PEDIATRIC MALIGNANT GLIOMA WITH TUBULORETICULAR INCLUSIONS AND MYCN AMPLIFICATION - REPORT OF A CASE WITH IMMUNOHISTOCHEMICAL, ULTRASTRUCTURAL, FLOW CYTOMETRIC, KARYOTYPIC, AND SOUTHERN BLOT ANALYSIS
V. Jay et al., PEDIATRIC MALIGNANT GLIOMA WITH TUBULORETICULAR INCLUSIONS AND MYCN AMPLIFICATION - REPORT OF A CASE WITH IMMUNOHISTOCHEMICAL, ULTRASTRUCTURAL, FLOW CYTOMETRIC, KARYOTYPIC, AND SOUTHERN BLOT ANALYSIS, Cancer, 73(7), 1994, pp. 1987-1993
Background. The authors described unusual pathologic features in a lef
t frontal lobe malignant glioma in a 3 1/2-year-old boy. The pathology
was similar in the initial excision and two subsequent recurrences at
9 and 11 months and at autopsy, when extensive subarachnoid spread wa
s noted. Methods. The tumor was studied by conventional histology, imm
unohistochemistry, flow cytometry, transmission electron microscopy (T
EM), immune electron microscopy (IEM), and cytogenetic and Southern bl
ot analysis. Results. The tumor revealed two different histologic patt
erns. One component showed large cells with eosinophilic cytoplasm, ve
sicular nuclei with prominent nucleoli, eosinophilic perinuclear inclu
sions, and immunoreactivity for glial fibrillary acidic protein (GFAP)
and vimentin. The other component consisted of undifferentiated cells
with hyperchromatic nuclei and scanty cytoplasm. By TEM, the perinucl
ear aggregates were composed of tubuloreticular inclusions, which were
also observed in endothelial cells within the tumor vasculature. By I
EM, the intermediate filaments in the tumor cell cytoplasm were decora
ted with GFAP. Flow cytometric results revealed a marked increase in t
he S-phase (48%), whereas cytogenetic analysis of short-term cultures
showed an abnormal karyotype containing marker chromosomes and double
minutes. In the second resection, additional karyotypic abnormalities
were noted, including 1p- and several additional markers. The first an
d second resections showed MYCN amplification by Southern Blot analysi
s in the 60- to 80-fold range. Conclusions. This tumor presents unique
histologic, ultrastructural, and cytogenetic findings as well as MYCN
amplification that is notable for a pediatric malignant glioma. Tubul
oreticular inclusions were a prominent feature in this tumor, which ag
ain is unique for a glioma.