Beckwith-Wiedemann syndrome associets visceromegaly, gigantism, macroglossia, abdominal wall defects, craniofacial abnormalities, midfacial hypoplasia and adrenocortical hyperplasia.
Macroscopy
enlarged adrenal glands up to 16g
cerebriform and nodular
large lobulated adrenal glands
Microscopy
bilateral adrenal cytomegaly
- large and pleomorphic nuclei
- nuclear pseudoinclusions
medullary hyperplasia
variable hemorrhagic macrocysts
See also
Beckwith-Wiedemann syndrome (BWS)
- 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, pheochromocytoma
- is due to abnormality of 11p15.5