Early Diagnosis And Postoperative Follow-up Of Children With Craniopharyngioma

Background and Objectives:Craniopharyngioma (CP) is a relatively common congenital intracranial tumor, CP represents 1-7% of intracranial tumor. It usually occurs in children and adolescents. It is the most common benign tumor in the saddle area and one of the most common childhood cancer after leukemia. CP grows slowly with special location. It is located near important structures of the endocrine and nervous system such as the hypothalamus, the optic nerve, pituitary stalk, pituitary and internal carotid. It can invade the third ventricle and obstruct cerebrospinal fluid circulation. There are three main symptoms in children with CP including visual acuity and visual field damage, intracranial hypertension, neuroendocrine dysfunction. Surgery can damage surrounding tissues of the tumor and result in severe complications. Children are in a critical period of growth and development. The location of CP is near the key position of the endocrine center. The structural damage and dysfunction can affect growth, development, physical and psychological health, and even threatening life.The purpose of this study is to investigate the changes of endocrine hormone of children with CP to diagnose the tumor earlier, and by long-time followed changes of hormone of postoperative children of CP to give proper hormone replacement to reduce the bad influence of complications. It is very necessary to diagnose tumor earlier, relieve potential harm of tumor, diagnose and treat endocrine dysfunction and complications without delay for prolonging survival and improve life quality of children with CP.Methods:This retrospective study was carried out at the Pediatric and Neurosurgery Department of Shandong Provincial Hospital Affiliated to Shandong University between January 2011 and January 2015 using query system of medical record.30 children diagnosed as CP were included as group CP.30 age-matched healthy children were included as the control group without other systemic diseases and other diseases affecting endocrine levels. Data were recorded including gender, age, clinical symptoms, preoperative and postoperative neuroendocrine hormones and imaging.Results:1. Preoperative clinical manifestations and expression of pituitary MRI:in group CP, there were 14 males and 16 females, the average age was (5.16+2.27) years (range 1.5-14), there were 22 children (73.3%) below five years of age. Vision dysfunction, seizure and growth retardation are the most common reasons for treatment. And 12 children (40%) with headache, seizure and eye injury at the same time. CP were clearly showed by the hypothalamus-pituitary MRI and the incidence of structural damage or position deviation of pituitary or pituitary stalk was bigger than any other structure and the tumor were proved by pathology.2.Preoperative hormone levels:in 10 children with growth retardation in group CP, the incidence of low levels of thyroid stimulating hormone (TSH), free triiodothyronine (FT3) or free thyroxine (FT4) and growth hormone (GH) or insulin-like growth factor 1(IGF-1) was higher than other hormone.7 children (70%) were with abnormal levels of prolactin (PRL),5 with low levels of PRL and 2 with high levels of PRL. In 10 children with growth retardation in group CP, the preoperative levels of ACTH, COR, FT3, FT4, TSH, GH, IGF-1, and PRL were significantly decreased (P<0.01)3.Postoperative clinical manifestations, expression of pituitary MRI and postoperative hormone levels in CP group:The incidence of accident such as growth retardation and polydipsia and polyuria was significantly higher(P<0.05), with the disappeared high intracranial pressure and improved eye injury after surgery. And 3 children with CP relapse after the first year of surgery. Comparing with the control group, levels of hormone of the axis of hypothalamic-pituitary-growth, hypothalamic-pituitary-thyroid, hypothalamic-pituitary-adrenal were significantly lower (P<0.01).4.The time, order, and dosage of replacement therapy of CP after surgery. Hydrocortisone:acute phase 50mg/m2, this amount was gradually decreased to (8.5 ±1.6) mg/(m2d), bid or tid. Levothyroxine sodium:(2.33±0.66) ug/(kg·d), qd. Desmopressin Acetate:(0.14±0.06) mg/(m2·d), q8h. If there was no CP relapse after the first year of surgery, the dosage of rhGH was (0.11±0.01) U/(kg·d).Conclusions:1.Children with the main symptoms of sudden vision changes and intracranial pressure symptoms should be tested by hypothalamus-pituitary MRI to exclude craniopharyngioma earlier.2.Pituitary hormones and MRI should be tested to exclude craniopharyngioma for children with sudden growth retardation. The appearance of growth hormone deficiency, secondary hypothyroidism and low level of PRL at the same time may be the helpful to early diagnosis of craniopharyngioma.3. Children with craniopharyngioma after surgery tend to be complicated by multiple pituitary hormone deficiency. Biochemical and hormone levels should be monitored, diagnosed, and treated early after surgery.4.Children with craniopharyngioma after surgery with delay of hormone replacememt therapy tend to have danger and accident.