Background: Advance in treatment and prompted diagnosis improves survival in children with primary brain tumors. However, many survivors develop neuroendocrine consequences and lifelong health problems. To date, no national guidelines for screening these problems have been established.
Objective: To assess the prevalence of neurological and endocrinological outcomes in childhood primary brain tumor survivors.
Patients and methods: A cross-sectional study was performed on all patients diagnosed with primary brain tumors before 18 years old, survived more than one year after treatment completion, had no residual tumor, and followed up between January 2019 to June 2020. Clinical parameters and biochemical profiles were collected and presented as median (range) for continuous data and number (proportion in percent) for categorical data. We also performed multivariable logistic regression analysis to estimate risk factors.
Results: Twenty-nine patients (16 girls, 55.2%) were included. The median age at tumor diagnosis was 6.5 years (4 months – 12 years). Tumors were located at posterior fossa (46%), suprasellar (20%), supratentorial (20%), pineal region (10%), and brainstem (4%). Fifty-five percent of the patients had undergone combined surgery, chemotherapy, and radiotherapy. The median follow-up time after treatment completion was 6.5 years (1-20 years). At least one of the neurological sequelae, including the motor deficit, low vision/blindness, and gait disturbance required special help or education, were found in sixty percent of patients. Five patients (17.2%) had epilepsy, two of which had intractable epilepsy. Supratentorial tumors significantly increased the risk of epilepsy (adjusted odds ratio [OR] 44; 95%CI 3.183-608.158; P = 0.003). Pituitary dysfunction (PD) diagnosed by at least one pituitary hormone deficiency was found in 16 patients (55.2%), six patients (20.7%) with suprasellar tumors, and ten patients (34.5%) with tumors located elsewhere. The median time of diagnosis was four years (1 month - 14.8 years) after tumor diagnosis. Central adrenal insufficiency (CAI) is the most prevalent (41.4%). PD was diagnosed before or together with the suprasellar tumors but diagnosed afterward with the tumor at other locations. Patients with growth hormone deficiency (GHD), hypogonadotropic hypogonadism (HH), and central diabetes insipidus (CDI) presented with symptoms (GHD: poor height velocity and short stature; HH: delayed or arrested of puberty; CDI: polyuria). In contrast, patients with CAI and central hypothyroidism (CH) were asymptomatic. Suprasellar tumor group had a higher risk of developing HH (adjusted OR 22; 95%CI 1.69-285.89; P = 0.02). There was no significant correlation between the age of tumor diagnosis, age of cranial radiation, tumor location, and pituitary dysfunction.
Conclusion: Neurological impairment is common in childhood primary brain tumor survivors. PD is also prevalent and could develop at tumor diagnosis or later. The diagnosis of PD is challenging since some hormonal deficiencies are asymptomatic. Therefore, a national guideline for long-term follow-up should be established for early detection of PD in these patients.
