Experience in the treatment of desmoplastic infantile astrocytoma/ganglioglioma

Objective To explore the clinical characteristics, treatment and prognosis of desmoplastic infantile astrocytoma/ganglioglioma(DIA/DIG). Methods A retrospective analysis was conducted from April 2007 to October 2015 on a total of 6 cases with DIG/DIA who were admitted to Department of Neurosurgery, Beijing Children's Hospital, Capital Medical University. Among them, 4 cases were male and 2 were female, and they were aged 2-19 months. The typical imaging findings included nodular enhancement commonly presenting cystic changes in superficial areas of cerebral hemispheres. The size of tumor was relatively large and could involve multiple lobes, in particular, the frontal and parietal lobes. Pathological examination revealed two-way differentiation of tumor cells to glial cells and neuroepithelium with GFAP (+ ), S-100 (+ ) and Vimentin (+ ), and Ki-67 (+ ) proliferation index was relatively low. Tumor resection was performed in all 6 cases. Postoperative subdural effusion and shunting were conducted in 1 case, and 2 cases underwent postoperative subdural effusion. The follow-up time ranged from 1 to 9 years. Results All 6 children with DIA/DIG (grade WHO Ⅰ) underwent total resection of tumors. Among them, 3 cases without undergoing subdural effusion or shunting surgery achieved good outcomes. Except temporary decrease in muscle strength occurring in 1 case, no other neurological disorders or epileptic seizures were reported. Among the 3 cases undergoing subsequent subdural effusion or shunting surgery, 1 case had good outcome, 1 case developed neurologic dysfunction, and 1 case died of infection. No metastasis spread through cerebrospinal fluid or tumor recurrence was observed. Conclusions DIG/DIA is a rare kind of intracranial tumor with common onset at less than 2 years old. The tumors are usually large and located in the superficial areas of the frontal and parietal lobes. DIG/DIA could have its unique imaging characteristics and the diagnosis could be established based on its typical pathological presentation. Good outcomes could be achieved through total resection of tumors. Key words: Glioma; Disease attributes; Therapy; Desmoplastic infantile astrocytoma/ganglioglioma