Intracranial Neoplasms in the First Year of Life: Results of a Third Cohort of Patients From a Single Institution

Abstract

BACKGROUND\nBrain tumors in the first year of life are rare and their management remains challenging.\n\n\nOBJECTIVE\nTo report on the contemporary management of brain tumors in infants with reference to previous series from our institution.\n\n\nMETHODS\nRetrospective cohort study design. Electronic/paper case note review of all brain tumors diagnosed at our institution in children aged <1 yr since the publication of our previous series.\n\n\nRESULTS\nNinety-eight patients were seen. The most common presentations were with vomiting and macrocrania, at a median age of 184 d. Sixty-two percent of tumors were supratentorial. Ninety-one patients underwent 230 procedures; 7 patients had no surgery. One hundred eighteen operations were directly on brain tumors (biopsy 37, subtotal resection 47, gross total resection 34). Ninety-one cerebrospinal fluid diversions, 9 endoscopic procedures, and 13 preoperative embolizations were performed. Operative mortality was 4.4%. Tumor types in order of frequency were choroid plexus papillomas (CPP, 17), primitive neuroectodermal tumor (12), atypical teratoid/rhabdoid tumor (10), high-grade glioma (9), optic glioma (9), ependymoma (8), low-grade glioma (6), pilocytic astrocytoma (6), choroid plexus carcinoma (5), and teratoma (5), with 11 miscellaneous tumors. Survival was 93% at 1 mo (91/98), 64% at 1 yr (61/95), 44% at 5 yr (32/73), 28% at 10 yr (16/58). No patients with CPP or low-grade glioma died. Five-year survival rates were lowest for anaplastic ependymoma, primitive neuroectodermal tumor, and atypical teratoid/rhabdoid tumor. Seventy-seven percent of children reaching school age were in mainstream schooling.\n\n\nCONCLUSION\nOverall survival from neonatal brain tumors remains similar to previous series; analysis of tumor subtypes reveals improvements for CPP and gliomas. Despite increasing operative intervention, operative mortality continues to decline for this group of challenging patients.