adrenal cytomegaly
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Definition: Adrenal cytomegaly is characterized by the presence of large polyhedral cells with eosinophilic granular cytoplasm and enlarged nuclei in the adrenal cortex. Adrenal cytomegaly is found in 0.8% pediatric autopsies.
Adrenal cytomegaly is relatively common finding within fetal cortex in newborns, particularly premature infants (3-7%) or infants with Rh incompatibility.
Adrenal cytomegaly may be associated with Beckwith-Wiedemann syndrome
Focal adrenal cytomegaly can be observed in normal adults (#3778124#)
Macroscopy
hyperplastic adrenal glands
Microscopy
numerous markedly enlarged (up to 150 microns) and bizarre polyhedral cells with eosinophilic granular cytoplasm and large hyperchromatic nuclei with pseudoinclusions in adrenal cortex.
large polyhedral cells in the adrenal cortex
- eosinophilic granular cytoplasm
- enlarged nuclei
false intranuclear inclusions (#6264884#)
no/rare mitotic figures
Electron microscopy
nuclear inclusions composed of the invaginated portion of the cytoplasm surrounded by double membrane and contained numerous organelles. (#6264884#)
Etiology (Exemples)
Beckwith-Wiedemann syndrome (#1849635#, #2845376#)
isolated (#3778124#, #3838445#)
- in adults (#3778124#)
Associations
diaphragmatic hernia (#10879233#)
premature infants (3-7%)
infants with Rh incompatibility
Case records
Differential diagnosis
adrenal CMV infection
- usually infants
- basophilic cytoplasm
- large intranuclear inclusion surrounded by a clear halo
References
Ong BB, Wong KT. Adrenal cytomegaly associated with diaphragmatic hernia: report of a case.Malays J Pathol. 1996 Dec;18(2):121-3. PMID: #10879233#
Fasano M, Greco MA. Proliferative activity of adrenal glands with adrenocortical cytomegaly measured by MIB-1 labeling index. Pediatr Pathol Lab Med. 1996 Sep-Oct;16(5):765-76. PMID: #9025875#
Favara BE, Steele A, Grant JH, Steele P. Adrenal cytomegaly: quantitative assessment by image analysis. Pediatr Pathol. 1991 Jul-Aug;11(4):521-36. PMID: #1946074#
Camuto PM, Wolman SR, Perle MA, Greco MA. Flow cytometry of fetal adrenal glands with adrenocortical cytomegaly. Pediatr Pathol. 1989;9(5):551-8. PMID: #2813201#
Yamashina M. Focal adrenocortical cytomegaly observed in two adult cases. Arch Pathol Lab Med. 1986 Nov;110(11):1072-5. PMID: #3778124#
Feuerstein IM, Tiamson EM. Focal cytomegaly of the adult adrenal cortex. A rare variant of black adenoma. Arch Pathol Lab Med. 1985 Feb;109(2):198-200. PMID: #3838445#
Nakamura Y, Yano H, Nakashima T. False intranuclear inclusions in adrenal cytomegaly. Arch Pathol Lab Med. 1981 Jul;105(7):358-60. PMID: #6264884#
Gau GS, Bennett MJ. Fetal adrenal cytomegaly. J Clin Pathol. 1979 Mar;32(3):305-6. PMID: #429599#
Marsden HB, Zakhour HD. Cytomegalic adrenal hypoplasia with pituitary cytomegaly. Virchows Arch A Pathol Anat Histol. 1978 Jun 2;378(2):105-10. PMID: #149412#
Mamelle JC, David M, Riou D, Gilly J, Trouillas J, Dutruge J, Gilly R. [Congenital adrenal hypoplasia of cytomegalic type. Recessive, sex-linked form. Apropos of 3 cases] Arch Fr Pediatr. 1975 Feb;32(2):139-59. French. PMID: #1217948#
Aterman K, Kerenyi N, Lee M. Adrenal cytomegaly. Virchows Arch A Pathol Pathol Anat. 1972;355(2):105–122.
Niggemeyer H, Sandhage K. [Thymus gland hyperplasia: congenital, in association with adrenal gland hypoplasia and cytomegaly or myocarditis respectively]. Klin Padiatr. 1972 Nov;184(6):501-7. PMID: #4346363#
Borit A, Kosek J. Cytomegaly of the adrenal cortex. Electron microscopy in Beckwith’s syndrome. Arch Pathol. 1969 Jul;88(1):58-64. PMID: #5793692#
Birdwell TR, Dimmette RM. Cytomegaly of the adrenal gland. An interesting finding: report of three cases. Am J Clin Pathol. 1967 May;47(5):585–587.