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adrenal cytomegaly

Wednesday 18 January 2006

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

- Case #10479

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.

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  • Focal adrenal cytomegaly in 47,XXX fetus ith ring chromosome (...)
  • Focal adrenal cytomegaly in 47,XXX fetus ith ring chromosome (...)
  • Focal adrenal cytomegaly in 47,XXX fetus ith ring chromosome (...)