Adrenal gland & paraganglia
Pheochromocytoma / paraganglioma
Hyperplasia-paraganglia
Author: Lan L. Gellert, M.D., Ph.D.
Last author update: 1 June 2017
Last staff update: 9 June 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Hyperplasia paraganglia
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosis | Board review style question #1 | Board review style answer #1Cite this page: Gellert LL. Hyperplasia-paraganglia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalparagangliahyperplasia.html. Accessed December 22nd, 2024.
Definition / general
- Usually in carotid bodies (may be due to chronic hypoxemia, Am J Pathol 1978;91:497), associated with high altitude or cardiopulmonary disease
- Adrenal medulla occasionally affected, if so, usually bilateral, syndromic (e.g. multiple endocrine neoplasia type 2/MEN2, mutations in SDHB/succinate dehydrogenase iron-sulfur subunit), or sporadic
- Adrenal medullary hyperplasia may be a precursor for pheochromocytoma in MEN2 syndrome (Neoplasia 2014;16:868)
- May also affect vagal and aorticopulmonary paraganglia
Clinical features
- From nonsymptomatic to hypertensive
Case reports
- 25 year old patient with bilateral adrenomedullary hyperplasia and germline MAX mutation (Endocr Pathol 2017;28:302)
- Hyperplasia of the aorticopulmonary paraganglia in patients with sudden infant death syndrome (Cardiologia 1998;43:953)
- 12 cases of adrenal medullary hyperplasia (Int J Endocrinol 2014;2014:752410)
Treatment
- Adrenalectomy if symptomatic and clinically indicated
Gross description
- Marked increase in weight, could be over 30 g
- Diameter > 5 mm
Microscopic (histologic) description
- Increased number of lobules, some confluent, composed of increased numbers of sustentacular cells and chief cells with hyperchromatic and mildly enlarged nuclei
Microscopic (histologic) images
Contributed by Lan L. Gellert, M.D., Ph.D.
Adrenal medullary hyperplasia
Positive stains
- Chief cells: synaptophysin+, chromogranin+
- Sustentacular cells: S100+
Differential diagnosis
- Paraganglioma: extra-adrenal, usually > 300 mg, may have higher density of chief cells
- Pheochromocytoma: > 1 cm, but otherwise morphologically similar to adrenal medullary hyperplasia
Board review style question #1
Which of the following differentiate adrenal medullary hyperplasia from pheochromocytoma?
- Cytological atypia
- Infiltrative border
- Mitosis
- Size
Board review style answer #1
D. Size. In clinical practice, adrenal medullary hyperplasia is defined as adrenal medullary proliferation with a size < 1 cm, while larger lesions are considered as pheochromocytoma.
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