CNS tumor
Neuronal and mixed neuronal - glial tumors
Desmoplastic infantile astrocytoma / ganglioglioma

Author: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 30 August 2017, last major update November 2013

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Desmoplastic infantile astrocytoma ganglioglioma

Cite this page: Abdelzaher, E. Desmoplastic infantile astrocytoma / ganglioglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumordesmoplasticinfantileganglioglioma.html. Accessed September 25th, 2017.
Definition / general
  • Supratentorial superficially located cystic neuroepithelial tumors of infancy characterized by prominent desmoplasia with neoplastic glial component (desmoplastic infantile astrocytoma, DIA) or neoplastic glioneuronal component (desmoplastic infantile ganglioglioma, DIG)
  • Corresponds to WHO grade I
  • DIA first described in 1982 by Taratuto et al. (J Neurosurg 1987;66:58)
  • DIG first described in 1987 by VandenBerg et al.
  • Since both lesions have similar radiological and clinical presentation, they are categorized together as desmoplastic infantile astrocytoma / ganglioglioma (DIA / DIG) in the WHO classification
Epidemiology
  • Incidence: rare ( < 0.1% of CNS tumors)
  • Age and sex:
Sites
  • Supratentorial
  • Usually frontoparietal
Clinical features
  • Rapidly increasing head circumference, hydrocephalus, seizures
Radiology description
  • Supratentorial
  • Superficially located
  • Large, involves more than one lobe
  • Cystic with solid area / mural nodule
  • Enhancing
  • Desmoplastic area exhibits hypointense signal on T2
Radiology images

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Large cystic and solid mass; solid mass enhances with contrast

Large right hemispheric mass

Prognostic factors
Case reports
  • 3 1/2 month old boy with mixed conventional and desmoplastic infantile ganglioglioma (Mod Pathol 2001;14:720)
Treatment
  • Gross total resection
  • Chemotherapy if infiltrative or progressive
  • Residual disease may not grow and may spontaneously regress (Neurosurgery 2003;53:979)
Gross description
  • Large (up to 13 cm), often involves multiple lobes
  • Deep macrocystic portion
  • Superficial solid leptomeningeal portion, firm to hard, focally attached to overlying dura
Microscopic (histologic) description
  • Well delineated from normal brain
  • Desmoplastic leptomeningeal component
    • Involve the subarachnoid space and extends into Virchow-Robin spaces
    • Neoplastic neuroepithelial cells in desmoplastic spindled stroma arranged in fascicular and storiform patterns with pericellular reticulin deposition lending a mesenchymal appearance
    • Neoplastic neuroepithelial cells:
      • Astrocytic cells:
        • Only component in DIA
        • Spindled or gemistocytic neoplastic astrocytes
      • Neuronal component:
        • Seen in DIG in addition to neoplastic astrocytes
        • Small ganglion cells
        • Uncommonly large ganglion cells or areas resembling ganglioglioma
  • Immature small cell component (unclear prognostic significance)
    • Hypercellular poorly differentiated neuroepithelial cells
    • No desmoplasia
    • May show mitoses, vascular proliferation or necrosis
  • Calcification common, chronic inflammatory cells uncommon
  • Exceptionally, frank anaplastic features are encountered (high mitotic rate, vascular proliferation, palisading necrosis and high proliferation index)
Microscopic (histologic) images

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Desmoplastic infantile astrocytoma



Desmoplastic ganglioglioma:

Desmoplastic infantile ganglioglioma

Round to triangular cells

GFAP, NeuN, synaptophysin, reticulin


No necrosis or endothelial proliferation

Extensive desmoplasia (trichrome)

Extensive pericellular reticulin

Strong diffuse GFAP+

Cytology description
  • Low cellularity
  • Dispersed or variably sized clusters of large neuronal cells with abundant granular cytoplasm, eccentric hyperchromatic nuclei with undulating nuclear membranes and occasional binucleation, prominent nucleoli
  • Astroglial cells with smaller cytoplasmic rim, nuclear hyperplasia and more prominent irregularities in nuclear membranes
  • May have prominent degenerative changes, foamy macrophages
  • No vascular structures (Cytojournal 2005;2:1)
Cytology images

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Desmoplastic infantile ganglioglioma

Immunohistochemistry
Electron microscopy description
  • Astrocytic tumor cells are partly invested by pericellular basal lamina