Prognostic Factors in Infants and Very Young Children with Intracranial Ependymomas

Abstract

The Pediatric Oncology Group (1986–1990) conducted a study in which 48 children <3 years of age with intracranial ependymomas were treated with prolonged postoperative chemotherapy (CT) and delayed RT. Thirty-one children, 0–23 months of age at diagnosis (Gp A) received 2 years of CT followed by RT; while 17 children, 24–36 months of age at diagnosis (Gp B) received CT for 1 year followed by radiation. One-year survivals were 87% (Gp A) and 94% (Gp B) and 2-year survivals were 67% (Gp A) and 82% (Gp B). In subsequent years a significant divergence in survivals according to age has been noted (p = 0.04). Five-year survivals were 25.7% (Gp A) vs. 63.3% (Gp B). The curves began to diverge 1 year following diagnosis. Other than age, the only significant prognostic factor was degree of surgical resection: 5-year survivals were 66% (total resection) vs. 25% (subtotal resection). Neither the presence of metastases, degree of anaplasia nor the degree of surgical resection varied significantly according to age at diagnosis. The most likely reason for the difference in survivals between the two age groups relates to the timing of radiation following CT, i.e., 1-year delay in children 24–36 months of age compared to a 2-year delay in children 0–23 months of age. An alternative but less likely hypothesis is that ependymomas in the younger children have a more aggressive biology. In contrast, survivals in the 24- to 36-month group are much better than previous reports in the literature suggesting that prolonged postoperative CT may allow both a delay in CRT as well as provide improved survivals. Based on these results, future treatment trials should emphasize maximal surgical resection and a delay in radiation of no more than 1 year.

Pediatric Ependymoma: Biological Perspectives

“…More than half of all cases occur in children under 5 years of age (4)(5)(6). Almost 90% of pediatric ependymomas are intracranial in origin, with two thirds arising in the posterior fossa (4)(5)(6)(7)(8).…”

“…More than half of all cases occur in children under 5 years of age (4)(5)(6). Almost 90% of pediatric ependymomas are intracranial in origin, with two thirds arising in the posterior fossa (4)(5)(6)(7)(8). Nevertheless, pediatric ependymomas are capable of occurring anywhere within the CNS, including the parenchyma of the cerebral hemispheres and, rarely, the spinal cord (9).…”

“…Historically, children below 3 years of age and infratentorial ependymomas have been associated with a poor outcome (6,(22)(23)(24). It remains unclear whether this reflects tumor biology, the surgical inaccessibility of posterior fossa tumors, which are more prevalent in younger children, or the avoidance of adjuvant radiotherapy in early life resulting from concerns about long term clinical sequelae (4,14,23,25,26).…”

“…Several studies have shown conflicting results about an association between grade and patient outcome (1,4,5,13,14,18,22,(27)(28)(29)(30)(31).…”

Current Treatment Approaches for Infants with Malignant Central Nervous System Tumors

“…Nearly half of the 25 evaluable patients showed a partial or complete response to two courses of cyclophosphamide and vincristine. The 5-year PFS rate was 27% (Ϯ8.2%) [71]. Conclusions of the study emphasized the importance of maximal surgical resection and a delay in radiation therapy of Յ1 year from diagnosis.…”