Cran-38. Clinicopathologic Features of Suprasellar and Lateral Cerebral Desmoplastic Infantile Astrocytoma and Ganglioglioma
Year: 2018
Type: conference-abstract
Abstract: Desmoplastic infantile ganglioglioma (DIG; also known as desmoplastic infantile astrocytoma, DIA) is a rare, World Health Organization (WHO) grade-I central nervous system tumor that most often arises in very young children, often with rapidly enlarging head circumference. We reviewed the records of all infants diagnosed with pathology-confirmed DIG/DIA at Mayo Clinic from 1997 to 2016. Immunohistochemical staining was performed on tumor specimens to assess expression of IDH1 R132H, BRAF V600E, H3K27me3 and INI1. Seven patients were included in this study: age range was 2 - 9 months, and four were male. Six patients presented with rapidly enlarging head circumference, and one presented with isolated seizures. On immunohistochemical analysis, all tumors were negative for R132H-mutant IDH1. INI1 expression was retained in all specimens, and BRAF V600E expression was negative or only very focally positive. H3K27me3 expression was partly lost in four tumors. Three tumors were located in the suprasellar region, and four were located in the lateral cerebral hemispheres. All patients underwent surgery for tumor resection; a gross total resection was achieved in the lateral cerebral tumors, and a subtotal resection was achieved in the suprasellar tumors. At 2-year follow-up, none of the patients with a hemispheric tumor developed long-term sequelae from their surgery, but each patient with a supresellar tumor had a neurologic or endocrinologic deficit from tumor progression or surgery, including visual impairment, panhypopituitarism and/or hemiparesis. These results highlight the importance of tumor location in prognosticating these challenging tumors, suggesting that suprasellar location is associated with a poorer prognosis.
Source: Neuro-Oncology
Institution: Mayo Clinic
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W2808759801