Adrenal gland and paraganglia
Neuroblastic tumors
Ganglioneuroblastoma - other, intermixed


Topic Completed: 1 October 2014

Revised: 7 June 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Ganglioneuroblastoma [title] intermixed

See Also: Nodular variant

Carmen Perrino, M.D.
Debra Zynger, M.D.
Page views in 2018: 831
Page views in 2019 to date: 765
Cite this page: Perrino C, Zynger D. Ganglioneuroblastoma, intermixed variant. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalganglioneuroblastomaother_intermixed.html. Accessed August 18th, 2019.
Definition / general
  • Ganglioneuroblastoma: neoplasm of neuroectodermal origin comprised of mixture of neuroblasts and ganglion cells in varying proportions
    • Divided into stroma-rich (well-differentiated, intermixed, nodular) and stroma-poor categories depending on amount of Schwannian, spindle cell stroma
  • Intermixed variant: composite tumor in the "stroma-rich" category
    • Ganglioneuromatous tissue with interspersed, sharply defined, unencapsulated nests of variably differentiated neuroblastic cells
Epidemiology
  • 4th most common tumor in childhood
  • 75 - 85% occur within first 4 years of life
  • M=F
Sites
  • Occurs anywhere in anatomic distribution of sympathoadrenal neuroendocrine system
  • ~80% arise in abdomen or adrenal gland, ~20% arise in thoracic cavity
Etiology
  • Clonal proliferation of immature cells of neural crest origin
Clinical features
Radiology description
  • MRI: hypointensity on T1-weighted image with rapid enhancement and hyperintensity on T2-weighted image (Intern Med 1995;34:1168)
Radiology images

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Spiral CT scan

Prognostic factors
Case reports
Treatment
  • Depends on prognostic stage (Pediatr Blood Cancer 2009;53:563, UpToDate - Treatment and prognosis)
    • Low risk
      • Surgical resection alone is mainstay
      • Chemotherapy only if tumor is unresectable or symptoms of spinal cord/respiratory/bowel compromise
      • Expectant observation in some infants with small adrenal masses, localized neuroblastoma, or asymptomatic stage 4S disease
    • Intermediate risk
      • Surgical resection
      • Moderate chemotherapy
      • Radiation only if disease progresses despite surgery/chemotherapy
    • High risk
      • Induction: intensive chemotherapy
      • Local control: surgical resection, radiation
      • Consolidation: chemotherapy, myeloablative therapy, autologous stem cell transplant
      • Maintenance: cis-retinoic acid or immunotherapy
Gross description
  • In comparison with neuroblastoma, more homogeneous and mature appearance
  • Varies, from circumscribed, ovoid mass to large, multilobulated tumor
  • Calcification (chalky white, yellow areas) and cystic degeneration may occur
Gross images

Contributed by Dr. Carmen Perrino and Dr. Debra Zynger
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Composite types

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Stroma-rich, intermixed type



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Brownish gray solid cut surface

Microscopic (histologic) description
  • Architecture: lobular, diffuse/solid, organoid
  • Ganglioneuromatous tissue with scattered, sharply defined, unencapsulated nests of variably differentiated neuroblastic cells
  • Neuroblasts
    • Homer Wright pseudorosettes: circular, ovoid, angular zones of pale-staining neuritic cell processes surrounded by tumor cell nuclei; may rarely palisade
    • Minimal cytoplasm, may have cytoplasmic tail
    • Round to ovoid nuclei with stippled salt and pepper chromatin, inconspicuous nucleoli
  • Ganglion cells
    • Distinct cell borders
    • Abundant granular eosinophilic cytoplasm (Nissl substance is rough endoplasmic reticulum)
    • Nuclear enlargement, eccentric nuclei, prominent nucleoli
  • May see neuromelanin pigment (brown, finely granular; rarely present), cystic degeneration, hemorrhage, dystrophic calcification
Microscopic (histologic) images

Contributed by Dr. Carmen Perrino and Dr. Debra Zynger
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Composite types

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Intermixed type

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Ganglion cells

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Fibrillary background


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Aggregate of ganglion cells

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Lymphocyte aggregate

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Area with Schwannian stroma

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Maturing ganglion cells

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Stroma-rich, intermixed type


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Ganglion cells and
undifferentiated
small round cells

Cytology description
  • Ganglion cells: larger cells, abundant cytoplasm, fine chromatin, prominent nucleoli
  • Neuroblasts: uniform, small, blue cells with scant, eosinophilic, fibrillary cytoplasm; hyperchromatic to vesicular chromatin
    • May form Homer Wright pseudorosettes
Positive stains
Negative stains
Electron microscopy description
Molecular / cytogenetics description
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