Malignant predominantly minigemistocytic glioma in two infants: a distinctive glioma variant?

We report unusual distinctive histopathological features in malignant supratentorial tumours of two infants (patient 1: congenital, patient 2: 30 months). Both patients had paraventricularly located well-delineated tumours. Gross total resection could be performed and postoperative chemotherapy was administered. At the last follow-up, 18 (patient 1) and 10 months (patient 2) postoperatively, both patients were in continuous complete remission. Histologically, both tumours were characterized by high cellular density and monomorphic appearance. Tumour cells were small to medium-sized and the majority of cells showed a distinctive minigemistocytic shape. A small fraction of cells lacked a distinct cytoplasm. Mitotic figures were abundant, tumour necrosis and hypertrophic vascular proliferations were absent. Immunohistochemically, the tumour cells expressed glial (GFAP, S100) and focally neuronal (NFP) proteins. Comparative genomic hybridization showed few, dissimilar chromosomal aberrations in the two tumours. Although sharp demarcation and monomorphic architecture of both tumours are reminiscent of a primitive neuroectodermal tumour, cytological and immunohistochemical glial differentiation refer to a glial tumour origin. To our knowledge the histopathological features of the described tumours do not correspond unequivocally to any established glioma variant and could represent a distinctive new glioma subtype.