Neuroblastoma, Ganglioneuroblastoma and Ganglioneuroma
Definition
- Neoplasm arising from either the dorsal root ganglion of the spinal cord or the medulla of the adrenal gland, exhibiting variable degrees of neuroblastic maturation
Diagnostic Criteria
- Composed of neuroblasts exhibiting variable degrees of differentiation
up to ganglion cells
- Neuroblasts
- Small round nuclei with stippled ("salt and pepper") chromatin
- Large cell/large nucleolar phenotype
- Comprises approximately 8-10% of all neuroblastomas
- Nuclei 1.5 to 2 times larger than those of typical neuroblastoma cells
- 1-4 prominent nucleoli
- Usually seen in the undifferentiated and poorly differentiated subtypes of neuroblastoma (see below)
- Associated with MYCN amplification and a poor prognosis
- Large cell/large nucleolar phenotype
- Scant eosinophilic cytoplasm
- Indistinct cell borders
- Small round nuclei with stippled ("salt and pepper") chromatin
- Ganglion cells
- Large round nuclei with prominent nucleoli
- Abundant eosinophilic cytoplasm
- Nissl substance in cytoplasm
- Basophilic granules/bodies composed of endoplasmic reticulum
- Neuroblasts
- Background stroma also shows different levels of differentiation
- Schwannian stroma resembles collagen
- Requires S100 stain (positive) for identification
- Neuropil
- Pink, fibrillary extracellular material
- Dense lymphoid infiltrate occasionally present
- Lymphoid follicles may be present
- Often seen in patients with Opsoclonus-Myoclonus-Ataxia (OMA) paraneoplastic syndrome
- Schwannian stroma resembles collagen
- Seven subtypes of neuroblastic tumors are recognized according to the degree of neuroblastic maturation and the amount of background schwannian stroma
- Neuroblastoma, undifferentiated
- Composed entirely of neuroblasts, no ganglion cell maturation
- Immunohistochemistry is often required to confirm neuroblastic lineage
- Schwannian stroma poor
- Less than 50% of background stroma is schwannian
- May be absent
- Less than 50% of background stroma is schwannian
- Composed entirely of neuroblasts, no ganglion cell maturation
- Neuroblastoma, poorly differentiated
- Predominantly neuroblasts, <5% maturing/mature ganglion cells
- At least one focus of neuropil
- Schwannian stroma poor
- Less than 50% of background stroma is schwannian
- May be absent
- Less than 50% of background stroma is schwannian
- Neuroblastoma, differentiating
- Predominantly neuroblasts but >5% maturing/mature ganglion cells
- At least one focus of neuropil
- Schwannian stroma poor
- Less than 50% of background stroma is schwannian
- May be absent
- Less than 50% of background stroma is schwannian
- Ganglioneuroblastoma, nodular
- Predominantly maturing/mature ganglion cells but at least one well circumscribed nodule of residual neuroblasts
- Neuroblast nodules may correspond to hemorragic nodules in gross specimen
- Schwannian stroma rich
- 50% or more of background stroma is schwannian
- Predominantly maturing/mature ganglion cells but at least one well circumscribed nodule of residual neuroblasts
- Ganglioneuroblastoma, intermixed
- Predominantly maturing/mature ganglion cells but at >1 foci of residual neuroblasts intermixed with ganglion cells
- No distinct hemorragic nodules in gross specimen
- Schwannian stroma rich
- 50% or more of background stroma is schwannian
- Predominantly maturing/mature ganglion cells but at >1 foci of residual neuroblasts intermixed with ganglion cells
- Ganglioneuroma, maturing
- Entirely composed of maturing and mature ganglion cells, no residual neuroblasts
- No distinct hemorragic nodules in gross specimen
- Schwannian stroma rich
- 50% or more of background stroma is schwannian
- Entirely composed of maturing and mature ganglion cells, no residual neuroblasts
- Ganglioneuroma, mature
- Entirely composed of mature ganglion cells, no residual neuroblasts
- No distinct hemorragic nodules in gross specimen
- Schwannian stroma rich
- 50% or more of background stroma is schwannian
- Entirely composed of mature ganglion cells, no residual neuroblasts
- Neuroblastoma, undifferentiated
Florette K. Gray Hazard MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/updates: 1/11/11, 1/29/11, 6/23/13
