Neurogenic tumours
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Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder characterised by a unique set features which can consist of :
•macroglossia - most common clinical finding 4
•otic dysplasia ref
•omphalocoele
•localised gigantism / macrosomia
•hemihypertrophy
•cardiac anomalies
•pancreatic islet cell hyperplasia
•organomegalies
nephromegaly hepatosplenomegaly
Epidemiology
The incidence is reported at ~ 1:13,000-37,000 live births.
Pathology
The majority of cases are sporadic while a small proportion (~15%) is autosomal dominant with abnormality related to chromosome 11p15.5. The syndrome carries a well recognised increased risk for the development of neoplasia 4. Another 10-20 of cases show paternal disomy 11.
Associations
•Wilms tumour(s) 1 of kidneys
•polyhydramnios
•non malignant renal diseases 3
◦medullary renal cysts
◦calyceal diverticula
◦nephrolithiasis
•pre-malignant renal diseases
◦perilobar nephroblastomaosis
• adrenocortical carcinoma
• gonadoblastoma
• rhabdomyosarcoma
• neuroblastoma
• pancreatoblastoma
• hepatoblastoma
• placental pathology
◦placentomegaly
◦molar degeneration of placenta
• post natal hypoglygaemia
• genito-urinary anomalies
◦hypospadias
◦cryptorchidism
Etymology
First described by Beckwith and Wiedemann in 1965.
Adrenal metastases
Adrenal metastases are the most common malignant lesions involving the adrenal gland. Metastases are usually bilateral but may also be unilateral. When unilateral involvement is thought to be more common on the left side (ratio of 1.5:1) 4.
Epidemiology
They are thought to be present in up to 27% of patients with known malignant epithelial tumours at autopsy.
Pathology
Primary sites
There are many priary tumours that can potentially metastasise to the adrenal glands.
Common primary sites of tumours that metastasize to the adrenal glands include
17.lung cancer
18.bowel - colorectal carcinoma
19.breast cancer
20.pancreatic cancer
Other reported primary tumours include
•renal cell carcinoma
•hepatocellular carcinoma 3
•malignant melanoma
Radiographic features
CT
Adrenal metastases can have a variable CT appearances 4. Usually demonstrates les than 50% washout.
In patients with renal cell carcinoma and hepatocellular carcinoma who undergo dedicated adrenal CT imaging for known adrenal lesions, the percentage enhancement washout of adrenal metastases can be similar to that of lipid-poor adrenal adenomas
MRI
Exact signal characteristics can vary dependant on the type of tumour.
In general, commonly described signal characteristics include
•T1 - usually exhibit low signal intensity 2
•T2 - usually exhibit high signal intensity 2
•C+ (Gd) - usually has progressive enhancement after administration of contrast material 2.
An important diagnostic feature is the lack of signal loss on out-of-phase images (in contradistinction to that seen with adrenal adenoma)
See also
•adrenal lesions
tumours of the adrenal gland
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