Primary Intracranial Pineal Germinomas In Children:Report Of Three Cases And Review Of Literature

Research backgroundPrimary intracranial germ cell tumor(ICGCT) is a rare tumor that are known to arise from the midline structure, especially the pineal gland or suprasellar region due to aberrant migration during embryogenesis. ICGCT occurs as midline lesions around the third ventricle, most commonly involving the pineal gland and the neurohypophyseal region of the brain, which includes the infundibulum and/or pituitary stalk.Intracranial genn cell tumors (ICGCT) occur primarily in the 2nd and 3rd decades of life and in 2-11% of children with brain tumors between 0-19 years of age.Clinical manifestation of ICGCT is related to the patients age, location and size of tumor and implantation metastasis.ICGCTs are sensitive to radiation and can be successfully treated with radiation therapy. Craniospinal irradiation (CSR) has traditionally been considered as the gold standard of treatment against intracranial germinoma.Although intracranial GCT is sensitive to treatment, a high recurrence rate, treatment-related long-term complications and the heterogeneity of this tumor group make treatment complicated. Concern about late neurologic detrimental effects of CSR, especially in young patients, led physicians to omit spinal irradiation and try to reduce the radiation field as much as possible in limited disease.ObjectiveTo study the manifestation, diagnosis,treatment and prognosis of primary intracranial germinoma.MethodsReport three cases of primary intracranial pineal germinoma and review of the literature.ResultsWe describe three cases of primary intracranial pineal germinoma.Three male patients are between 10 to 14 years old,average age is 11.5. An initial brain magnetic resonance image (MRI) revealed a well-enhanced mass in the pineal gland.All the patients without a past medical history presented to hospital due to headache, two cases presented with nausea and vomiting,one case presented with diplopia,the other case presented with polydipsia and polyuria.Only case one had been get pineal mass biopsy by craniotomy, germinoma was confirmed and was treated with whole cranial radiotherapy.However,recurrence was discovered one year later at the primary site.Although the patient was subsequently treated with 6 cycles of chemotherapy,spinal metastases was discovered 2 mouths later.The patient was presented with rapid deterioration and death now.Other two cases were diagnosed with pineal germinoma based on MRI and diagnostic radiotherapy,and were treated with craniospinal radiotherapy,no recurrence until now.Conclusion1. Diagnosed with ICGCT based on tumor biopsy is more specific than MRI and diagnostic radiotherapy.But there is not of great significance in prognosis evaluation in our report.2. Two cases who were treated with craniospinal radiotherapy have good prognosis.Case one who was only treated with whole cranial radiotherapy had poor prognosis, recurrence and rapid deterioration.So CSR is still necessary to be choice for treatment against ICGCT.3. Case one patient was subsequently treated with 6 cycles of chemotherapy after primary tumor recurrence,and during chemotherapy treatment, whose imaging showed reduction of tumor mass.It means the significance of chemotherapy.Howere,how to achieve high long-term disease control rate and good quality of life of the survivors, carefully selected multimodality treatment needs to be study.