BOF: 6
A 45
year old female presents with a history of left flank pain and haematuria. On
examination an abdominal mass is palpable in the left flank.
The
patient undergoes an MRI and the radiologist reports the presence of an
angiomyolipoma in the left kidney
Which
of the following underlying conditions is the patient likely to have?
a)
Tuberose sclerosis
b)
Neurofibromatosis
c)
Sturge Weber syndrome
d)
Von Hippel Lindau syndrome
e)
Osler Rendu Weber syndrome
Answer: a)
Angiomyolipoma
ü
Benign
renal neoplasm
ü
Composed of fat, vascular, and smooth muscle elements
May
be:
ü
Isolated angiomyolipoma
ü
Angiomyolipoma associated with tuberous sclerosis
Clinical features
ü
Majority asymptomatic
ü
Main
presenting symptoms are related to intratumoral or retroperitoneal haemorrhage
Other
symptoms (patients with associated tuberous sclerosis)
ü
Flank
or abdominal pain
ü
Weight
loss
ü
Hypertension
ü
Fever,
ü
Nausea
ü
Almost
half of these cases tumours are bilateral ( follow up important )
Revision Tip
Revise
tuberose sclerosis KEYS to SUCCESS in Medicine
page 484-486
