A review of the factors influencing the prognosis of medulloblastoma

The importance of cell differentiation

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✓ The clinical, therapeutic, and histological features of 54 patients with medulloblastoma were analyzed retrospectively by a multivariate approach with regard to prognosis. The overall 5-year survival rate was 60%, with 48% of patients free of recurrence at 5 years. Cell differentiation, when present, was associated with a significantly longer recurrence-free period. Seventy-two percent of patients with the histological finding of cell differentiation were recurrence-free at 5 years. A marginally significant increase in the 5-year survival rate was also seen in association with differentiation. Only 34% of the patients whose tumor exhibited necrosis were alive at 5 years.

There was no statistically significant difference in 5-year survival for children under 3 years of age or for the group of children aged 5 years or under. However, a significantly larger fraction (72%) of the group aged 5 years or under had a recurrence-free period of 5 years or more. Other factors including sex, extent of surgical resection, Chang tumor stage, posterior fossa radiation dose, and adjuvant chemotherapy did not influence prognosis.

Article Information

Address reprint requests to: David C. McCullough, M.D., Department of Neurosurgery, Children's Hospital National Medical Center, 111 Michigan Avenue, N.W., Washington, D.C. 20010.

© AANS, except where prohibited by US copyright law.

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Figures

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    Photomicrographs of an undifferentiated medulloblastoma specimen obtained at the first operation. A: This field is comprised of large, slightly pleomorphic, hyperchromatic nuclei with sparse, poorly defined cytoplasm. In the lower right corner a necrotic focus is evident. H & E, × 860. B: Spongioblastic and/or astrocytic differentiation is responsible for the palisades of nuclei arrayed vertically with their cytoplasmic processes trailing between them in the horizontal axis. H & E, × 860. C: A highly differentiated oligodendroglioma with the characteristic “fried egg” appearance is illustrated. A fascicle of crowded undifferentiated medulloblastoma cells streams toward the upper left corner. H & E, × 860. D: Multiple Homer Wright rosettes evince early neuroblastic differentiation. H & E, × 430. E: In mid field, two large, clear nuclei with prominent nucleolus depict neuronal differentiation. In the upper right and lower left corners relatively undifferentiated medulloblastoma cells are haphazardly distributed. Nissl, × 1720. F: Scattered among the clusters of undifferentiated medulloblastoma cells are cells with abundant, clearly outlined, eosinophilic cytoplasm. While well-defined gross striations were not identified, these cells stained for myoglobin (medullomyoblastoma). H & E, × 860.

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    Survival curve with respect to age (left) and tumor recurrence (right), comparing the group aged 5 years or over with the group under 5 years old.

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    Recurrence-free period curve with respect to the extent of surgical resection. Gross total resection was compared to all other less complete resections (see Table 2).

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    Survival curve with respect to the presence or absence of necrosis.

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    Survival curve (left) and recurrence-free period curve (right) with respect to the presence (PNET-D) or absence (PNET-U) of cell differentiation.

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    Recurrence-free period curve with respect to the presence or absence of necrosis (left) and presence or absence of leptomeningeal spread (right).

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