Adrenal gland & paraganglia
Pheochromocytoma / paraganglioma
Hyperplasia-medulla
Author: Pallav Gupta, M.D.
Last author update: 1 November 2013
Last staff update: 4 July 2020
Copyright: 2002-2019, PathologyOutlines.com, Inc.
PubMed Search: Adrenal medullary hyperplasia [title]
Table of Contents
Definition / general | Etiology | Clinical features | Diagnosis | Laboratory | Radiology description | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Gupta P. Hyperplasia-medulla. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalhyperplasia.html. Accessed December 4th, 2024.
Definition / general
- Increase in mass of adrenal medullary cells and expansion into areas of gland where not normally present, such as tail
Etiology
- Sporadic cases associated with cystic fibrosis, sudden infant death syndrome, nonfamilial Beckwith-Wiedemann syndrome
- Familial cases associated with MEN 2a, 2b/3, von Hippel-Lindau disease, neurofibromatosis type 1
Clinical features
- May cause hypertensive symptoms similar to pheochromocytoma, Cushing Syndrome
- Bilateral; either nodular or diffuse
- Note: nodular hyperplasia in MEN 2a or 2b patients may act similar to pheochromocytoma
Diagnosis
- Based on morphometry (medullary volume > 10% of gland)
Laboratory
- Increased urinary levels of catecholamines
Radiology description
- On ultrasound, bilateral adrenal medullary hyperplasia is seen as a highly echogenic linear structure
- On CT, seen as high-density linear structure (Crit Rev Diagn Imaging 1992;33:437)
Case reports
- 61 year old man with bilateral adrenal medullary hyperplasia associated with an SDHB mutation (J Clin Oncol 2011;29:e200)
- Adrenomedullary function in patients with nonclassic congenital adrenal hyperplasia (Horm Metab Res 2010;42:607)
Treatment
- Bilateral adrenalectomy (JSLS 2013;17:433)
Gross description
- Familial cases usually have multiple, unencapsulated, gray-tan nodules in both glands
Gross images
Images hosted on other servers:
Left adrenal gland
Microscopic (histologic) description
- Alveolar, trabecular or solid patterns of medullary cells with variable size and shape
- Often medullary tissue in alar and tail regions of gland
Microscopic (histologic) images
Images hosted on other servers:
Diffuse and nodular adrenal medullary hyperplasia
Positive stains
- Chromogranin, synaptophysin, NSE
Negative stains
- Inhibin
Electron microscopy description
- Resembles normal medullary cells
Molecular / cytogenetics description
- May be monoclonal
Differential diagnosis
- Cortical atrophy: makes medulla appear prominent
- Pheochromocytoma: usually > 1 cm; increased urinary excretion of epinephrine, norepinephrine, VMA and metanephrines
