Abstract

Congenital brain tumors are devastating problems that usually result in the death of the child with a high level of permanent disability in survivors. Most of these children are very large and presentation with macrocephaly is the rule leading to the need for many of these babies to be delivered by caesarian section. For the purpose of communication, it is essential that we use words precisely and that a word means the same thing to all readers. While the classification that the authors suggest is understandable and reasonable, there seems to be an acceptance of the diagnosis of “congenital tumors” to relate to diagnosis in utero or in the first 28 days of life. Is there a compelling reason to change that definition? There is reason to believe that many of the tumors that are found early in life were indeed “congenital,” including most primitive neuroectodermal tumors. The use of the term, though, must mean that the tumor is diagnosed in the perinatal period. This diagnosis carries with it a large number of issues regarding management and the ethics of the treatment of babies diagnosed at this very fragile stage of development. Should any treatment be offered to these babies? In this series, four of the nine patients survived, of which, three are said to have normal development. One of these patients had a posterior fossa capillary hemangioma, which, it might be argued, is not a tumor at all. The surviving patient with an astrocytic tumor and the patient with a capillary hemangioma were managed without adjunctive chemotherapy or radiation therapy. We were not told whether adjunctive chemotherapy was utilized in the patients with intracranial and orbital teratomas who survived with good outcomes. These babies are a great challenge, and in the very young the outcome may not be obvious. We have treated four infants with huge supratentorial malignant astrocytic tumors with staged resection followed by chemotherapy, with longterm survival in three of the patients. It is clear that there is a significant cognitive cost of the treatment of the effect of the tumor. To develop a significant algorithm for the diagnosis and management of these rare and devastating tumors, a great deal of energy would be needed to produce a registry of tumors in the very young. The fact that there were four survivors and that three of these had good developmental outcomes would make it truly valuable to have such a registry, not so much for epidemiology, which would be very difficult, but to find some information to define hopeful cases at the onset. This would be an excellent project for the education committee of the International Society for Pediatric Neurosugery, with the help of the editorial board of Child’s Nervous System. Childs Nerv Syst (2006) 22:1433 DOI 10.1007/s00381-006-0116-5

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