The endocrine spectrum of intracranial cysts in childhood and review of the literature

Intracranial cysts (ICC) may cause a wide spectrum of endocrinological disorders. We evaluated 27 patients who were diagnosed with ICC during investigation for neuroendocrine dysfunctions and reviewed the relevant literature. The types of ICC in the patients were arachnoid cysts (n = 13); Rathke cleft cysts (n = 7); pineal cysts (n = 5); an ependymal cyst (n = 1) and a cavum septum pellucidum cyst (n = 1). The neuroendocrine dysfunctions of the patients were obesity (n = 7), isolated growth hormone deficiency ( n = 6), central precocious puberty (n = 6), multiple pituitary hormone deficiency (n = 3), central diabetes insipidus (n = 1), growth hormone deficiency and central precocious puberty (n = 1), obesity and galactorrhea (n = 1), obesity and hypogonadotropic hypogonadism (n = 1) and growth hormone neurosecretory dysfunction (n = 1). Only three patients, who had arachnoid cysts, showed neurologic symptomatology. Although three patients underwent surgery, no improvements in endocrinological dysfunctions were observed. ICC should be considered when evaluating patients with endocrinological problems and patients with coincidental ICC should be recommended for follow-up

Neonatal effects of thyroid diseases in pregnancy and approach to the infant with increased TSH: Turkish Neonatal and Pediatric Endocrinology and Diabetes Societies consensus report

Thyroid functions in the fetus and newborn carry importance in terms of the baby's health and development of the central nervous system. Maternaliodine deficiency, exposure to iodine, thyroid diseases (Hashimoto thyroiditis, Graves') and drugs used by the mother affect thyroid functions in the fetus. Reflections of these effects are observed immediately after delivery. Investigation of the mother in terms of thyroid diseases during pregnancy, recognition and appropriate assessment of the required conditions, screening of all newborns in the first days of life in terms of congenital hypothyroidism, timely and appropriate evaluation of the screening results, early diagnosis and appropriate treatment of cases of congenital hypothyroidism, assessment and management of cases of transient thyroid hormone disorders and close monitoring of the thyroid functions and development of patients in whom treatment has been initiated with a diagnosis of hypothyroidism are crucial in terms of developmental outcomes of the babies who have thyroid function disorders or hypothyroidism. This guideline was written with the objective of guiding pediatricians, neonatologists and pediatric endocrinologists in the issue of assessment, diagnosis and management of thyroid function disorders and thyroid diseases concerning the fetus and baby during gestation and neonatal period

Rare childhood tumors in a Turkish pediatric oncology center

Background: It has been estimated that rare tumor rate is about 15% of all childhood cancer in United States. According to Turkish Pediatric Oncology Group (TPOG) datas, 8889 children were diagnosed between 2002 and 2008 in our country and 3.7% of them were diagnosed as rare tumors. Aim: To investigate the frequency and clinical features of rare tumors in our pediatric oncology center. Materials and Methods: A total of 43 cases that have diagnosed as rare tumor in 574 cancer patients between the yaer 2002 and 2012 were reviewed retrospectively. All cases definitive diagnosis were established by histopathological and immunohistochemical studies. Results: Frequency of rare tumors was 7.4% in our center. Benign and border line rare tumors were 27 (62.7%) cases, malignant rare tumor were 16 (37.2%) cases. Median follow-up period was 48 months (between 1 and 110 months). Six of the malignant rare tumors were died with progressive disease (synovial sarcoma, mixed malignant mesenchymal tumor, undifferentiated sarcoma, plexus choroideus carcinoma, renal peripheral primitive neuroectodermal tumor, adrenocortical carcinoma). Malignant rare tumor mortality rate was found 37.5% in our clinic. Conclusion: We have found that our rare tumor rate (7.4%) was higher than Turkish rare tumor rate (3.7%) according to TPOG's datas. However, it was still lower than rare tumor rates of western countries (15%), probably due to difficulties of diagnosis and referral problems