Adrenal gland and paraganglia - Beckwith-Wiedemann syndrome

Home Chapter Home Jobs Conferences Fellowships Books

Adrenal gland and paraganglia

Congenital anomalies

Beckwith-Wiedemann syndrome

Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 6 February 2013, last major update February 2005
Copyright: (c) 2002-2013, PathologyOutlines.com, Inc.

General
=========================================================================

● Visceromegaly, gigantism, macroglossia, abdominal wall defects, craniofacial abnormalities, midfacial hypoplasia and adrenocortical hyperplasia
● Due to abnormality of 11p15.5
● Incidence of 1 per 13,000 births, usually sporadic
● May have brain damage due to hypoglycemia, causing mental retardation or death
● May develop Wilm’s tumor, adrenocortical carcinoma, neuroblastoma, pancreatoblastoma or pheochromocytoma

Gross description
=========================================================================

● Enlarged adrenal glands up to 16g, may be cerebriform and nodular

Gross images
=========================================================================

Large lobulated adrenal glands

Micro description
=========================================================================

● Bilateral cytomegaly with large and pleomorphic nuclei and cytoplasmic nuclear pseudoinclusions
● Also medullary hyperplasia; variable hemorrhagic macrocysts

Micro images
=========================================================================

Adrenal cytomegaly

End of Adrenal gland and paraganglia > Congenital anomalies > Beckwith-Wiedemann syndrome

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).