Molecular and cellular pathogenesis of adamantinomatous craniopharyngioma.

Adamantinomatous craniopharyngiomas (ACPs) are the most common pituitary tumours in children. Although histologically benign, these are clinically aggressive tumours, difficult to manage and associated with poor quality of life for the patients. Several human and mouse studies have provided unequivocal evidence that the over-activation of the WNT/β-catenin signalling pathway underlies the molecular aetiology of these tumours. Recently, research using genetically modified mouse models of human ACP have revealed a critical and unexpected non-cell autonomous role for pituitary stem cells in ACP tumourigenesis, which has expanded the cancer stem cell paradigm. As the result of this basic research, the pathogenesis of ACP is being unveiled, with promising implications for the development of novel treatments against these childhood neoplasms. These benign tumours may additionally represent a unique model to provide insights into the initial steps of oncogenesis

Craniopharyngioma - Transnasal Endoscopic Approach

Craniopharyngiomas are slow growing tumours arising from remnants of the craniopharyngeal duct and occupy the sellar region. The patients may remain asymptomatic for long duration or present with headache or visual disturbances. Surgery is the mainstay of the treatment. Traditionally these tumours have been removed by neurosurgeons through the cranial approach but the advent of nasal endoscopes has opened new avenues for ENT surgeons to treat such patients. We hereby present a case of craniopharyngioma who was successfully treated by Trans-nasal Hypophysectomy

Stereotactic Neurosurgical Treatment Options for Craniopharyngioma

Craniopharyngioma are the most common non-glial tumors in childhood. The results of different studies indicate that radical excision surgery is not an appropriate treatment strategy for childhood craniopharyngioma with hypothalamic involvement. Stereotactic neurosurgery provides save, minimal invasive and cost-efficient options in the treatment of childhood craniopharyngioma. In this review a summary of the contribution of the stereotactic neurosurgery in the interdisciplinary treatment regime of childhood craniopharyngioma will be given and discussed in detail

Sarcoidosis of the hypothalamus and pituitary stalk

We report a rare case of sarcoidosis of the hypothalamic and suprasellar region, with clinical course and the magnetic resonance imaging follow-up

The birth and rise of a craniopharyngioma: the radiological evolution of an incidental craniopharyngioma detected on serial MRI during medical treatment of a macroprolactinoma

This case demonstrates the rare coexistence of a prolactinoma with craniopharyngioma and documents its radiological growth. This case suggests that patients with pituitary neoplasms should be followed closely and although prolactinomas can often be managed medically, a coexistent other lesion may require surgery for histological assessment and to reduce mass effect

Obesity and craniopharyngioma.

An epidemic of pediatric obesity has occurred across the world in recent years. There are subgroups within the population at high-risk of becoming obese and especially of having experience of precocious cardiovascular and metabolic co-morbidities of obesity. One of these subgroups comprises patients treated for childhood cancers and namely survivors of craniopharyngioma. The high incidence of obesity in this group makes these patients an important disease model to better understand the metabolic disturbances and the mechanisms of weight gain among cancer survivors. The hypothalamic-pituitary axis damage secondary to cancer therapies or to primary tumor location affect long-term outcomes. Nevertheless, the aetiology of obesity in craniopharyngioma is not yet fully understood. The present review has the aim of summarizing the published data and examining the most accepted mechanisms and main predisposing factors related to weight gain in this particular population

Craniofaringioma cístico: quimioterapia intratumoral com interferon alfa

OBJECTIVE: To assess whether the cystic craniopharyngiomas can be controlled with the use of intratumoral applications of interferon alpha. METHOD: Nineteen patients with the diagnosis of cystic craniopharyngioma were treated with intratumoral chemotherapy with interferon alpha from January 2002 to April 2006. All patients underwent placement of an intracystic catheter connected to an Ommaya reservoir. Through this reservoir were made applications during chemotherapy cycles. Each cycle corresponded to application of 3,000,000 units of interferon alpha three times per week on alternate days totalizing 36,000,000 units. Response to treatment was evaluated by calculating the tumor volume on MRI control after one, three and six months after the end of each cycle. Patients who developed worsening of symptoms or who had insignificant reduction in tumor volume during follow-up underwent repeat cycle chemotherapy. RESULTS: Four patients received four cycles of chemotherapy, three patients received three cycles, six patients received two cycles and six patients received one. The lower percentage of reduction in tumor volume was 60% and the bigger reduction was 98.37%. Eleven patients had a reduction greater than 90%. Five patients had a tumor reduction between 75 and 90% and in three patients the tumors were reduced by less than 75%. No deaths occurred during treatment and side effects of interferon alpha were well tolerated. No treatment was discontinued. Follow-up after the last application ranged from one year and five months to three years and nine months. CONCLUSION: The intratumoral chemotherapy with interferon alpha decreases the volume of cystic craniopharyngiomas and so far can be considered a new therapeutic alternative.OBJETIVO: Avaliar se os craniofaringiomas císticos podem ser controlados com aplicações intratumorais de interferon alfa. MÉTODO: De janeiro de 2002 a abril de 2006, 19 pacientes foram submetidos à colocação de um cateter intracístico conectado a reservatório de Ommaya para aplicações intratumorais de ciclos de 36.000.000 de unidades de interferon alfa. A resposta ao tratamento foi avaliada pelo cálculo do volume tumoral na ressonância magnética de controle ao término de cada ciclo. RESULTADOS: Os pacientes receberam de um a quatro ciclos de quimioterapia. Onze pacientes apresentaram uma redução do volume tumoral maior que 90%; cinco pacientes apresentaram uma redução entre 75% e 90% e três pacientes uma redução menor de 75%. Não houve óbitos durante o tratamento e os efeitos colaterais do inferferon alfa foram bem tolerados. Nenhum tratamento foi interrompido. CONCLUSÃO: A quimioterapia intratumoral com interferon alfa diminui o volume dos craniofaringeomas císticos e pode ser considerada uma nova alternativa terapêutica.Federal University of São Paulo Department of Neurosurgery and Section of Pediatric NeurosurgeryFederal University of São Paulo Department of PediatricsFederal University of São Paulo Laboratory of Pediatric Oncology InstituteUNIFESP, Department of Neurosurgery and Section of Pediatric NeurosurgeryUNIFESP, Department of PediatricsUNIFESP, Laboratory of Pediatric Oncology InstituteSciEL