Adrenal gland and paraganglia
Neuroblastic tumors
Ganglioneuroma

Author: Carmen Perrino, M.D. (see Authors page)
Editor: Debra Zynger, M.D.

Revised: 22 January 2016, last major update December 2014

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Ganglioneuroma [title] adrenal

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
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
Clinical Images
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Various images

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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COW #319, 34 year old man


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Well circumscribed gray-white tumor

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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

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Composite ganglioneuroma and pheochromocytoma

Micro 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®)
Micro Images
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Case of the Week #319:

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Contributed by Professor D. Y. Cohen, Herzliyah Medical Center, Israel: retroperitoneal tumor

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Chromogranin

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Neurofilament

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S100


Flickr images courtesy of 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


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Polygonal tumor cells in well defined nests

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Atypical polygonal chromaffin cells

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Ganglion cells and Schwann cells

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Abundant Schwann cells

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Clusters of ganglion cells

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Ganglion cells surrounded by Schwann cells

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Leydig cells

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H&E, S100, neurofilament

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S100

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Vimentin+, synaptophysin+

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