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.

Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.

Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.

Hypothalamic hamartoma presenting with gelastic seizures, generalized convulsions, and ictal psychosis

We report a case of hypothalamic hamartoma in an adult female who presented with gelastic seizures, generalized convulsions, and ictal aggressive psychotic behavior. Anticonvulsant treatment was ineffective in controlling the epileptic seizures. Surgical excision after accurate imaging diagnosis 3 decades after the onset of symptoms markedly ameliorated her condition. Delayed and erroneous diagnosis had unnecessarily prolonged the suffering of our patient

Clinical and imaging features of hypothalamic hamartoma in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study clinical and imaging features of hypothalamic hamartoma in children.</p><p><b>METHODS</b>Imaging findings and clinical manifestations of 38 children with hypothalamic hamartomas were retrospectively reviewed. The patients included 25 boys and 13 girls, ranging in age of onset from 1 month to 15 years. All the 38 patients were examined with pre-contrast and post-contrast T1 weighted MR imaging and with non-contrast T2 weighted MR imaging. Meanwhile, 10 patients received CT scan. Hypothalamic hamartomas were confimed by pathologic examinations in the 38 patients.</p><p><b>RESULTS</b>Gelastic epilepsy, precocious puberty, other types of epilepsy and disturbance of intelligence were main manifestations in the 38 patients. Gelastic epilepsy was found as an initial clinical symptom in 17 cases, precocious puberty in 13 cases and other types of epilepsy in 8 cases. All masses were located in the suprasellar and interpeduncular cistern. They showed iso-density on CT scan, and homogeneous signal iso-intense to gray matter on T1 and T2 weighted MR images. With contrast MR images, there was no enhancement in the mass. The size or pedunculation of the mass was not correlated with clinical features.</p><p><b>CONCLUSIONS</b>Hypothalamic hamartoma may be characterized by precocious puberty and/or gelastic epilepsy and specific imaging findings mentioned above.</p>

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

A case of fever of unknown origin after removal of hypothalamic tumor / 대한내과학회지

The preoptic area and anterior hypothalamus plays a pivotal role in body temperature regulation, and damage in this region causes hyperthermia. This hyperthermia is particularly troublesome because of the possibility that it may reflect an occult infectious process. We report a case of fever of unknown origin in a patient after removal of neoplasm involving the hypothalamus. A 29-year old man underwent craniotomy and removal of hypothalamic choroid meningioma. Seventy days after the removal of his tumor, his body temperature began to rise. But, there was no evidence of infection, inflammatory disease, metabolic disease, drug fever and recurred tumor. Repeated administration of antipyretic agent did not reduce body temperature. So, we considered that the elevated temperature had a central basis. The patient was treated with chlorpromazine in an attempt to lower his temperature. This drug reduced successfully his body temperature.

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

Gelastic epilepsy--a case report with SPECT studies.

A 24 years male presented with daily episodes of uncontrollable laughter followed by urinary incontinence since the age of nine years. Some of these attacks progressed to generalized tonic-clonic seizures. General and neurological examination did not reveal any abnormality. Ictal and interictal video EEGs were normal. MRI showed a hypothalamic hamartoma. Interictal SPECT scan showed normal perfusion in the hamartoma. SPECT scan obtained four minutes after beginning of seizure showed that the perfusion increased in right cingulate gyrus but not in the hamartoma, suggesting the involvement of the cingulate gyrus in the seizure origin or pathway.

Management of Chiasmatic-Hypothalamic Gliomas in Children

OBJECTIVE: Management strategies for pediatric chiasmatic-hypothalamic gliomas(CHG) include surgery, irradiation, chemotherapy and a combination of these modalities. This study was performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. MATERIAL AND METHOD: We have reviewed the results of management of 6 children with a diagnosis of CHG, who were observed closely during the last 8 years. The patients were aged 7 months to 15 years. Our patients presented with diencephalic syndrome, endocrine dysfunction, and progressive visual loss. None of these had evidence of neurofibromatosis-1. Treatment consisted of surgery alone(2), surgry and irradiation(2), surgery, irradiation and chemotherapy(1), and surgery and chemotherapy(1). RESULTS: Four children had large exophytic suprasellar tumors and two showed diffuse midline lesions. Obstructive hydrocephalus was present in all patients. Pathologic examination revealed anaplastic astrocytoma in 1 and low-grade astrocytoma in 5. Two patients, recently treated with radiation therapy following radical subtotal resection, showed significant tumor reductions and good clinical status. Four patients had partial tumor resection. Of these patients, two developed disease stabilization during follow-up period of 7 and 8 years, respectively. Life-threatening complications were noted in remaining two patients. CONCLUSION: CHG may follow an unpredicatable course and show a various reponse to each treatment modality. Further studies are indicated to define the optimal method of treatment of CHG in childhood.

Hypodipsic hypernatremia with intact AVP response to non-osmotic stimuli induced by hypothalamic tumor: A case report

Anatomical lesions of hypothalamic area associated with hypodipsic hypernatremia have been reported only rarely. We report here a case of hypodipsic hypernatremia induced by a hypothalamic lesion. A 25-yr-old man, who had been treated with radiation for hypothalamic tumor 5-yr before, was admitted for evaluation of hypernatremia and hypokalemia. He never felt thirst despite the elevated plasma osmolality and usually refused to drink intentionally. Plasma arginine vasopressin (AVP) level was normal despite the severe hypernatremic hyperosmolar state and urine was not properly concentrated, while AVP secretion was rapidly induced by water deprivation and urine osmolality also progressively increased to the near maximum concentration range. All of these findings were consistent with an isolated defect in osmoregulation of thirst, which was considered as the cause of chronic hypernatremia in the patient without an absolute deficiency in AVP secretion. Hypokalemia could be induced by activation of the renin-angiotensin-aldosterone system as a result of volume depletion. However, inappropriately low values of plasma aldosterone levels despite high plasma renin activity could not induce symptomatic hypokalemia and metabolic alkalosis. The relatively low serum aldosterone levels compared with high plasma renin activity might result from hypernatremia. Hypernatremia and hypokalemia were gradually corrected by intentional water intake only.

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.

A Case of Diencephalic Syndrome / 대한소아내분비학회지

Diencephalic syndrome is a rare cause of failure to thrive in infancy and early childhood. The syndrome is characterized by profound emaciation with normal appetite, loss of cutaneous adipose tissue, hyperactivity, euphoria, and nystagmus. It commonly occurs in association with chiasmatic and hypothalamic gliomas. It has also been described in association with other histologic types. There is the marked increase of serum growth hormone, which may exhibit an inappropriate, even paradoxical response in stimulation test. A male infant of 12 months of age, showed markedly elevated growth hormone but he had failure to thrive findings. Evenly enhanced round mass was seen at suprasella area in brain CT. Its histological findings was "Desmoplastic infantile ganglioglioma", very rare histologic type. Here we report a case of diencephalic syndrome presented by failure to thrive in association with hypothalamic tumors.

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

Progressive cerebral occlusive disease after hypothalamic astrocytoma radiation therapy.

An 18 year-old woman received radiation therapy for hypothalamic astrocytoma at the age of 11 years. She developed progressive cerebral occlusive vascular disease with moyamoya vessels formation in both carotid systems. Apart from diabetes mellitus, she had no other risk factors for occlusive cerebrovascular disease. The site of occlusion was confined to the field of radiation and the development of moyamoya vessels strongly suggestive of a radiation-induced cause. Radiation therapy around the sella and parasellar region appears to be the most common risk factor for this vasculopathy. Progressive irradiation-induced cerebral vasculopathy is due to accelerated atherosclerosis.

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.