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

Abstract

Background: Brain tumors in the first year of life are rare and their management remains challenging.

Objective: To report on the contemporary management of brain tumors in infants with reference to previous series from our institution.

Methods: Retrospective 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.

Results: Ninety-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.

Conclusion: Overall 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.

Keywords: ATRT; Brain tumor; Choroid plexus papilloma; Glioma; Infant; PNET; Survival.