Treatment outcome of optic pathway glioma in children with diencephalon syndrome

Objective To explore the clinical treatment outcomes of the optic pathway glioma in children with diencephalic syndrome. Methods The clinical data of 8 cases of optic pathway glioma with diencephalic syndrome were analyzed retrospectively who were admitted to Department of Neurosurgery, Beijing Children′s Hospital, Capital Medical University from July 2015 to December 2018. The range of the children′s ages was from 8.0 to 42.2 months. No weight gain was reported in all those patients before the age of 1 year, and they showed a cachexia state of extreme emaciation at admission. The range of children′s preoperative weights was from 5.5 to 11.5 kg. The weight-for-age Z score was lower than -2 in all 8 patients who underwent microsurgical resection of tumor followed by chemotherapy post operation. Results Among the 8 cases of optic pathway glioma combined with diencephalic syndrome, subtotal resection was achieved in 7 cases, near-total resection in 1, and there was no death related to operation. Postoperative pathological examination confirmed 2 cases of pilocytic astrocytoma and 6 cases of pilomyxoid astrocytoma. The range of follow-up period of 8 children was from 1 to 18 months, the range of postoperative weight was from 8.0 to 20.0 kg, the weight of all patients increased to various degrees, and some of the patients′ clinical symptoms were alleviated. One patient died after discharge, and chemotherapy was applied to the rest of 7 patients. Residual tumor shrank in 4 patients, progressed in 2 and disseminated in 1. Conclusions Subtotal tumor resection in children with optic pathway gliomas and diencephalic syndrome could significantly improve the symptom of emaciation and alleviate some other symptoms. There seems to be great variability in the tumor sensitivity to postoperative chemotherapy. Key words: Glioma; Child; Optic pathway glioma; Diencephalic syndrome; Treatment outcome