If the
tumor markers alpha-fetoprotein and
human chorionic gonadotropin were not detected in serum and there was significant ventricular dilation visualized on neuroimages, neuroendoscopic surgery was first applied for
tumor debulking with tissue diagnosis and gross morphological analysis of the
tumor and the intraventricular structures, followed by third
ventriculostomy. Subsequent procedures were determined on the basis of verified individual
tumors. For treatment of
germinomas and
pineoblastomas, if no
tumor dissemination was confirmed by pre-, intra-, or postoperative findings, stereotactic
radiotherapy or radiosurgery was performed after one course of
chemotherapy with the
ICE regimen (isofomid,
cisplatin, and
etoposide) and followed by two additional courses of
chemotherapy. For treatment of malignant
germ cell tumors, after extensive surgery,
adjuvant chemotherapy with the
ICE regimen was performed in three courses in all cases. Then
radiotherapy was started using various methods, depending on the evidence of
tumor dissemination. For treatment of teratomatous and
neuroectodermal tumors other than
pineoblastomas, extensive surgical removal was performed. As for adjuvant
therapy, if the
tumor was a low-grade
glioma or if the patient was younger than 5 years of age, postoperative treatment did not include
radiotherapy. If the
tumor was a
malignant teratoma or high-grade
glioma, conventional focal
radiotherapy was performed, followed by
chemotherapy with
ICE for 1 year. All but two treated patients had ventriculomegaly. Neuroendoscopic procedures were performed in six of 15 treated patients. Neuroendoscopic biopsy with
tumor debulking offered enough material for tissue diagnosis, including immunohistochemical analysis and, in one case, revealed evidence of
tumor dissemination undetectable on neuroimaging. With one exception, no shunt was required in any patient undergoing
endoscopic third ventriculostomy. Stereotactic
radiotherapy was performed in indicated cases. Favorable therapeutic outcomes were obtained in all cases of
germinoma and
pineoblastoma, with follow-up periods ranging from 24 months to 6.5 years.
CONCLUSIONS: Our minimally invasive preferential regimen clarified the precise indication for neuroendoscopic procedures, and the majority of our patients with dilated ventricles and no evidence of
tumor markers were treated satisfactorily with effective neuroendoscopic procedures as the initial procedure, avoiding unnecessary
craniotomy and
radiotherapy and promising excellent therapeutic outcomes. The treatment for malignant pineal region
tumors remains a subject for further study.