Complications and visual outcomes following surgical resection of pediatric optic pathway/hypothalamic gliomas: a systematic review and meta-analysis.

Pediatric optic pathway/hypothalamic gliomas (OPHG) pose challenges in treatment due to their location and proximity to vital structures. Surgical resection plays a key role in the management of OPHG especially when the tumor exhibits mass effect and causes symptoms. However, data regarding outcomes and complications of surgical resection for OPHG remains heterogenous. The authors performed a systematic review on pediatric OPHG in four databases: PubMed, EMBASE, Cochrane Library, and Google Scholar. We included studies that reported on the visual outcomes and complications of OPHG resection. A meta-analysis was performed and reported per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A total of 26 retrospective studies were included. Seven hundred ninety-seven pediatric patients with OPHG undergoing surgical resection were examined. A diagnosis of NF1 was confirmed in 9.7%. Gross total resection was achieved in 36.7%. Intraorbital optic pathway gliomas showed a significantly higher gross total resection rate compared to those located in the chiasmatic/hypothalamic region (75.8% vs. 9.6%). Postoperatively, visual acuity improved in 24.6%, remained unchanged in 68.2%, and worsened in 18.2%. Complications included hydrocephalus (35.4%), anterior pituitary dysfunction (19.6%), and transient diabetes insipidus (29%). Tumor progression post-resection occurred in 12.8%, through a mean follow-up of 53.5 months. Surgical resection remains an essential strategy for treating symptomatic and large pediatric OPHG and can result in favorable vision outcomes in most patients. Careful patient selection is critical. Patients should be monitored for hydrocephalus development postoperatively and followed up to assess for tumor progression and adjuvant treatment necessity.

Comparison of MRI findings of hypothalamic-optic chiasmatic gliomas and craniopharyngiomas.

BACKGROUND: Differential diagnosis of hypothalamic-optic chiasmatic gliomas (HOCGs) and craniopharyngiomas on magnetic resonance imaging (MRI) can be quite challenging. PURPOSE: To compare the MRI features of HOCGs and cranipharyngiomas. MATERIAL AND METHODS: Patients diagnosed with HOCG or craniopharyngioma in histopathological evaluation between 2012 and 2022 and who underwent preoperative contrast-enhanced brain MRI were included. Various MRI features were retrospectively evaluated for each lesion: T2-weighted imaging and fluid attenuation inversion recovery hyperintensity, calcification, cystic change, T1-weighted (T1W) imaging hyperintensity of the cystic component, hemorrhage, involvement of sellar, suprasellar or other adjacent structures, lobulated appearance, presence of hydrocephalus, and contrast enhancement pattern. Apparent diffusion coefficient (ADC) values were also evaluated and compared. RESULTS: Among 38 patients included, 13 (34%) had HOCG and 25 (66%) had craniopharyngioma. Craniopharyngiomas had a significantly higher rate of cystic changes, calcification, and T1W imaging hyperintensity of the cystic component than HOCGs (P <0.05). Of HOCGs, 92% had chiasm involvement, 23% had optic nerve involvement, and 31% had brain stem involvement. On the other hand, chiasm involvement was observed in 8% of craniopharyngiomas, but none had optic nerve and/or brain stem involvement (P <0.05). While 62% (8/13) of HOCGs had diffuse homogeneous enhancement, 80% (20/25) of craniopharyngiomas had a diffuse heterogeneous enhancement pattern. Mean ADC values were significantly higher in craniopharyngiomas compared to HOCGs (2.1 vs. 1.6 ×10-3mm2/s, P <0.05). CONCLUSION: Although some neuroimaging findings may overlap, features such as presence of cyst and calcification, brain stem and optic pathway involvement, different enhancement patterns, and ADC values may be helpful in the differential diagnosis of HOCGs and craniopharyngiomas.

Pilomyxoid Astrocytoma: Case Report

The pilomyxoid astrocytoma (PMA) is a rare glioma recently described as a separate entity, which is generally located on the hypothalamic area. The PMA was previously described as pilocytic astrocytoma (PA) due to similarities shared between them. Recent studies provided a deeper understanding of PMA, setting it as a separate entity, though PMA is still considered by many authors a variant of PA. The PMA is considered to be more aggressive than PA; however, further studies are necessary for a better comprehension of its behavior and, hence, for neurosurgeons and neurologists to get to a consensus about its management. This study presents a 16-year-old female patient who looked for medical assistance complaining of headaches of over 6 months and vomiting for 2 weeks prior to the visit to the doctor. She presented no other symptoms. The physical examination displayed only bilateral papilledema. The magnetic resonance imaging (MRI) scans showed an intraventricular and thalamic lesion composed of solid and cystic material associated with peritumoral edema. The patient underwent ventriculoperitoneal shunt and subtotal resection of the lesion. The histological and immunohistochemical studies showed typical features of PMA. The patient started adjuvant therapy with chemotherapy and radiosurgery. She has been asymptomatic for 9 months and has shown no signs of progression of the disease on the follow-up scans.

O astrocitoma pilomixoide (APM) é um raro glioma, recentemente descrito como uma entidade separada, que geralmente se localiza na região hipotalâmica. Anteriormente, o APM era descrito como astrocitoma pilocítico (AP) devido a características semelhantes que ambos apresentam. Estudos recentes permitiram um melhor entendimento do APM, configurando-o como uma entidade separada, embora o APM ainda seja considerado por muitos autores uma variante do AP. O APM é considerado mais agressivo que o AP; no entanto, mais estudos são necessários para um melhor entendimento do comportamento do tumor e, consequentemente, para que neurocirurgiões e neurologistas cheguem a um consenso sobre sua terapêutica. Este estudo apresenta uma paciente de 16 anos que procurou atendimento médico com queixas de dores de cabeça por mais de 6 meses e vômitos nas 2 semanas antecedentes à visita ao médico. Outros sintomas não eram apresentados. O exame físico revelou apenas papiledema bilateral. As imagens de ressonância magnética mostraram uma lesão intraventricular e hipotalâmica de componentes sólido e cístico associados a edema peritumoral. A paciente foi submetida a derivação ventriculoperitoneal e a ressecção subtotal da lesão. Os estudos histológico e imunohistoquímico demonstraram características típicas de APM. A paciente iniciou terapia adjuvante com quimioterapia e radiocirurgia. A paciente está em acompanhamento por 9 meses e, até o momento, manteve-se assintomática e não houve sinais de progressão da doença nos exames de imagem.

Mixed germ cell tumor of the pituitary-hypothalamic region presenting as craniopharyngioma: case report and review of the literature / Tumor misto de células germinativas da região hipotálamo-hipofisária apresentando-se como craniofaringioma: relato de caso e revisão da literatura

Craniopharyngiomas and germ cell tumors (GCT) may affect the pituitary-hypothalamic region during childhood. Although different in origin, their clinical and radiological features may be similar. In this article we present a 5-year-old girl with clinical and radiological findings (computer tomography calcification) that were initially considered as craniopharyngioma. However clinical outcome, blood and cerebral spinal fluid tumoral markers, and results from anatomopathology and immunohistochemistry disclosed a mixed GCT. This case report highlights that some clinical features and radiological findings of pituitary-hypothalamic tumors may be misdiagnosed as craniopharyngioma mainly when there is a mature teratoma with cartilaginous tissue differentiation.

Craniofaringiomas e tumores mistos de células germinativas (TCG) podem acometer a região hipotálamo-hipofisária durante a infância. Embora tenham diferentes origens, as manifestações clínicas e achados radiológicos podem ser semelhantes. Nosso objetivo é relatar o caso de uma paciente de 5 anos de idade, cujas manifestações clínicas e achados radiológicos (presença de calcificações à tomografia computadorizada [TC]) foram inicialmente considerados como provável craniofaringioma. No entanto, a piora clínica progressiva, marcadores tumorais séricos e liquóricos elevados, assim como os resultados do estudo anatomopatológico e imunoistoquímico revelaram tratar-se de TCG. Este caso enfatiza que alguns achados clínicos e radiológicos de tumores da região hipotálamo-hipofisária podem ser erroneamente diagnosticados como craniofaringiomas, principalmente se houver presença de teratoma maduro com diferenciação em tecido cartilaginoso.

Pituitary tumor diagnosis using gadolinium-enhanced magnetic resonance imaging in a dog with hyperadrenocorticism. A case report

Cushing’s disease is a common endocrine disorder of dogs, caused by persistent high concentration of cortisol in circulating blood. Almost eighty five percent of the cases of spontaneous hyperadrenocorticism in dogs are the result of a functional pituitary tumor or hyperplasia. Advanced imaging techniques like computed tomography (CT) or magnetic resonance imaging (MRI) are the best means to obtain a diagnosis of a pituitary tumor in dogs with pituitary-dependent hyperadrenocorticism (PDH). Moreover these imaging techniques are required to asses the size and location of the pituitary tumor when planning surgical removal of the tumor mass or of the complete pituitary gland. In Colombia, the use of these imaging techniques is very limited in the small animal practice, but it has recently become available for veterinary patients thanks to agreements with human clinical institutions. This report describes the case of an eleven year-old golden retriever with PDH as a result of a functional pituitary tumor, visualized by magnetic resonance imaging using gadolinium as contrast agent.

Infundibulo neurohipofisitis linfoplasmocitaria con compromiso quiasmatico e hipotalamico. Presentacion de un caso / Linfoplasmocitary infundibulohipophisitis with chiasmal and hypothalamic involvement. Case presentation

Objective: to present a 38 year old female patient, with central diabetes insipidus, panhipopituitarims, and severely impaired vision. Description: magnetic resonance imaging demonstrated a large mass involving the hypothalamus, infundibulum, optic nerves, and chiasm. Intervention: at surgery the optic pathways were found to be grossly involved within the inflammatory mass. Histological examination demonstrated anonspecific, mixed inflammatory infiltrate, composed predominantly of lymphocytes and plasma cells. She responded dramatically to dexamethasone, with mass reduction on serial imaging studies and vision improvement. In addition, she received hormone replacement therapy. Infundibulohypophisitis is a rare disease. Surgical biopsy and dexamethasone were an effective treatment

Hamartoma hipotalámico, una causa de pubertad precoz: caso clínico / Hypothalamic hamartoma causing precocious puberty: report of 1 case

Hypothalamic hamartomas are non neoplastic lesions that may cause precocious puberty with or without complex seizures, personality disorders and mental retardation. We report a 14 years old male that had a precocious puberty at the age of 11 and a prolonged episode of altered sensorium with automatism, that was diagnosed as a complex seizure. Physical examination showed a sexual development classified as Tanner stage III-IV, a height of 168 cm and a weight of 61 kg. Neurological examination was normal. A CAT scan showed a 13x13x9 mm mass in the suprasellar cistern, between the infundibulum and the brain stem, without exerting a mass effect over adjacent structures. It was diagnosed as an hypothalamic hamartoma

Acesso sub-frontal a lesões selares e para-selares / Subfrontal approach in sellar and suprasellar injuries

Os processos expansivos situados ao nível da região selar constituem um variável número de patologias que, apesar da localização semelhante, têm características clínicas, radiológicas, patológicas e origens diferentes. Assim sendo, diferentes tipos de abordagens são utilizados no tratamento cirúrgico destas lesões. Dentre os acessos transcranianos convencionais e os mais sofisticados acessos à base do crânio, o acesso sub-frontal proporciona visão direta das estruturas neurovasculares da região. Este estudo descreve 19 tumores operados por esta via, no período compreendido de fevereiro de 1997 a março de 1998. Onze destas lesões eram adenomas pituitários, 3 eram craniofaringiomas, 2 eram meningiomas, 1 germinoma e 1 lesão inflamatória do nervo óptico. A remoção total dos tumores foi conseguida em 17 casos. Não houve mortalidade operatória e as complicações mais comuns foram: anosmia unilateral, que ocorreu em 12 casos; diabetes insipidus transitório, que acometeu 4 pacientes; e déficit isquêmico progressivo, que ocorreu em 1 paciente. Estes resultados demonstram que a via sub-frontal é uma opção segura para a cirurgia dos tumores desta região.

GnRH neurones population in the diencephalon of the coypu myocastor coypus

El objetivo del presente estudio fue investigar la distribución y morfología de las neuronas que sintetizan y almacenan el factor de liberación de gonadotrofinas (GnRH), en el diencéfalo del coipo (Myocastor coypus), roedor sudmericano del suborden histricomorpha. Para tales fines los encéfalos de tres coipos machos, adultos, fueron fijados por perfusión intra-arterial, utilizando una solución de paraformaldehído y ácido pícrico. Los bloques del hipotálamo fueron separados del resto del encéfalo y seccionados en micrótomo de congelación, obteniendo láminas coronales de 40 µm de espesor. Las secciones fueron sometidas a un proceso de recuperación antigénica por ultrasonido, para desenmascarar los antígenos ocultos, luego las secciones fueron procesadas utilizando una técnica de inmunohistoquímica para evidenciar las neuronas GnRH, usando un anticuerpo monoclonal (LRH 13). Un grupo de secciones seriadas fue coloreada con violeta de cresilo (técnica de NissI), mientras que las otras secciones fueron coloreadas usando el método de Kuver Barrera (azul luxol rápido y violeta de cresilo), para observar los núcleos y tractos nerviosos del hipotálamo, con el microscopio óptico. Los análisis morfométrico y cuantitativo de los cuerpos neuronales fue realizado usando un analizador de imágenes. Las neuronas GnRH inmunoreactivas observadas eran bipolares y alargadas. El número total de cuerpos neuronales para esta especie fue estimado en 1072 ñ 27. Todas las células fueron localizadas en el hipotálamo rostral, principalmente en el área preóptica. Pocas neuronas fueron observadas en el núcleo de la estría terminal y en el núcleo medial preóptico hipotalámico. Las fibras inmunorreactivas fueron visibles en la capa externa de la eminencia media. De acuerdo a los datos obtenidos, se concluye que la distribución de las neuronas GnRH en el coipo coinciden con la de otros roedores, tales como la rata

Hypothalamic pilocytic astrocytoma and subarachnoid hemorrhage: case report

In this report, we present a case of a young women with a pilocytic astrocytoma in the chiasmo-hypothalamic region, in which the clinical presentation was subarachnoid hemorrhage

Polysyndactyly, gelastic seizures, hypothalamic hamartoma and precocious puberty - a variant of Pallister-Hall syndrome?

Os autores relatam o caso de uma menina portadora de polissindactilia, que apresenta crises gelásticas desde o período neonatal. Aos dois anos de idade, foi detectada, através da ressonância magnética, lesäo hipotalâmica sugestiva de hamartoma. Logo após, a partir do surgimento de pelos pubianos, foi diagnosticada puberdade precoce. As características mencionadas sugerem tratar-se de uma variante da síndrome de Pallister-Hall. A conduta terapêutica tem sido conservadora, apesar da resposta pobre aos anticonvulsivantes.

Puberdade precoce central por tumor hipotalâmico / Central precocious puberty by hypothalamic tumors

Descrevemos o caso de um menino com três anos de idade e puberdade precoce central por tumor hipotalâmico (hamartoma). O diagnóstico de processo expansivo do sistema nervoso central foi estabelecido pela tomografia computadorizada e ressonância magnética de crânio. As alteraçöes hormonais mais importantes na investigaçäo foram a elevaçäo do nível sérico de testosterona e a resposta das gonadotrofinas ao hormônio liberador das gonadotrofinas (GnRH). O tratamento com o agonista do GnRH, após a cirurgia, tornou-se útil para bloquear a progressäo da precocidade central.

Hypothalamic tumor associated with atypical forms of anorexia nervosa and diencephalic syndrome

We report the case of a 10-year-old girl with a mature teratoma in the hypothalamic region. The patient presented a 2-month history of anorexia, psychic disturbances and a 37% loss of body weight. These symptoms had led initially to a diagnosis of major depression and atypical anorexia nervosa. She also presented some signs and symptoms of diencephalic syndrome. This case illustrates the importance of considering a slow-growing mass as a rare but real possibility in the differential diagnosis of anorexia nervosa, mainly in atypical cases.

Sindrome de Raeder e tumor hipotalamico / Raeder Syndrome and hypothalamic tumor

Os autores apresentam um caso de sindrome paratrigeminal de Raeder associada com um tumor (oligodendroglioma) hipotalamico com expansao para-selar. A literatura relevante abordando a incidencia, etiopatogenia e sintomatologia e revisada.

Desconexión interhemisférica en tumor del cuerpo calloso / Interhemispheric disconnection by corpus callosum tumor

Se presenta un paciente con un tumor del cuerpo calloso con obnubilación, amnesia de tipo axial y una desconexión interhemisférica somestésica y visual (hemialexia y afasia hemianóptica). A pesar de ser zurdo, la evidencia clínica sugiere que el hemisferio especializado para el lenguaje era el izquierdo. Se plantea que este hemisferio tiene capacidad de juicio lógico y que el derecho tiene capacidad de juicio de realidad

Aspectos clínicos y neuroendócrinos de un granuloma hipotalámico (TBC) y neurolués / Clinical and neuroendocrine aspects of an hypothalamic tuberculous granuloma and neurosyphilis

Se discuten los hallazgos en un paciente de 36 años, sexo masculino, homosexual que comienza, 6 meses antes de la consulta, con un cuadro de diabetes insípida al que se le agregan, 3 meses después, astenia, adinamia, somnolencia, diplopía fugaz, cefaleas parieto-occipitales, bradispsiquia y alucinaciones auditivo-visuales. Como antecedentes personales de importancia refería traumatismo severo de ojo derecho (a los 10 años) y osteomielitis tuberculosa de clavícula izquierda (a los 29 años). Examen físico de ingreso: temblor fino distal en miembro superior derecho y hemiparesia en miembro inferior homolateral. Laboratorio: anemia leve normocítica-normocrómica; eritrosedimentación acelerada; incremento moderado de GPT, GOAT, y GTP y F. Alcalina; hiposmolaridad plasmática; hipostenuria; VDRL y FTA reactivos (plasma y LCR). T.A.C. lesión expansiva supraselar, yuxtaventricular con gran edema perilesional. Estudios neuroendocrinos: alteración del ritmo circadiano y de la respuesta a la hipoglucemia insulínica de la prolactina y cortisol; biporespuesta de LH, FSH y TSH a estímulos específicos. Punción biopsia estereotáxica: granuloma inflamatorio inespecífico. A los 45 días de inicio de tuberculostáticos y dexametasona se efectuá nueva TAC: normal, desparece la diabetes insípida y se instaura penicilinoterapia. Nueva TAC (al año): normal. Como conclusiones merecen destacarse la presentación de un granuloma hipotalámico tuberculoso y diabetes insípida de curación clínica y desparición tomográfica en breve plazo asociado a neurolúes y disfunción hipotálamo-hipofisaria conformando un hallazgo inusual en la literatura médica