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.

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.

Atualização terapêutica no tratamento dos craniofaringiomas / Therapeutic update on the treatment of craniopharyngiomas

O craniofaringioma é uma neoplasia de natureza benigna, pouco frequente, responsável por 1 por cento a 3 por cento de todos os tumores intracranianos, sendo a mais frequente neoplasia intracraniana não neuroepitelial na criança. Geralmente o tumor é restrito à região selar e ao III ventrículo, mas, em decorrência da infiltração e frequente aderência ao sistema nervoso central, apresenta comportamento clínico muitas vezes desfavorável, sendo classificado pela Organização Mundial de Saúde (OMS) como grau I, caracterizado como tumor de baixo ou incerto potencial de malignização. As sequelas endocrinológicas ganham destaque devido ao importante impacto na qualidade de vida dos pacientes, na maioria das vezes crianças. O hipopituitarismo e a obesidade hipotalâmica são complicações frequentes, sendo o tratamento desse tumor um grande desafio para endocrinologistas e neurocirurgiões. A combinação da cirurgia, radioterapia e aplicação de drogas e radioisótopos intratumorais tem como objetivo maximizar as chances de cura e tentar minimizar as sequelas pós-operatórias, mas, mesmo assim, a recidiva ainda é frequente. A escolha da modalidade de tratamento mais adequado para os craniofaringiomas é uma decisão difícil e que deve sempre ser individualizada para cada paciente. Com o objetivo de explorar as múltiplas opções terapêuticas para o craniofaringioma, foi realizada revisão na literatura com ênfase nas possibilidades terapêuticas e complicações inerentes ao tratamento dessa patologia.

Craniopharyngioma is an uncommon benign neoplasm, accounting for 1 percent-3 percent of all intracranial tumors, and the most common non-neuroepithelial intracranial neoplasm in childhood. Usually, the tumor is confined to the sellar region and the third ventricle, but due to frequent infiltration and adherence to the central nervous system, it often has an unfavorable clinical behavior. Therefore, it is classified by the World Health Organization (WHO) as a tumor of low or uncertain malignant potential. Endocrine after effects, mainly hypothalamic hypopituitarism, obesity and diabetes insipidus are highlighted due to their important impact on the quality of life of patients, mostly children. Optimal treatment of this tumor is a major challenge for neurosurgeons and endocrinologists. The combination of surgery, radiation, and application of radioisotopes and intratumoral drugs, aims at maximizing the chances of cure with minimal complications. Yet, recurrence is still frequent. Choosing the best treatment modality for craniopharyngiomas is a difficult decision, and it should always be specific for each case. In order to explore the multiple therapeutic options for craniopharyngiomas, we reviewed the literature with emphasis on the therapeutic possibilities and complications inherent to the treatment of this disease.

Tumores intracranianos em pacientes encaminhados para estudos por tomografia de coerência óptica como portadores de glaucoma sem hipertensão ocular: relato de dois casos / Intracranial tumors in patients referred for optical coherence tomography examination as glaucoma suspects: case report

A tomografia de coerência óptica (OCT) tem se mostrado muito útil na avaliação de pacientes com glaucoma. São relatadas duas pacientes referidas com a suspeita de glaucoma sem hipertensão para avaliação por tomografia de coerência óptica que, na verdade, eram portadoras de tumores intracranianos - um cordoma de clivo no primeiro caso e um craniofaringeoma no segundo. Os achados à tomografia de coerência óptica - diminuição difusa da espessura da camada de fibras nervosas circumdiscais desproporcionalmente acentuada nos setores nasal e temporal - levantaram a suspeita de acometimento na região do quiasma e permitiram o diagnóstico destes importantes tumores intracranianos.

Optical coherence tomography (OCT) has proved to be a very valuable tool in the assessment of patients with glaucoma. In this report, intracranial tumors were discovered in two glaucoma suspects referred for diagnostic confirmation by OCT - a clivus chordoma and a craniopharyngeoma. Optical coherence tomography findings - marked asymmetrical diffuse attenuation of the peripapillary nerve fiber layer in nasal and temporal sectors - raised concerns about lesions in chiasmatic region and permitted the timely diagnosis of these intraocular tumors.

Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

We report a rare case of petroclival craniopharyngioma with no connection to the sellar or suprasellar region. MRI and CT images revealed a homogenously enhancing retroclival solid mass with aggressive skull base destruction, mimicking chordoma or aggressive sarcoma. However, there was no calcification or cystic change found in the mass. Here, we report the clinical features and radiographic investigation of this uncommon craniopharyngioma arising primarily in the petroclival region.

Craneofaringioma, a propósito de un caso / Craniopharyngioma, a case report

Los craneofaringiomas representan aproximadamente 6-10% de los tumores cerebrales en la población pediátrica. Su manifestación clínica puede variar desde un estado asintomático hasta un amplio espectro de síntomas neurológicos, psicológicos, visuales y endocrinos. Presentamos el caso de un preescolar masculino de 2 años y 11 meses de edad con craneofaringioma gigante diagnosticado por tomografía simple de cerebro. Fue referido y hospitalizado por presentar desnutrición severa marasmática.

The comparison of conventional pterional and transciliary keyhole approaches: pro and con.

For years, neurosurgical operations have developed. Treatment modalities involving new devices and instruments have been innovated. Another principle of management that has been created and developed is the approach to enter the intracranial structure. Yasargil was credited to the standard and may be the most important approach, pterional or frontotemporal approach. Many modifications of this approach by new neurosurgeons has been created. Until now the concept of minimally invasive neurosurgery has been well accepted. The fundamental tendency to be as minimally invasive as possible with a minimum of iatrogenic traumatization and to achieve a maximum of efficiency in the treatment of a patient has existed since the beginning of surgery. The development of unconventional or "difficult approaches", which is based on increased knowledge of microsurgical anatomy, improved preoperative diagnostic techniques, and well-adapted microsurgical instruments, definitely forms one important aspect of "refinement in microneurosurgical operating". The supraorbital keyhole via the eyebrow incision is one of the minimally invasive approaches. Until now, there are debates between the advantage and disadvantage of this approach. The authors present the comparative approaches between these young and old methods. The pros and cons are listed in detail.

Craniopharyngioma in the Temporal Lobe: A Case Report

Herein, we report on an unusual case of craniopharyngioma arising in the temporal lobe with no prior history of surgery and with no connection to the craniopharyngeal duct. MR images showed a cystic tumor with a small solid portion. To the best of our knowledge, this is the first case of a craniopharyngioma occurring in the temporal lobe.

Correlation of CT scan and histopathology in brain tumours

The objective of this study was to determine the preoperative diagnostic accuracy of CT Scan in brain tumours and compare it with post operative histopathology results. This study was conducted at PNS Shifa Naval Hospital Karachi over a period of four years from December 1995 to December 1999. A total of 100 patients were included who underwent computed tomography [CT] scan for suspected brain tumours. Selection criteria included those single intracranial masses labeled as brain tumour on the basis of their CT features. Multiple lesions were excluded. The patients' age ranged from 13 85 years, mean age 43 years with male to female ratio 2:1 The common clinical presentations were headache, diplopia, seizures, personality changes, vertigo, vomiting and progressive neurological deficit. The results of our study revealed that out of hundred cases gliomas were 48, meningiomas 25, pituitary adenomas 12, craniopharyngiomas 6, pineal region tumours 4, acoustic neuromas 3 and choroid plexus papilloma 2. The diagnostic accuracy of CT scan was found to be 8396 on average, when correlated with histopathology

Infrasellar craniopharyngioma mimicking a clival chordoma: a case report.

An unusual case of entirely infrasellar craniopharyngioma mimicking a clival chordoma is described. Only 22 cases of craniopharyngioma with nasopharyngeal extension have been reported in the literature. Of the reported cases, most were primarily intracranial with secondary downward extension; only two were thought to originate from an infrasellar location. The present case is another example of an entirely infrasellar craniopharyngioma, with extensive clival destruction, mimicking a clival chordoma. Relevant literature on the subject is reviewed.

Infrasellar craniopharyngioma: case report

We report a case of infrasellar craniopharyngioma in a 34 year-old woman who presented with progressive headache and diplopia. Computed tomographic and magnetic resonance images showed a heterogeneous tumor originating from the sphenoid bone with ethmoid sinus and sella turcica extension. A sublabial rhinoseptal transsphenoidal surgery was performed. Craniopharyngiomas with infrasellar development are very rare. Infrasellar craniopharyngioma is uncommon, thirty-five cases has been reported in literature. The embryology, clinical features and radiographic investigation of these tumors are discussed

Tumores sellares y suprasellares que afectan al quiasma y nervios ópticos. II. Tumores derivados de la hipófisis y de los restos de la bolsa de Rathke / Sellar and suprasellar tumors afecting optic chiasm II. Pituitary derived tumors and rathkes pouch remainders

Los tumores derivados de la hipófisis y de la bolsa de Rathke incluyen los adenomas (cromófilos y cromófobos) y los arqueoblastomas (craneogaringiomas, quistes coloides, tumor de Erdheim, etc.) Estos tumores en su evolución biológica lesionan a la vía visual anterior y las manifestaciones clínicas más tempranas son visuales y le siguen las endócrinas y mecánicas. El tratamiento depende de la prontitud del diagnóstico. Los métodos diagnósticos actuales de imagenología permiten un diagnóstico temprano (microadernomas). Se mencionan los hechos histológicos más sobresalientes en relación con la clínica

Astrocitoma da neurohipófise com expansäo supra-selar simulando craniofaringeoma: relato de caso / Astrocytoma of the neurohypophysis with supraselar extension simulating craniopharyngioma: case report

Este trabalho descreve um caso de astrocitoma da neurohipófise e os dados clínicos, de neuroimagem e as características patológicas deste tumor. A paciente, de 17 anos de idade, do sexo feminino, apresentava-se com atraso puberal, baixa estatura, diabetes insipidus, déficit visual e hipertensao endocraniana. O megateste hormonal revelou pan-hipopituitarismo. A tomografia computadorizada de crânio mostrou massa pituitária com expansao supra-selar e hidrocefalia. O tumor foi removido totalmente pela via transesfenoidal e o material obtido, estudado por microscopia e pelo método imuno-histoquímico, apresentou imunorreaçao positiva para a proteína ácida gliofibrilar. Discute-se também o diagnóstico diferencial dos tumores desta regiao em jovens e a via de acesso utilizada.

Craneofaringioma. Estudio multicéntrico retrospectivo en cinco centros hospitalarios de la ciudad de Guayaquil

Se realizó un estudio multicéntrico retrospectivo en 5 hospitales de la ciudad de Guayaquil, desde 1984 a 1993 recopilándose 20 casos con los datos suficientes para el estudio- De los 20 casos, 12 fueron del sexo masculino (60 por ciento ) y 8 del sexo femenino (40 por ciento). El grupo de edad de mayor incidencia fue entre los 5 a 14 años con 12 casos (60 por ciento). El cuadro clínico más largo de evolución fue de 5 años en 2 casos (10 por ciento). el motivo de ingreso más frecuente fue la cefalea en 19 pacientes (95 por ciento). El síndrome neurológico que más se presentó fue el de hipertensión endocraneal que se presentó en todos los pacientes y dentro de éste la cefalea fue el síntoma más referido en los 19 casos. En todos los pacientes se realizó estudios radiológicos de cráneo siendo el hallazgo más frecuente las calcificaciones en 6 pacientes. El estudio por medio de tomografía computarizada (TC) demostró que el 50 por ciento de los tumores eran supreselar; en 9 casos del tumor fue mixto (45 por ciento), solo cinco tumores (25 por ciento) reportó calcificaciones, la prouección tumoral hacia el III ventrículo fue la más observada. La técnica quirúrgica más usada fue la subfrontal con 12 pacientes siendo el tipo de resección subtotal en 19 casos el más usado. Las complicaciones postquirúrgicas más observadas fue la diabetes insípida en 11 casos (55 por ciento) la mortalidad de la serie fue del 40 por ciento...

Pubertad retrasada constitucional o hipogonadismo hipogonadotrófico? / Constitutional puberty delay or hypogonadotrophic hypogonadism?

This review is focused on the diagnosis, clinical and general therapeutic approach of constitutional growth and puberty delay and hypogonadotrophic hypogonadism in males, 2 entities that are difficult to distinguish. Clinical history and physical examination must be carefully performed. Delayed puberty is due to constitutional growth and puberty delay in the vast majority children. These must be distinguish from a small fraction of boys with hypogonadism, a pathological condition. A number of laboratory test allow the prediction of puberty onset and progression. Nevertheless, the advent of highly sensitive immnuessay and radiometric immunoassay systems for LH, FSH and testosterone has not entirely solved the problem, since their values may overlap between normal and pathological conditions