Pituicytoma: a clinicopathological analysis of twenty-one cases / 中华病理学杂志

Objective: To investigate the clinicopathological features and treatment strategies of pituicytoma. Methods: Twenty-one cases of pituicytoma were collected at the First Affiliated Hospital of Nanjing Medical University and Jinling Hospital, Nanjing, China from 2009 to 2020. The clinical data of 21 pituicytoma patients was retrospectively analyzed, and the relevant literature was reviewed. Results: Twenty-one patients aged 4 to 68 years, including 8 males and 13 females. All patients underwent surgical treatment. Histologically, the tumor was consisted almost entirely of elongate, bipolar spindle cells arranged in a fascicular or storiform pattern. Mitotic figures were rare. Immunohistochemically, tumor cells were diffusely positive for S-100 protein (21/21), vimentin (15/15) and TTF1 (14/14), while they were weakly or focally positive for GFAP (13/16) and EMA (6/12). CKpan was negative in all cases and Ki-67 proliferation index was low (<5%). Among the 18 patients with follow-up, all survived and 2 relapsed after surgery. Conclusions: Pituicytoma is a rare low-grade glioma of the sellar area. It is easily confused with other sellar tumors. Preoperative diagnosis is difficult. It needs to be confirmed by histopathology and immunohistochemistry. Microsurgery is the main treatment method at present.

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

Left out - persistent pediatric depression in Panhypopituitarism: A case report

@#This is a case of a 15-year-old Filipino male with a history of craniopharyngioma who underwent trans-sphenoidal surgery with panhypopituitarism as its sequelae. The biological factors of the patient's disease contributed to his persistent depression and were perpetuated by psychosocial and cognitive factors. A biopsychosocial approach was used in understanding this case to arrive at individualized treatment and management.

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

Diagnóstico precoce de craniofaringioma na infância - relato de caso / Early diagnosis of craniopharingioma in childhood - case report

OBJETIVO: Relatar um caso de craniofaringioma em um pré escolar com sintomatologia de início recente. RELATO DO CASO: Menino de cinco anos com cefaleia de início há 20 dias, acompanhada de vômitos. Na admissão foi realizada uma tomografia computadorizada (TC) de crânio que mostrou uma hidrocefalia com presença de uma massa sugestivo de craniofaringioma. Realizou a ressecção microcirúrgica em outro serviço, sendo que no pós-operatório paciente evoluiu com pan-hipopituitarismo, trombose assintomática do seio sagital superior e oscilações do sódio sérico. CONSIDERAÇÕES: A combinação clínica de cefaleia, deficiência visual, diminuição da taxa de crescimento e polidipsia/poliúria levar à suspeita de craniofaringioma como diagnóstico diferencial. O diagnóstico é realizado por exames de imagem como TC ou RM. O principal tratamento é a resseção cirúrgica. Algumas complicações como alteração no eixo hipotálamo-hipofisário, diabetes insipido e obesidade hipotalâmica são frequentes nesses pacientes

OBJECTIVE: To report a case of craniopharyngioma in a preschool with symptoms of recent onset. CASE REPORT: Five-year-old boy with headache starting 20 days ago, accompanied by vomiting. Upon admission, a computed tomography (CT) scan of the skull was performed, which showed hydrocephalus with the presence of a mass suggestive of craniopharyngioma. Microsurgical resection was performed in another service, and in the postoperative period the patient developed pan-hypopituitarism, asymptomatic thrombosis of the upper sagittal sinus and oscillations in serum sodium. CONSIDERATIONS: The clinical combination of headache, visual impairment, decreased growth rate and polydipsia / polyuria lead to the suspicion of craniopharyngioma as a differential diagnosis. The diagnosis is made by imaging exams such as CT or MRI. The main treatment is surgical resection. Some complications, such as changes in the hypothalamic-pituitary axis, diabetes insipidus and hypothalamic obesity are common in these patients

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.

Idiopathic Granulomatous Hypophysitis with Rapid Onset: A Case Report

Idiopathic granulomatous hypophysitis (IGH), a rare disease, requires differentiation from more common mass lesions of the sella such as pituitary adenoma, craniopharyngioma, Rathke's cleft cyst, or pituitary tuberculoma. IGH usually presents with an insidious onset of visual defects and headaches. On the other hand, rapid onset of neurologic and visual symptoms in an IGH patient is exceptionally rare. Here, we present a biopsy-proven case of IGH with rapid onset and satisfactory outcome after high dose steroid treatment.

A Case of Adipsic Hypernatremia in a Patient with Panhypopituitarism Treated with Growth Hormone Replacement

Adipsic hypernatremia is a rare disease where patients do not feel thirst even in the increased serum osmotic pressure and results in electrolyte imbalance, severely increased osmotic pressure and neurologic symptoms like nausea, vomiting, and seizures. We report a 12-year-old male patient who had underwent a trans-sphenoidal surgery for craniopharyngioma newly diagnosed with adipsic hypernatremia after having growth hormone replacement for growth hormone deficiency. The patient visited emergency room complaining of generalized weakness, tremor in both legs, and poor oral intake including water after starting growth hormone replacement therapy. Laboratory test revealed serum sodium 168 mmol/L and serum osmolality 329 mOsm/kg, despite the patient didn't feel any thirst at all. We treated him with scheduled water intake of 2.5 L a day with intranasal vasopressin. He admitted to Soonchunhyang University Gumi Hospital and Soonchunhyang University Seoul Hospital for 4 times during the following 8 months and serum sodium level and osmolality was controlled by scheduled water intake combined with intranasal vasopressin treatment. It is still unclear whether growth hormone replacement worked as a trigger of hypernatremia.

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.

Giant Sellar Xanthogranuloma after Surgical Treatment of Symptomatic Rathke's Cleft Cyst

Extremely massive sellar xanthogranuloma (XG) are rare, and the surgical outcome and prognosis are not well known. XG remain unknown whether they are derived from Rathke's cleft cysts (RCCs) or craniopharyngiomas (CPs) following extensive inflammation and metaplasia, to the point that no epithelium is readily identifiable. These lesions usually tend to occur in younger patients (mean 28.3 years), have a smaller diameter, and remain primarily intrasellar region with infrequent calcification. This 36-year-old man presented our hospital with visual deterioration. At the time of visit, there were no neurological problems other than visual field defect and hormonal disorder. He visited our hospital in 2007 due to headache and decreased vision, and underwent transphenoid surgery for pituitary RCC. Since then, he has received treatment at our hospital for postoperative hormonal disorders. Through preoperative imaging study, the author suspected CP and underwent surgery. During the operation, the adhesion of the tumor to the surrounding major neurovascular structures was severe in the naked eyes, but the tumor could be removed more easily than expected. The postoperative histological findings were confirmed as XG. The postoperative course was uneventful. Compared to the previous literature, this case is a case where the size of XG is very large in a sellar region and it can be proved that it originated from the RCC. And regular follow-up is necessary to confirm the prognosis after surgery.

Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report

Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.

Cirugía endoscópica avanzada de base de cráneo, cavidad nasal y senos paranasales: experiencia de 40 meses en un hospital universitario / Advanced endoscopic surgery of skull base, nasal cavity and paranasal sinus: experience of 40 months in a university hospital / Cirugia endoscopica avançada de base de craneo, cavidade nasal e seios paranasales: experiência de 40 meses num hospital universitário

INTRODUCCIÓN: El uso del endoscopio junto con el avance de la tecnología ha facilitado la extensión de los límites tradicionales en la cirugía de la base del cráneo, la cavidad nasal y los senos paranasales. OBJETIVO: Describir los hallazgos epidemiológicos, clínicos e histopatológicos en los pacientes intervenidos por cirugía endoscópica avanzada en una unidad de Otorrinolaringología en 40 meses. MATERIAL Y MÉTODOS: Estudio retrospectivo. Los protocolos quirúrgicos e historias clínicas fueron revisados. Se realizó un análisis univariado descriptivo con aplicación de test de Chi-cuadrado para significancia estadística (p<0,05)...

INTRODUCTION: The use of the endoscope and the advance of the technology has facilitated the extension of the traditional limits in the surgery of skull base, nasal cavity and paranasal sinuses. OBJECTIVE: To describe the epidemiologic, clinical and histopathological findings in patients intervened under advanced endoscopic surgery in an Otorhinolaryngology unit in 40 months. MATERIAL AND METHODS: Retrospective study. Surgical protocols and medical records were reviewed. A descriptive univariate analysis was performed with the application of Chi-square test for statistical significance (p <0.05)...

INTRODUÇÃO: O uso do endoscópio junto com o avanço da tecnologia facilitou a extensão dos limites tradicionais na cirurgia da base do crânio, a cavidade nasal e os seios paranasais. OBJETIVO: Descrever os achados epidemiológicos, clínicos e histopatológicos em pacientes submetidos a cirurgia endoscópica avançada em uma unidade de Otorrinolaringologia em 40 meses. MATERIAL E MÉTODOS: Estudo retrospectivo. Os protocolos cirúrgicos e os registros médicos foram revisados. Uma análise descritiva univariada foi realizada com a aplicação do teste Qui-quadrado para significância estatística...

Resonancia magnética en pacientes con tumores más frecuentes en la región selar / Magnetic resonance in patients with more frequent tumors in the selar region

La resonancia magnética constituye el examen ideal en pacientes con presunción clínica de tumores en la región selar, pues no emite radiaciones ionizantes y debido a su visión multiplanar y discriminación tisular, posibilita adoptar una conducta terapéutica adecuada y un seguimiento sin riesgos en estos casos. Se describen las imágenes obtenidas con esa técnica sobre los principales tumores selares, correspondientes a macroadenomas hipofisarios, meningioma intraselar, craneofaringioma supraselar y como caso interesante perteneciente a esta región: el cordoma del clivus, por poseer en la actualidad mejores posibilidades diagnósticas que en décadas anteriores

Magnetic resonance constitutes the ideal exam in patients with clinical presumption of tumors in the selar region, because it doesn't emit ionizing radiations and due to its multiplanar vision and tissular discrimination, it facilitates the adoption of an appropriate therapeutic behavior and a follow up without risks in these cases. The images obtained with that technique on the main selar tumors are described, corresponding to hypophysary macroadenomas, intraselar meningioma, supraselar craneopharyngioma and as an interesting case belonging to this region: the clivus chordoma, for having better diagnostic possibilities at the present time than in previous decades

Postoperative otorhinolaryngologic complications in transnasal endoscopic surgery to access the skull base / Complicações pós-operatórias otorrinolaringológicas em cirurgias endoscópicas transnasais para acesso a base do crânio

Abstract Introduction: The large increase in the number of transnasal endoscopic skull base surgeries is a consequence of greater knowledge of the anatomic region, the development of specific materials and instruments, and especially the use of the nasoseptal flap as a barrier between the sinus tract (contaminated cavity) and the subarachnoid space (sterile area), reducing the high risk of contamination. Objective: To assess the otorhinolaryngologic complications in patients undergoing endoscopic surgery of the skull base, in which a nasoseptal flap was used. Methods: This was a retrospective study that included patients who underwent endoscopic skull base surgery with creation of a nasoseptal flap, assessing for the presence of the following post-surgical complications: cerebrospinal fluid leak, meningitis, mucocele formation, nasal synechia, septal perforation (prior to posterior septectomy), internal nasal valve failure, epistaxis, and olfactory alterations. Results: The study assessed 41 patients undergoing surgery. Of these, 35 had pituitary adenomas (macro- or micro-adenomas; sellar and suprasellar extension), three had meningiomas (two tuberculum sellae and one olfactory groove), two had craniopharyngiomas, and one had an intracranial abscess. The complications were cerebrospinal fluid leak (three patients; 7.3%), meningitis (three patients; 7.3%), nasal fossa synechia (eight patients; 19.5%), internal nasal valve failure (six patients; 14.6%), and complaints of worsening of the sense of smell (16 patients; 39%). The olfactory test showed anosmia or hyposmia in ten patients (24.3%). No patient had mucocele, epistaxis, or septal perforation. Conclusion: The use of the nasoseptal flap has revolutionized endoscopic skull base surgery, making the procedures more effective and with lower morbidity compared to the traditional route. However, although mainly transient nasal morbidities were observed, in some cases, permanent hyposmia and anosmia resulted. An improvement in this technique is therefore necessary to provide a better quality of life for the patient, reducing potential complications.

Resumo Introdução: O grande crescimento no número de cirurgias endoscópicas transnasais para a base do crânio ocorreu a partir de um maior conhecimento anatômico da região; do desenvolvimento de materiais e instrumentais específicos e, principalmente, após o uso do retalho nasosseptal como uma barreira entre o trato sinusal (cavidade contaminada) e o espaço subaracnóideo (área estéril), com redução de grandes riscos de contaminação. Objetivo: Avaliar as complicações otorrinolaringológicas nos pacientes submetidos à cirurgia endoscópica da base do crânio, na qual foi usado o retalho nasoseptal. Método: Estudo retrospectivo, no qual foram avaliados os pacientes submetidos à cirurgia da base do crânio por via endoscópica com retalho nasosseptal, quanto à presença no pós-operatório das seguintes complicações: fístula liquórica, meningite, formação de mucocele, sinéquia nasal, perfuração septal (anterior à septectomia posterior), insuficiência de válvula nasal interna, epistaxe e alteração olfatória. Resultados: Foram avaliados 41 pacientes submetidos à cirurgia. Desses, 35 eram portadores de adenomas hipofisários (macro ou microadenomas; selares e extensão supraselar), três meningiomas (dois de tubérculo selar e um da goteira olfatória), dois craniofaringiomas e um abscesso intracraniano. As complicações observadas foram: fístula liquórica (três pacientes - 7,3%), meningite (três pacientes - 7,3%), sinéquia em fossa nasal (oito pacientes - 19,5%), insuficiência de válvula nasal interna (seis pacientes - 14,6%) e queixa de pioria do olfato (16 pacientes - 39%). O teste olfatório evidenciou anosmia ou hiposmia em 10 pacientes (24,3%). Nenhum paciente apresentou mucocele, epistaxe ou perfuração septal. Conclusão: O uso do retalho nasosseptal proporcionou uma revolução na cirurgia da base do crânio por via endoscópica e tornou os procedimentos mais eficazes e com baixa morbidade, comparado com a via tradicional. Porém, passou a ocasionar morbidades nasais principalmente transitórias, mas em alguns casos permanentes, como hiposmia e anosmia. Assim, torna-se necessário um aperfeiçoamento dessa técnica para proporcionar uma melhoria na qualidade de vida do paciente e diminuir possíveis complicações.

Asociación de craneofaringioma y síndrome de Klinefelter en la transición puberal: un desafío diagnóstico / Craniopharyngioma and Klinefelter syndrome during the pubertal transition: A diagnostic challenge

Los craneofaringiomas son de los tumores hipofisarios más frecuentes en la niñez y, sea por su evolución o por el tratamiento que requieren, pueden comprometer el desarrollo puberal. El síndrome de Klinefelter es la causa más frecuente de hipogonadismo hipergonadotrópico en el varón. La presentación concomitante de ambas entidades es extremadamente baja (1/10(9)) y plantea un interrogante acerca de una probable asociación fisiopatológica. Se presenta el caso de un paciente belga de 18 años, con diagnóstico de craneofaringioma en la niñez y panhipopituitarismo luego del tratamiento quirúrgico y radioterápico. Al llegar a los 14 años, se inició la inducción puberal con gonadotropinas. Ante la falta de respuesta clínica, se completó una evaluación genética, que evidenció, de manera homogénea, una trisomía XXY. La falta de respuesta al tratamiento de inducción con gonadotropina exógena reveló la asociación de hipogonadismo primario y secundario, que demostró la importancia del seguimiento multidisciplinario que estos pacientes requieren.

Craniopharyngioma is the most common pituitary tumor in childhood. It can compromise the pubertal development because of its evolution or treatment. Syndrome of Klinefelter is the most common cause of hipergonadotrophic hypogonadism in males. The concomitant presentation of both entities is extremely low (1/10(9)) and the pathophysiological association is questionned. We present the case of a 18-year-old Belgian patient. He had a diagnosis of craniopharyngioma in childhood and he presented with panhypopituitarism after radiotherapy and surgical treatment. At the age of 14, he started pubertal induction with gonadotropin therapy without clinical response. A genetic evaluation confirmed a homogeneous 47, XXY karyotype. Failure of exogenous gonadotropin therapy revealed the hidden association of primary and secondary hypogonadism, demonstrating the importance of the followup and a multidisciplinary approach in these patients.

Evaluación nutricional de la población con antecedente de craneofaringioma infantil en el Hospital "Prof. Dr. Juan P. Garrahan" / Nutritional assessment of a population with a history of childhood craniopharyngioma seen at Hospital "Prof. Dr. Juan P. Garrahan"

Introducción. los craneofaringiomas son malformaciones histológicamente benignas entre el hipotálamo y la hipófisis que pueden afectar la secreción hormonal clave en la regulación endócrina y modulación de la saciedad. Si bien se trata de una enfermedad relativamente benigna, la combinación de obesidad grave de origen hipotalámico y las comorbilidades asociadas a ella disminuyen la calidad de vida. Objetivo. Evaluar el estado nutricional de pacientes operados de craneofaringioma infantil. Población y métodos. Pacientes menores de 21 años al momento del estudio que requirieron cirugía por craneofaringioma en el Hospital de Pediatría Garrahan y que firmaron el consentimiento informado. Se realizó la evaluación antropométrica, composición corporal con impedanciometría, gasto energético con calorimetría indirecta e ingesta energética. Se determinó la resistencia a la insulina y la dislipidemia. Resultados. Se incluyeron 39 pacientes; 41%, normopeso y 59%, obesos. El 68% de los pacientes presentó distribución grasa central; 40%, resistencia a la insulina; y 32%, dislipidemia. No se encontraron diferencias significativas en la presencia de resistencia a la insulina, dislipidemia, gasto energético en reposo ni en la ingesta entre obesos vs. normopeso. El 77% de los pacientes obesos presentó bajo gasto energético independiente del porcentaje de masa magra (62 ± 2,7% vs. 61,2 ± 1,8% de gasto energético en reposo normal vs. bajo; p 0,8). Conclusiones. El 59% de la población estudiada presentó obesidad. No se encontraron diferencias significativas en complicaciones metabólicas entre pacientes obesos y normopeso. Se observó menor gasto energético independiente del porcentaje de masa magra y similar ingesta energética.

Introduction. Craniopharyngiomas are histologically benign malformations located between the pituitary and hypothalamus that may affect key hormone secretion for endocrine regulation and satiety modulation. Although this is a relatively benign disease, the combination of severe hypothalamic obesity and associated comorbidities results in a reduced quality of life. Objective. To assess the nutritional status of patients after craniopharyngioma surgery. population and Methods. Patients younger than 21 years old at the time of the study who required craniopharyngioma surgery at Hospital de Pediatria Garrahan and who signed an informed consent. Anthropometric characteristics, body composition by impedance analysis, energy expenditure by indirect calorimetry and energy intake were assessed. Insulin resistance and dyslipemia were estimated. Results. A total of 39 patients were included; 41% had a normal weight and 59% were obese. Overall, 68% of patients had a central fat distribution; 40% had insulin resistance; and 32%, dyslipemia. No significant differences were observed in terms of insulin resistance, dyslipemia, energy expenditure at rest, or energy intake between normal weight and obese patients. Among obese patients, 77% had a low energy expenditure, regardless of their percentage of lean body mass (62 ± 2.7% versus 61.2 ± 1.8% of normal versus low energy expenditure at rest; p = 0.8). Conclusions. A total of 59% of the studied population was obese. No significant differences were observedinterms ofmetabolic complications between normal weight and obese patients. A lower energy expenditure was observed, regardless of the lean body mass percentage and a similar energy intake.

Differential Expression of Stem Cell Markers in Human Adamantinomatous Craniopharyngioma and Pituitary Adenoma

Background/Aims: Although craniopharyngioma (CP) is histologically benign, it is a pituitary tumour that grows rapidly and often recurs. Adamantinomatous CP (ACP) was associated with an activating mutation in ß-catenin, and it has been postulated that pituitary stem cells might play a role in oncogenesis in human ACP. Stem cells have also been identified in pituitary adenoma. Our aim was to characterize the expression pattern of ABCG2, CD44, DLL4, NANOG, NOTCH2, POU5F1/OCT4, SOX2, and SOX9 stem cell markers in human ACP and pituitary adenoma. Methods and Results: We studied 33 patients (9 ACP and 24 adenoma) using real-time quantitative PCR (RT-qPCR) and immunohistochemistry. SOX9 was up-regulated in ACP, exhibiting positive immunostaining in the epithelium and stroma, with the highest expression in patients with recurrence. CD44 was overexpressed in ACP as confirmed by immunohistochemistry. SOX2 did not significantly differ among the tumour types. The RT-qPCR array showed an increased expression of MKI67,OCT4/POU5F1, and DLL4 in all tumours. NANOG was decreased in ACP. ABCG2 was down-regulated in most of the tumours. NOTCH2 was significantly decreased in the adenomas. Conclusion: Our results confirm the presence of stem cell markers in human pituitary tumours as well as the different expression patterns of ACP and adenoma. These findings suggest that ACP may originate from a more undifferentiated cell cluster. Additionally, SOX9 immunodetection in the stroma and the highest expression levels related to the relapse of patients suggest a contribution to the aggressive behaviour and high recurrence of this tumour type.