Adrenal gland and paraganglia
Neuroblastic tumors
Ganglioneuroma


Topic Completed: 1 December 2014

Revised: 7 June 2019

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

PubMed Search: Ganglioneuroma [title] adrenal "loattrfree full text"[sb]

Carmen Perrino, M.D.
Debra Zynger, M.D.
Page views in 2018: 12,551
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Cite this page: Perrino C. Ganglioneuroma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalganglioneuroma.html. Accessed August 25th, 2019.
Definition / general
  • Mature, benign neoplasm that originates from neural crest cells of sympathetic ganglia or adrenal medulla
  • On a spectrum, from least → most differentiated: neuroblastomaganglioneuroblastoma → ganglioneuroma
Epidemiology
  • At all sites, usually age ≥7 years old
  • Patients with adrenal ganglioneuroma usually 4th to 5th decade (BMC Res Notes 2014;7:791)
  • Patients with ganglioneuroma of retroperitoneum or posterior mediastinum usually children (BMC Res Notes 2014;7:791)
  • M=F
  • Familial disposition and associated with Turner syndrome, multiple endocrine neoplasia type 2 (MEN2) (BMC Res Notes 2014;7:791)
Sites
  • Located along distribution of sympathetic nervous system
  • Most commonly in posterior mediastinum, followed by retroperitoneum (especially presacral space)
  • May occur in many locations, including adrenal gland (~20-30% of cases), cervical and parapharyngeal area, urinary bladder, prostate, bone, pancreas, skin, orbit, paratesticular area, appendix, gastrointestinal tract (Lack: Tumors of the Adrenal Glands and Extraadrenal Paraganglia, Vol.8, 2007)
Etiology
  • Clonal proliferation of cells of neural crest origin
Clinical features
  • Usually asymptomatic
  • May have non-specific signs/symptoms such as abdominal pain, palpable abdominal mass
  • Rarely, catecholamine synthesis may cause hypertension
Diagnosis
  • Tumor comprised of admixture of ganglion cells and Schwannian stroma/cells
Laboratory
  • Usually non-functional
  • Occasionally increased vanilmandelic acid and homovanillic acid in urine
Radiology description
  • Ultrasound: well-defined, homogeneous, hypoechogenic mass (BMC Res Notes 2014;7:791)
  • Computed tomography (CT): circumscribed and well-defined, hypodense, homogeneous or slightly heterogeneous lesion, surrounds peripheral blood vessels without compression or occlusion, may have fine/punctate calcifications (~40-60%), poorly enhanced by contrast medium (<40 Hounsfield units) (BMC Res Notes 2014;7:791)
  • Magnetic resonance imaging (MRI): (BMC Res Notes 2014;7:791)
    • T1: homogeneous, signal intensity lower than liver
    • T2: heterogeneous, signal intensity greater than liver; no absolute change in signal intensity on chemical shift imaging
    • Gadolinium administration: delayed and progressive, non-intense enhancement
Radiology images

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Various images

Prognostic factors
  • Benign tumor, good prognosis following adrenalectomy, long term follow-up is recommended (J Med Case Rep 2014;8:131)
  • Rarely malignant transformation of Schwannian stroma to malignant peripheral nerve sheath tumor (MPNST), may occur de novo or following abdominal radiation (Clin Endocrinol (Oxf) 2014;80:342)
Case reports
Treatment
Gross description
  • Well circumscribed
  • May have true capsule or fibrous capsule
  • Size varies, 8 cm to > 15 cm
  • Firm, resilient texture
  • Gray-white to tan-yellow
  • May have trabecular or whorled appearance
Gross images

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Well circumscribed multinodular tumor

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Trabecular or
whorled appearance
resembling leiomyoma

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Adrenal ganglioneuroma

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Adrenal ganglioneuroma, cut surface

Microscopic (histologic) description
  • Admixture of ganglion cells and Schwann cells
  • Ganglion cells
    • Mature to mildly dysmorphic
      • Mature: compact, eosinophilic cytoplasm with distinct cell borders, single eccentric nucleus, prominent nucleolus
      • Dysmorphic: single or multiple pyknotic nuclei
    • Vary in distribution and number, may be quite sparse
    • May contain finely granular, gold to brown pigment (lipofuscin or neuromelanin)
  • Schwann cells
    • May ensheath neuritic processes
    • May be arranged in small intersecting fascicles which are separated by loose myxoid stroma
  • Two histologic subtypes:
    1. Mature = every ganglion cell is mature
    2. Maturing = minor component of scattered collections of differentiating neuroblasts or maturing ganglion cells
    • Unlike intermixed subtype of ganglioneuroblastoma, these immature foci do not form distinct microscopic nests
  • Background may include lobules of mature adipose tissue (especially at periphery of lesion), mast cells, chronic inflammation, dense collagenized stroma
  • Mild variation in cellularity is permitted
  • No significant atypia, mitoses, or necrosis should be present
  • Masculinizing ganglioneuroma: admixture of ganglioneuroma and Leydig cells with crystalloids of Reinke or strands/clusters of cells resembling adrenal cortical cells
  • Composite tumor: rare; usually ganglioneuroma and pheochromocytoma (Ƭcomposite pheochromocytomaĆ®)
Microscopic (histologic) images

Contributed by Carmen Perrino, M.D. and Debra Zynger, M.D.
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Prominent myxoid background

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Scattered mature ganglion cells

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Cluster of mature ganglion cells

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Intervening stroma


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Ganglioneuroma (top) and residual normal adrenal cortical cells (bottom)

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Mature ganglion cells admixed with stroma

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Mature ganglion cells

Virtual slides

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Adrenal gland ganglioneuroma

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Pheochromocytoma
with focus of
ganglioneuroma

Cytology description
  • Biphasic with large, polyhedral ganglion cells and fibrillary stroma with spindle-shaped cells with cigar-shaped nuclei (J Cytol 2014;31:57)
Cytology images

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Schwann cells and admixed ganglion cells (insets), neck

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Schwann cells (a, c) and ganglion cells (b, d), neck

Electron microscopy description
  • Mixture of neural bundles and normal appearing ganglion cells with eccentric nuclei and large numbers of cytoplasmic organelles
Molecular / cytogenetics description
Differential diagnosis
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