Microscopic Description:

H&E-stained sections reveal a well-differentiated tumor composed of mature ganglion cells and Schwann cells interspersed within a fibrous stroma. These cells also lack signs of malignancy, and should show no evidence of atypia, mitotic activity, or necrosis.

Certain foci show calcification.

Pathological examination show the ganglion cells which exhibit abundant eosinophilic cytoplasm and vesicular nuclei and prominent nucleoli, interspersed in the stroma with spindle-shaped Schwann cells and nerve bundles.

Immunostains performed:

Immunostains performed: positive S100, Neurofilament, synaptophysin; negative GATA3.

Discussion:

Ganglioneuroma is a rare, benign tumor that arises from the sympathetic nervous system, commonly in the posterior mediastinum or retroperitoneum, and in the adrenal gland as well.

Ganglioneuromas are rare tumors that most often start in autonomic nerve cells. Ganglioneuromas are typically asymptomatic and usually noncancerous (benign). They are also slow-growing tumors.

  • Though normally asymptomatic and slow-growing, when they start to compress/impact nearby structures and organs, they can cause symptoms.

Ganglioneuromas usually occur in people over 10 years of age.

Ganglioneuromas arise from the neural crest tissue and are considered a well-differentiated, mature type of neuroblastic tumor with matured ganglion cells and Schwann cells. The lack of immature neuroblastic components distinguishes them from neuroblastoma and ganglioneuroblastoma.

Histologically, ganglioneuroma is characterized by a mixture of mature ganglion cells within a Schwannian-rich stroma.

The IHC profile of ganglioneuroma shows expression of mature ganglion cells and Schwann cells (S-100 protein, synaptophysin, neuron-specific enolase (NSE), and while glial fibrillary acidic protein (GFAP)).

The primary treatment for ganglioneuromas is the surgical removal of the tumor. Ganglioneuromas rarely metastasize, so tumor resection is the most favorable treatment.

References:

Bharti RR, Kumar S, Kumar B. Adrenal Ganglioneuroma: A Rare Incidentaloma. National Journal of Laboratory Medicine. 2021;10(2):PC03-PC04

Eldin MM, Daum RE, Kumar P, Uecker J. Adrenal Ganglioneuroma: Diagnosis, Presentation, and Management of a Rare Tumor. Cureus. 2023;15(6):e39977. doi:10.7759/cureus.39977

Leão RR, Pereira BJ, Borges R, Grenha V. Adrenal ganglioneuroma: a rare incidental finding. BMJ Case Rep. 2013;2013:bcr2012008067. Published 2013 Jan 30. doi:10.1136/bcr-2012-008067

Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics. 2002;22(4):911-934. doi:10.1148/radiographics.22.4.g02jl15911

Mylonas KS, Schizas D, Economopoulos KP. Adrenal ganglioneuroma: What you need to know. World J Clin Cases. 2017;5(10):373-377. doi:10.12998/wjcc.v5.i10.373

Radhakrishnan S, Rao R, Lashkari HP, et al. A needle in the haystack: An unusual case presentation of ganglioneuroblastoma at a tertiary care center in Coastal Karnataka. Clin Case Rep. 2023;11(11):e8149. doi:10.1002/ccr3.8149

Rao RN, Kumar R, Kumar M, et al. Composite pheochromocytoma-ganglioneuroma of the adrenal gland: A case report. Urology Annals. 2013;5(2):115-118

https://www.sciencedirect.com/topics/medicine-and-dentistry/ganglioneuroma

https://www.cap.org/member-resources/pathology-case-challenge/adrenal-gland-4

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