Surgical treatment of brain tumors in infants younger than six months of age and review of the literature

Abstract

Objective: Brain tumors are rare in infants who are younger than six months of age. These tumors can be challenging to treat surgically. We analyzed a modern series of patients treated by a multidisciplinary team at a tertiary care center and performed a literature review of this unique population.

Methods: Retrospective clinical data were collected for patients surgically treated for intracranial mass lesions at The Children's Hospital of Philadelphia from 1998 to 2007. Dermoid cysts and other skull-based lesions were excluded from the analysis.

Results: Sixteen patients younger than six months of age underwent surgery for primary intracranial mass lesions. The median age of the patients at surgery was 5.2 months (range, 1.4-6 months of age). Children most often presented with a bulging fontanelle, hydrocephalus, or macrocephaly (seven patients). Vomiting was seen in five patients, cranial nerve palsies in one patient, and seizures in three patients. All patients had tumor resections and postoperatively were monitored in the intensive care unit. The final pathology consisted of atypical teratoid/rhabdoid tumor (three patients), primitive neuroectodermal tumor/medulloblastoma (three patients), choroid plexus papilloma (two patients), astrocytoma (two patients), ganglioglioma (two patients), desmoplastic infantile ganglioglioma (two patients), glioblastoma multiforme (one patient), and choroid plexus carcinoma (one patient). Two intraoperative deaths occurred. Of the surviving 14, a gross total resection was achieved in four. Adjuvant therapy was determined by a multidisciplinary team composed of neuro-oncology, neurosurgery, and radiation oncology. Seven patients were treated with chemotherapy, and one patient had proton beam therapy. Five-year overall survival was 45%. The eight surviving patients had neurological sequelae, and developmental outcome was variable.

Conclusions: Brain tumors are uncommon in children younger than six months of age. Patients present with a variety of tumor pathologies. Children who survive have neurological sequelae. More studies are necessary to understand the impact that different treatment options, tumor pathology, and tumor location have on neurological outcome.

Figure 1

Patient 1 was a 5 month-old girl that presented with increased head size and neck pain and stiffness. Axial (A) and sagittal (B) post-gadolinium T1 MR images demonstrated a large enhancing intraventricular mass with significant hydrocephalus. The patient underwent a craniotomy and resection. Post-operative axial (C) and coronal (D) post-gadolinium T1 imaging demonstrated gross total resection of the mass. On pathology the patient was found to have a choroid plexus papilloma.

Figure 2

Patient 16 was a 1 month-old girl that presented with increased head size and fussiness. Axial (A and B) and sagittal (C) post-gadolinium T1 MR images a demonstrated a large enhancing posterior fossa mass with significant hydrocephalus. The patient underwent a suboccipital craniotomy and resection. Post-operative axial (D and E) and sagittal (F) post-gadolinium T1 images demonstrated resection of the mass. On pathology the patient was found have a medulloblastoma with myoblastic differentiation.

Figure 3

Kaplan-Meier Survival Curve