Management of Chiasmatic-Hypothalamic Gliomas in Children.

Doh, Hong Jik; Kim, Il Man; Lee, Chang Young; Son, Eun Ik; Kim, Dong Won; Yim, Man Bin; Kim, Sang Pyo

Original Article

Journal of Korean Neurosurgical Society 2001;30(9): 1115-1119.

Hong Jik Doh, Il Man Kim, Chang Young Lee, Eun Ik Son, Dong Won Kim, Man Bin Yim, Sang Pyo Kim

¹Department of Neurosurgery, Keimyung University School of Medicine Dongsan Medical Center, Taegu, Korea.
²Department of Pathology, Keimyung University School of Medicine Dongsan Medical Center, Taegu, Korea.

ABSTRACT

OBJECTIVE
Management strategies for pediatric chiasmatic-hypothalamic gliomas(CHG) include surgery, irradiation, chemotherapy and a combination of these modalities. This study was performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. MATERIAL AND METHOD: We have reviewed the results of management of 6 children with a diagnosis of CHG, who were observed closely during the last 8 years. The patients were aged 7 months to 15 years. Our patients presented with diencephalic syndrome, endocrine dysfunction, and progressive visual loss. None of these had evidence of neurofibromatosis-1. Treatment consisted of surgery alone(2), surgry and irradiation(2), surgery, irradiation and chemotherapy(1), and surgery and chemotherapy(1).
RESULTS
Four children had large exophytic suprasellar tumors and two showed diffuse midline lesions. Obstructive hydrocephalus was present in all patients. Pathologic examination revealed anaplastic astrocytoma in 1 and low-grade astrocytoma in 5. Two patients, recently treated with radiation therapy following radical subtotal resection, showed significant tumor reductions and good clinical status. Four patients had partial tumor resection. Of these patients, two developed disease stabilization during follow-up period of 7 and 8 years, respectively. Life-threatening complications were noted in remaining two patients.
CONCLUSION
CHG may follow an unpredicatable course and show a various reponse to each treatment modality. Further studies are indicated to define the optimal method of treatment of CHG in childhood.

Key Words: Optic pathway glioma; Hypothalamic tumor; Radiotherapy; Chemotherapy; Children