Approach to the management of hypothalamic pituitary function in patients with craniopharyngioma after operation / 中华内分泌代谢杂志

Craniopharyngiomas develop from remnant epithelial cells of Rathke's pouch in the sella/parasella region with low histological grade. Clinical manifestations are related to visual impairment, increased intracranial pressure, and hypothalamic/pituitary deficiencies, including vision loss, headache, nausea, growth retardation, and hypogonadism. Despite benign epithelial tumors, craniopharyngiomas are adjacent to critical brain structures including hypothalamus, pituitary stalk, chiasma opticum and have recurrence tendency. Treatment-related complications including hypothalamic/pituitary deficiencies, diabetes insipidus, and hypothalamic obesity are major risk factors for reduced quality of survival and throw out a great challenge to the surgical treatment and the postoperative management. Clinical features of one case craniopharyngiomas with manifestation of severe hypopituitarism are described with the aim of exploring effective therapies.

Duodenal-jejunal bypass normalizes pancreatic islet proliferation rate and function but not hepatic steatosis in hypothalamic obese rats

Modifications in life-style and/or pharmacotherapies contribute to weight loss and ameliorate the metabolic profile of diet-induced obese humans and rodents. Since these strategies fail to treat hypothalamic obesity, we have assessed the possible mechanisms by which duodenal-jejunal bypass (DJB) surgery regulates hepatic lipid metabolism and the morphophysiology of pancreatic islets, in hypothalamic obese (HyO) rats. During the first 5 days of life, male Wistar rats received subcutaneous injections of monosodium glutamate (4 g/kg body weight, HyO group), or saline (CTL). At 90 days of age, HyO rats were randomly subjected to DJB (HyO DJB group) or sham surgery (HyO Sham group). HyO Sham rats were morbidly obese, insulin resistant, hypertriglyceridemic and displayed higher serum concentrations of non-esterified fatty acids (NEFA) and hepatic triglyceride (TG). These effects were associated with higher expressions of the lipogenic genes and fatty acid synthase (FASN) protein content in the liver. Furthermore, hepatic genes involved in β-oxidation and TG export were down-regulated in HyO rats. In addition, these rats exhibited hyperinsulinemia, β-cell hypersecretion, a higher percentage of islets and β-cell area/pancreas section, and enhanced nuclear content of Ki67 protein in islet-cells. At 2 months after DJB surgery, serum concentrations of TG and NEFA, but not hepatic TG accumulation and gene and protein expressions, were normalized in HyO rats. Insulin release and Ki67 positive cells were also normalized in HyO DJB islets. In conclusion, DJB decreased islet-cell proliferation, normalized insulinemia, and ameliorated insulin sensitivity and plasma lipid profile, independently of changes in hepatic metabolism.

Management of pituitary endocrine function in craniopharyngioma / 中华内分泌代谢杂志

Craniopharyngioma is a kind of benign tumor of the relict squamous epithelial cells originated from the pituitary gland of cranial cheek pouch in the process of embryonic development. With an overall incidence of 0.13 cases per 100 000 person-years, they may represent up to 5%-10% of intracranial tumors in children. There is always debate regarding the appropriate treatment for craniopharyngiomas which often present symptomatically given their proximity to critical brain structures, and pose significant surgical challenges. And the complications caused by the treatment such as diabetes insipidus, hypothalamic obesity, and hypopituitarism, that throw out a challenge to the postoperative management and the quality of the patients. Craniopharyngioma should be recognized as a chronic disease requiring constant monitoring along with the clinical and multidisciplinary treatment in order to provide optimal care of surviving 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.

Factors Affecting the Increments of Body Mass Index in Adult Survivors of Childhood Brain Tumors / 소아과

PURPOSE: The purpose of this study was to evaluate the factors affecting the increments of body mass index(BMI) in adult survivors from childhood brain tumors. METHODS: We retrospectively reviewed medical records of 63 patients who were diagnosed with brain tumors and treated at Seoul National University Children's Hospital between 1982 and 2000. We evaluated the factors affecting adult BMI(aBMI) at attainment of final adult height(FAH). Observed factors were as follows : characteristics of tumors, modes of treatment for tumors, existence of growth hormone deficiency(GHD) and growth hormone(GH) replacements, cumulative BMI changes according to GH replacements. RESULTS: aBMI in patients with GHD was greater than that in patients without GHD(23.5+/-3.9 kg/ m2 vs 20.4+/-2.8 kg/m2, P=0.012). Among patients with GHD, GH replacement didn't make any difference in aBMI between treated and untreated patients. Cumulative BMI changes from diagnosis of tumor to attainment of FAH( BMI) were positively correlated with cumulative BMI changes before and during GH replacement(r=0.740, P<0.001 and r=0.574, P<0.001, respectively). When analyzed by multiple stepwise linear regression, the strongest related factor for increment of BMI was the increment of cumulative BMI changes before GH replacements. BMI increments velocity(cumulative BMI change/year) before and during GH replacements were 2.3+/-2.5 kg/m2/year and 0.9+/-1.6 kg/m2/ year, respectively(P=0.005). Patients with extensive surgery were greater aBMI(P=0.036). CONCLUSION: This study suggests that GHD and damage of hypothalamus by tumor and/or modes of treatment contribute to the increment of BMI in adult survivors of childhood brain tumors. Early GH replacements may possibly prevent severe hypothalamic obesity, which should be further clarified.