento del componente quístico. Métodos: Estudio descriptivo de una serie de casos que compara evaluaciones clínicas e imagenológicas pre y postoperatoria en pacientes sometidos a cirugía resectiva de craneofaringioma. Además, se presenta una serie de casos de pacientes con craneofaringiomas predominantemente quísticos tratados con colocación de catéter de Ommaya intraquístico e Interferón. Resultados: Se analizaron 33 pacientes con craneofaringioma sometidos a resección quirúrgica. Al año de la cirugía, encontramos que el 51.52% de los casos (17 pacientes) se mantuvo sin crecimiento de la lesión y que el 48.48% (16 pacientes) presentó aumento o recidiva. En el postoperatorio todas las evaluaciones clínicas empeoraron. Fueron estadísticamente significativas la aparición de diabetes insípida (18.18% vs. 69.70%; p=0.04), obesidad (12.12% vs. 36.36%; p=0.005) y talla baja (33.33% vs. 57.58%; p=0.0006). En los tratados con Ommaya e Interferón, el 28.57% (2 casos) presentó reducción o estabilidad en el quiste y el 71.43% (5 casos) aumentó el tamaño, aunque presentó mejores resultados respecto a la restricción de la talla y obesidad. Conclusión: La exéresis quirúrgica de los craneofaringiomas genera una alta tasa de comorbilidades sumado a un porcentaje elevado de recidiva. El manejo conservador tratando el componente quístico en los casos en los que sea posible podría ser una opción viable. El tratamiento de los craneofaringiomas de la infancia debe abordarse de forma multidisciplinaria intentando preservar el bienestar y la calidad de vida de los pacientes

Introduction: Craniopharyngioma is a common benign tumor in children. Although surgery has traditionally been the treatment of choice, it has been controversial in recent years due to the associated high morbidity. The main aim of this paper is to present the clinical and surgical outcomes seen after craniopharyngioma resection in our institution. The secondary objective is to present a case series of patients treated with an Ommaya reservoir (OR) and interferon. Methods: This was a descriptive study comparing the pre and post-surgical clinical and image assessment in patients undergoing craniopharyngioma resection. Also, a case series including patients with craniopharyngioma managed with an Ommaya reservoir (OR) and Interpheron was included. Results: Thirty-three patients with craniopharyngioma undergoing surgical resection were assessed. We found no tumor growth in 51.52% (17) of the cases, and either tumor growth or recurrence in 48.48% of the cases at one year. Clinical worsening was observed in the post-operative period; the presence of diabetes insipidus (18.18% vs. 69.70%; p=0.04), obesity (12.12% vs. 36.36%; p=0.005) and short stature (33.33% vs. 57.58%; p=0.0006) were statistically significant. Of the patients treated with an OR and interferon, 28.57% (2) presented tumor cyst regression or stability, and 71.43% exhibited tumor cyst growth. Conclusion: The surgical resection of craniopharyngiomas is associated with a high recurrence rate and usually high mortality. A multidisciplinary management of craniopharyngiomas in childhood is advisable in order to preserve the wellbeing and quality of life of patients

Advance in factors affecting the long-term quality of life in children with craniopharyngioma / 国际儿科学杂志

Craniopharyngioma mainly occurs in children aged 5 to 14 years.It′s one of the most common intracranial tumors for children.Although the overall survival rate of pediatric patients with craniopharyngioma has been significantly improved, the long-term quality of life still couldn′t be missed due to the fact that it could invade the important structures around the sellar area, such as the hypothalamus, pituitary gland, and so on.The downward trend is observed among the 5-, 10- and 20-year overall survival rates.Compared with adult-onset patients, childhood-onset patients are at higher risk of hypothalamic involvement, endocrine dysfunction, obesity, have a lower long-term quality of life.In addition to a variety of physical diseases, pediatric patients with craniopharyngioma after surgery may also develop the psychological diseases.Craniopharyngioma should be recognized as a frequently chronic disease which requires constant monitoring of the consequences and medical resources for treatment in order to provide optimal quality of life for pediatric patients.This article reviews the craniopharyngioma-related researches in recent years on the long-term prognosis of childhood-onset patients.

Craneofaringiomas: cirugía transnasal via endoscópica / Craniopharyngiomas: endoscopic transnasal surgery

Introducción: Debido a su ubicación anatómica, los craneofaringiomas son tumores complejos en su tratamiento. Una resección completa tiene riesgos elevados de morbimortalidad y si se opta por una resección parcial la tasa de recurrencia es alta. Según su extensión supraselar y su relación con el tallo hipofisario, pueden dividirse en preinfundibulares, transinfundibulares y retroinfundibulares. Objetivo: El objetivo de este video es describir la técnica vía endoscópica para la resección de un craneofaringioma preinfundibular. Materiales y Métodos: Se seleccionó un caso de un paciente con un craneofaringioma preinfundibular operado en el Servicio de Neurocirugía del Hospital Italiano de Buenos Aires, por vía endoscópica transnasal. Resultados: Se realizó una exéresis completa de la masa tumoral, sin evidenciar complicaciones tales como, diabetes insípida o fistula de líquido cefalorraquídeo y con recuperación completa del déficit campimétrico. Conclusión: El tratamiento quirúrgico de los craneofaringiomas requiere un conocimiento detallado de la anatomía de base de cráneo así como de las diferentes técnicas quirurgicas. El uso de la endoscopia ha permitido un mejor acceso a éste tipo de lesiones, disminuyendo las comorbilidades en el paciente y la estadía hospitalaria. Un resultado quirúrgico satisfactorio se obtiene con la resección completa y la menor morbilidad posible para el paciente

Introduction: Due to its anatomical location, craniopharyngiomas are difficult tumors to treat. Complete resection has high morbidity and mortality and if a partial resection is chosen, the recurrence is common. According to their suprasellar extension and its relationship with stalk, it can be classified into: preinfundibular, transinfundibular and retroinfundibular. Objetive: The aim of this video is to describe the surgical technique we use for preinfundibular craniopharyngioma. Methods: We review a preinfundibular craniopharyngioma operated on the Neurosurgery Department of the Hospital Italiano de Buenos Aires, through a transnasal endoscopic approach. Results: Total removal was achived, no acute complications were found such as diabetes insipidus or cerebrospinal fluid leak and improved their visual field. Conclusion: Craniopharyngioma surgery requires detailed knowledge of skull base anatomy and approaches. The use of endoscopy has allowed better access to this type of lesions, reducing comorbidities and patient hospital stay. Complete resection with low morbidity are the treatment of this tumors

Multiplanar MRI imaging of sellar and parasellar lesions with clinical and pathological correlation

Background: Sellar and parasellar/ juxtasellar regions are complex areas of the brain, hold delicate neurovascular structures. A number of diseases that affect the pituitary-hypothalamic axis can have profound clinical, endocrinological as well as neurological consequences. Aim of this retrospective study was to identify the MR imaging characteristics and epidemiology of sellar and suprasellar lesions, to correlate the MRI findings with histopathological findings and to highlight the diagnostic superiority of MR imaging.Methods: Author studied the records of 65 patients with sellar and suprasellar lesions for which preoperative MR imaging films or reports were available. Radiological appearances were correlated with intraoperative findings and post-operative histopathology.Results: Majority of patients in this study belonged to the age group 21-40 years. Most common mass lesion found was pituitary macroadenoma comprising 58% of the total cases. The accuracy of MRI in diagnosing macroadenomas are 96.80%. MR was 87.50%accurate, in diagnosing craniopharyngioma. MR was 100% in diagnosing meningioma in our study.Conclusions: MRI is the modality for characterizing sellar and suprasellar lesions, morphology of lesions, nature of contrast material enhancement and extent of lesions. Hence MRI is the modality of choice for diagnosing sellar and suprasellar masses with high accuracy

Craneofaringioma: resección paso a paso en un paciente pediátrico / Craniopharyngioma: Step-by-Step Resection in a Pediatric Patient

Introducción: Describir detalladamente paso a paso la resección de un craneofaringioma por vía endoscópica endonasal en un paciente pediátrico, con el fin de guiar a neurocirujanos en formación. Materiales y métodos: Se describe detalladamente la técnica endoscópica endonasal transesfenoidal para la resección de un tumor de estirpe craneofaringioma adamantinomatoso en un paciente masculino de 3 años de edad con la variante de seno esfenoidal tipo conchal. Resultados: Se logró resección total de un craneofaringioma en paciente pediátrico mediante abordaje endoscópico endonasal. Se respetaron las estructuras vasculares circundantes y se pudo prevenir la fístula de líquido cefalorraquídeo con la utilización del flap nasoseptal. Conclusión: El abordaje endoscópico endonasal transesfenoidal ofrece una exposición amplia de la región selar y supraselar permitiendo una excelente resección de los craneofaringiomas en pacientes pediátricos. Consideramos de importancia la curva de aprendizaje para lograr una resección máxima sin agregar comorbilidades al paciente.

Objective: To give a detailed description, step by step, of endoscopic endonasal resection for craniopharyngioma in a pediatric patient. This manuscript was made to teach neurosurgeons in their former years. Methods: Detailed description with intraoperative images from a complete resection of adamantinomatous craniopharyngioma in a 3 years-old patient with a conchal variant sphenoid sinus. Results: We achieved a gross total resection of a craniopharyngioma in a pediatric patient by endoscopic endonasal approach. We preserve the surrounding vascular structures with no comorbidities. Cerebrospinal leak was prevented by using the nasoseptal flap covering the sphenoid defect. Conclusion: The endoscopic endonasal approach offers a wide exposure of the selar and supraselar región. This allowed us a great possibility for complete resection in selar and supraselar craniopharyngiomas in pediatric population. We consider that maximal resection should be balanced with a good learning curve to avoid comorbidities.

Magnetic resonance imaging characteristics and differential diagnosis of common sellar cystic lesions / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate magnetic resonance imaging (MRI) characteristic and differential diagnostic keypoints of common sellar regional cystic lesions with the purpose of improving differential diagnostic accuracy.@*Methods@#In total, 174 cases of the pathologically diagnosed cystic lesions in sellar region between March 2016 and June 2019 were included in the current retrospective analysis. These cases included Rathke′s cleft cyst (n=68), craniopharyngioma (n=48), pituitary adenoma (n=56) and arachnoid cyst (n=2) in the sellar region. The position, texture and morphology of leisions, signal of cystic and solid part, invasion of surrounding tissues and maximum diameter of cysts on the MRI images were evaluated and compared between different groups. SPSS 25.0 software was used for statistical analysis.@*Results@#There were several specific changes on several MRI sequences in Rathke′s cleft cysts, craniopharyngioma and pituitary adenoma groups. For Rathke′s cleft cysts, round shape, rare polycystic structure, lack of solid part and maximum diameter of cysts which was significantly less than that of craniopharyngioma and pituitary adenoma groups ((17.37±6.12) mm vs (30.29±13.51) mm vs (28.18±11.13) mm, t value was 6.680, 5.838, respectively, all P<0.05), were favorable diagnostic criteria. While, the intracystic nodules were mostly found in craniopharyngioma. There was no high signal intensity in cystic wall on T1WI and T2WI of pituitary adenoma. MRI signal of arachnoid cyst in sellar region was basically the same as CSF signal, with low signal intensity on T1WI and DWI, high signal intensity on T2WI, without enhanced signal.@*Conclusions@#Common sellar regional cystic lesions showed characteristic manifestations on different MRI sequences. Valuable information in the morphology, MRI signal intensity, maximum diameter measurements may improve accuracy of differential diagnosis for sellar regional cystic lesions.

Long-term effect of neuroendoscopy followed by radiotherapy on cystic craniopharyngiomas / 中国医师杂志

Objective@#To explore the long-term effect of neuroendoscopy followed by radiotherapy on cystic craniopharyngiomas.@*Methods@#Cystic craniopharyngiomas in 9 patients were treated with neuroendoscopic cyst aspiration and fenestration, followed by fractionated stereotactic radiotherapy (FSRT). The neuroendoscopic procedure focused on widening of cyst fenestration and extensive irrigation of the cyst contents. The collimator of FSRT ranged from 2.5 cm to 3.0 cm, and the target volume 1.1-43.8 cm3, dose per fraction 1.8 Gy, total dose 50.4 Gy.@*Results@#The median follow-up period was 72.9 months. Tumor control was achieved in 8 of 9 patients. Marked tumor volume reduction was obtained with the neuroendoscopic procedure alone at 6 months, 1 year, and 2 years. One recurrent case showed multilobulated cysts, and a second surgery was required 1 year after the treatment. Clinical symptoms such as headache and visual disruption were rapidly alleviated after the neuroendoscopic procedure. No new visual disturbances, endocrinopathy, or hypothalamic dysfunction was observed during follow up.@*Conclusions@#Stereotactic radiotherapy for cystic craniopharyngioma after endoscopic fenestration can effectively control the tumor for a long period of time, improve the clinical symptoms and avoid endocrine diseases.

Implication of BRAF V600E and CTNNB1 gene mutations in the pathological classification of craniopharyngioma / 中华病理学杂志

Objective@#To investigate the clinicopathological significance of BRAF V600E and CTNNB1 gene mutations in adamantinomatous craniopharyngiomas (ACP) and papillary craniopharyngiomas (PCP).@*Methods@#The retrospective study included a total of 67 craniopharyngiomas diagnosed from October 2009 to August 2018 at Xuanwu Hospital, Capital Medical University. The immunohistochemical staining for β-catenin and BRAF V600E expression, Sanger sequencing of exon 3 of CTNNB1, BRAF mutation analysis by scorpions amplification refractory mutation system (ARMS) fluorescence quantitative PCR were performed. Univariate survival analysis was used to correlate with tumor recurrence.@*Results@#Of the 67 patients, 53 were ACPs and 14 were PCPs. Four patients underwent multiple operations and one of them presented with malignant transformation into squamous cell carcinoma. Histologically, ACPs were characterized by whorl-like cell clusters, peripheral palisaded layer, stellate reticulum, finger-shaped protrusions, ghost cells and wet keratinous substances. While PCPs usually consisted of mature squamous epithelium associated with fibrovascular stroma resulting in papillary appearance. The nuclear immunopositivity for β-catenin was observed in 73.6% (39/53) of ACPs, and it was absent in PCPs (0/14). The nuclear translocation of β-catenin usually presented at whorl-like structures or around ghost cells. Of all the cases, mutations analysis in exon 3 of β-catenin gene CTNNB1 were successful in 46 cases and 42.1% (16/38) of ACP showed CTNNB1 gene mutation, while none of the PCPs harbored CTNNB1 gene mutation (0/8). The cytoplasmic immunopositivity for BRAF V600E mutant protein was found in all PCPs (14/14) and negative in all ACPs (0/53). ARMS-PCR results showed that BRAF V600E mutations were observed in 13/14 of PCPs but not seen in ACPs (0/53). Follow-up data were available in 35 patients with duration of 2 to 120 months. Ten patients experienced recurrences after the first surgery. Upon univariate survival analysis, only subtotal excision was found to be associated with increased recurrence (P=0.032), while pathological type, postoperative radiotherapy and CTNNB1 gene mutation were not (P>0.05).@*Conclusions@#There is significant difference in the expression of BRAF V600E and CTNNB1 genes between ACP and PCP, and their immunohistochemical and molecular detection therefore can be used in the diagnosis and differential diagnoses of craniopharyngiomas.

Approach to the patient with pancytopenia secondary to panhypopituitarism after craniopharyngioma surgery / 中华内分泌代谢杂志

Craniopharyngioma is the most common benign intracranial tumor in children. The major post-operative complication is dysfunction of pituitary, which can result in many complicate clinical manifestations with hormonal deficiencies. Normochromic anemia has been reported as a common hematologic abnormality. However, pancytopenia is rarely reported so far. Here we describe a 21-year-old inpatient with the main complaint of nasal bleeding, who accepted craniopharyngioma surgery 9 years ago. Laboratory tests showed pancytopenia secondary to panhypopituitarism. This paper aims to increasing the awareness of this disease and accumulating clinical experiences for the clinicians.

Long-term effect of neuroendoscopy followed by radiotherapy on cystic craniopharyngiomas / 中国医师杂志

Objective To explore the long-term effect of neuroendoscopy followed by radiotherapy on cystic craniopharyngiomas.Methods Cystic craniopharyngiomas in 9 patients were treated with neuroendoscopic cyst aspiration and fenestration,followed by fractionated stereotactic radiotherapy (FSRT).The neuroendoscopic procedure focused on widening of cyst fenestration and extensive irrigation of the cyst contents.The collimator of FSRT ranged from 2.5 cm to 3.0 cm,and the target volume 1.1-43.8 cm3,dose per fraction 1.8 Gy,total dose 50.4 Gy.Results The median follow-up period was 72.9 months.Tumor control was achieved in 8 of 9 patients.Marked tumor volume reduction was obtained with the neuroendoscopic procedure alone at 6 months,1 year,and 2 years.One recurrent case showed multilobulated cysts,and a second surgery was required 1 year after the treatment.Clinical symptoms such as headache and visual disruption were rapidly alleviated after the neuroendoscopic procedure.No new visual disturbances,endocrinopathy,or hypothalamic dysfunction was observed during follow up.Conclusions Stereotactic radiotherapy for cystic craniopharyngioma after endoscopic fenestration can effectively control the tumor for a long period of time,improve the clinical symptoms and avoid endocrine diseases.

Strictly Intraventricular Craniopharyngioma: Case Report and Literature Review

Strictly intraventricular craniopharyngiomas are a rare topographical variety of craniopharyngiomas. The correct diagnosis is important in order to define the surgical planning, as the surgical access is different for suprasellar tumors with secondary invasion of the third ventricle. An image diagnosis may be difficult, though suggestive patterns exist. The aim of the present case report and literature review is to add to the scarce literature on strictly intraventricular craniopharyngiomas, as well as to remind the neurosurgeon of this rare diagnosis so that the proper treatment is provided.

Craniofaringiomas puramente intraventriculares constituem uma rara variedade topográfica dos craniofaringiomas. O diagnóstico correto é fundamental para a definição do plano cirúrgico, posto que o acesso a este tipo de tumor difere dos tumores suprasselares com invasão secundária do terceiro ventrículo. A confirmação por neuroimagem pode ser difícil, embora existam características sugestivas. A presente descrição de caso, bem como a revisão de literatura, visa contribuir com a escassa literatura a respeito de craniofaringiomas puramente intraventriculares, além de remeter o neurocirurgião a este diagnóstico raro para a adoção da conduta correta de tratamento.

Craniopharyngioma with Intratumoral Hemorrhage and Superficial Siderosis

Superficial siderosis of the central nervous system (CNS) is a progressive and debilitating neurological disease manifesting sensorineural hearing loss, cerebellar ataxia, and pyramidal tract signs. Chronic extravasation of blood into the subarachnoid space results in the accumulation of hemoglobin derivate in the subpial layer of the CNS, which is toxic to the neural tissues. Craniopharyngioma is a benign third ventricle tumor, which rarely presents with tumor bleeding. We report a rare case of superficial siderosis associated with craniopharyngioma with intratumoral hemorrhage in a patient with no history of prior trauma or CNS surgery.