We present a case of desmoplastic cerebral astrocytoma of infancy (DCAI) in a 7 1/2-month-old girl and include immunohistochemical, ultrastructural and proliferative activity studies. The dural-based cystic tumor showed a biphasic pattern consisting of glial fibrillary acidic protein (GFAP)-positive astrocytes embedded in a desmoplastic stroma. The astrocytic processes were lined with basal lamina at their surface contacting the collagen. Scattered islands of undifferentiated small cells were seen acquiring GFAP positivity at their peripheral zone facing the collagen. Studies with silver nucleolar organizer region and proliferating cell nuclear antigen disclosed a high proliferative activity. Flow cytometric study showed an elevated S phase and 15% hypertetraploid cell population. These findings contrast the favorable prognosis of the tumor at 26 months follow-up. Probably, extracellular-matrix-induced maturation of the undifferentiated cells with the formation of basal lamina may account for this unique disparity.
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Clinical Neurology