Mixed conventional and desmoplastic infantile ganglioglioma: An autopsied case with 6-year follow-up

Takashi Komori, Bernd W. Scheithauer, Joseph E Parisi, Jan Watterson, John R. Priest

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

We describe a case of desmoplastic infantile ganglioglioma (DIG) arising in the ventral diencephalon of a 3-1/2-month-old boy. On biopsy, the tumor featured a desmoplastic, S-100 protein and GFAP immunoreactive stromal element, as well as a variable spectrum of ganglion cells. Electron microscopy demonstrated astrocytes, and morphologically fibroblasts, as well as neurons containing 120-nm dense core granules. In addition, tubular structures composed of tightly apposed cells with features of astrocytes and of Schwann-like cells were also noted. Devoid of fibroblasts, the tubular structures were surrounded by a single basal lamina. At autopsy 6 years later, the multinodular, cystic mass had replaced the diencephalon, extended into both temporal lobes as well as the optic nerves, and showed marked leptomeningeal involvement. Microscopically, superficial portions of the tumor consisted of typical DIG, whereas deep, nondesmoplastic portions exhibited pattern variation ranging from pilocytic astrocytoma to ganglioglioma and gangliocytoma. There was also a minor element of small, 'primitive-appearing' neuroepithelial cells. Dysplastic ganglion cells variously reactive for neurofilament protein and synaptophysin were present throughout the tumor. Our study not only confirms DIG as a variant of ganglioglioma, one capable of slow growth, infiltration, and fatal progression but suggests that its differentiating potential includes elements of both the central and peripheral nervous systems. If so, their derivation may be from multipotential cells of the neural plate.

Original languageEnglish (US)
Pages (from-to)720-726
Number of pages7
JournalModern Pathology
Volume14
Issue number7
DOIs
StatePublished - 2001

Fingerprint

Ganglioglioma
Diencephalon
Ganglia
Astrocytes
Fibroblasts
Neuroepithelial Cells
Ganglioneuroma
Neural Plate
Neurofilament Proteins
Neoplasms
Synaptophysin
S100 Proteins
Schwann Cells
Astrocytoma
Peripheral Nervous System
Temporal Lobe
Optic Nerve
Basement Membrane
Autopsy
Electron Microscopy

Keywords

  • Desmoplastic infantile ganglioglioma (DIG)
  • Divergent differentiation
  • Ganglioglioma
  • Schwann cell

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Komori, T., Scheithauer, B. W., Parisi, J. E., Watterson, J., & Priest, J. R. (2001). Mixed conventional and desmoplastic infantile ganglioglioma: An autopsied case with 6-year follow-up. Modern Pathology, 14(7), 720-726. https://doi.org/10.1038/modpathol.3880379

Mixed conventional and desmoplastic infantile ganglioglioma : An autopsied case with 6-year follow-up. / Komori, Takashi; Scheithauer, Bernd W.; Parisi, Joseph E; Watterson, Jan; Priest, John R.

In: Modern Pathology, Vol. 14, No. 7, 2001, p. 720-726.

Research output: Contribution to journalArticle

Komori, T, Scheithauer, BW, Parisi, JE, Watterson, J & Priest, JR 2001, 'Mixed conventional and desmoplastic infantile ganglioglioma: An autopsied case with 6-year follow-up', Modern Pathology, vol. 14, no. 7, pp. 720-726. https://doi.org/10.1038/modpathol.3880379
Komori, Takashi ; Scheithauer, Bernd W. ; Parisi, Joseph E ; Watterson, Jan ; Priest, John R. / Mixed conventional and desmoplastic infantile ganglioglioma : An autopsied case with 6-year follow-up. In: Modern Pathology. 2001 ; Vol. 14, No. 7. pp. 720-726.
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