Histologically Confirmed Primary Intracranial Germ Cell Tumors In Children; Clinical Analysis And Follow-up Of 52 Cases

ObjectiveTo summarize the clinical and pathological characteristics of histologically confirmed primary intracranial germ cell tumor (ICGCTs) in children and explore the clinical efficacy and prognosis of chemotherapy combined with low-dose radiotherapy.Methods1.Collect the information of 52 patients aged 1-18 years, who were diagnosed with histologically verified primary intracranial germ cell tumors in Peking Union Medical College Hospital from December 2002 to December 2015.2. Study the clinical characteristics of the 52 cases retrospectively.3. Evaluate the clinical efficacy of 31 patients treated with chemotherapy combined with low-dose radiotherapy and preliminary results of follow-up.Results1. Diabetes insipidus, growth retardation, headache, amenorrhea, precocious puberty etc. were common clinical manifestations. ICGCTs’endocrine features were secondary hypothyroidism and adrenal cortical dysfunction. Suprasellar region, pineal gland and basal ganglia region were their predilection sites.2. Germinoma was the most common histological type, followed by mixed germ cell tumors.3. The level of serum β-human chorionic gonadotropin (βHCG) was normal in 60% of patients, making it hard to make a diagnosis by conventional methods.4. The serum and cerebrospinal fluid(CSF) levels of PHCG and alpha fetoprotein (AFP) in patients with pure geminoma were significantly lower than those in patient with non-germinomatous germ cell tumors and germinoma with syncytiotrophoblastic giant cells (STGCs).5. The results showed that there were highly correlate relations between serum and CSF βHCG levels(r=0.884, P<0.01) and CSF (3HCG levels were higher than serum βHCG levels generally.There were also linear correlations between serum and CSF AFP levels(r=0.583, P<0.01), but CSF AFP levels were lower than serum AFP levels generally.6. The two-year overall survival(OS) in poor prognosis group was lower than that in good and intermediate prognosis groups.7. After chemotherapy followed by low-dose radiotherapy, the total effective rate was 87.1% and none developed dysnoesia and serious neurocognitive disorders.Conclusions1. When patients with normal serum PHCG levels were suspected intracranial germinoma clinically, minimally invasive neuroendoscopic biopsy is recommended in order to make definitive diagnoses and detect pathological types.2. The prognosis is related with pathological types. Prognostic classification based on pathological types appears to stratify prognosis better and helps to select suitable treatment and evaluate prognosis.3. The scheme of chemotherapy combined with low-dose radiotherapy has achieved good efficacy and improved children’s quality of life and neurocognitive function.