Treatment of central nervous system germ cell tumor in a general hospital: retrospective study from 2000 to 2020

<p>Management of central nervous system (CNS) germ cell tumors (GCTs) is one of major issues in pediatric and young adult patients. Germinoma has come to have a relatively good prognosis, yet recurrence occurs infrequently. Non-germinomatous GCTs (NGGCTs) are still challenging and require intensive chemotherapy with minimized radiotherapy. GCTs arise preferentially in midline structures, including pineal gland and neurohypophysis. Accordingly, patients suffer from hydrocephalus, ophthalmological and neuroendocrinological symptoms. Histopathological components of GCTs are classified into 5 subtypes: germinoma, teratoma, choriocarcinoma, yolk sac tumor, and embryonal carcinoma. GCTs often consist of more than one of these components. Diagnosis and decision making on treatment strategy depend both on biopsy specimens and on tumor markers including alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG).</p><p>We report here our series of 24 (male: female = 20: 4, ages: 9-32, mean: 19, median: 19) patients with CNS-GCTs treated from 2000 to 2020. Eighteen of them suffered from germinoma and others from NGGCTs (2 yolk sac tumor, 2 choriocarcinoma, 1 embryonal carcinoma and 1 immature teratoma). Ten germinoma patients and 2 yolk sac tumor patients were treated with ICE regimen (IFO/CDDP/VP-16). Eight germinoma patients were treated with CARE regimen (CBDCA/VP-16). Three NGGCTs patients (1 choriocarcinoma, 1 embryonal carcinoma and 1 immature teratoma) diagnosed after 2008 were treated with Japanese Pediatric Brain Tumor Consortium (JPBTC) regimen (CPA/CDDP/VP-16+intrathecal MTX) by the dedicated pediatric oncologist. Radiotherapy was basically 24 Gy whole ventricular irradiation with optional local boost for germinoma, while 47.6∼55 Gy involved-field radiotherapy sometimes including 22.4∼25.6 Gy craniospinal irradiation for NGGCTs. One of NGGCTs (yolk sac tumor) treated with ICE regimen before 2008 died 17 months due to dissemination, while other one of NGGCTs (choriocarcinoma) with family history of schwannoma experienced lumber spinal root schwannoma 10 years after initial treatment. One of germinoma experienced local recurrence 15 years after treatment, while other one died 19 years after onset unrelated to primary disease.</p><p>All types of GCTs are treated according to the best contemporary protocol and dedicated specialists obtained. Patients must be followed up for long-term, not only due to their neuroendocrinological sequelae, but also due to potential late complications including secondary cancer and recurrence.</p>