Vol 44 # 2 June 2012 - Kma.org.kw

June 2012
KUWAIT MEDICAL JOURNAL 152
anemia or hemorrhage [7, 8] . Our patient had abdominal
pain, vomiting, mass, anemia and retroperitoneal
hemorrhage. Patients with angiomyolipoma associated
with tuberous sclerosis present at a younger age, are
more likely to be symptomatic, have large bilateral
tumors that are more likely to grow and therefore
more frequently require surgery. In the series by
Steiner et al [15] , the average size of angiomyolipoma in
patients with tuberous sclerosis was 9.6 ± 4.8 cm, and
in those without tuberous sclerosis, was 4.1 ± 3.4 cm.
Angiomyolipoma may grow to be large and bulky and
extend into the perirenal space, which sometimes makes
it difficult to differentiate from perirenal liposarcoma.
However, perirenal liposarcoma is unilateral and
usually spares the architecture of the kidney because
it arises from retroperitoneal fat. In comparison, renal
angiomyolipoma arises from the renal parenchyma and
involves the kidney itself [16] . Our patient had bilateral
massive angiolipomatosis, the renal parenchyma being
inseparable from the lesion. The mass on the right side
measured 24.3 cm x 20.9 cm x 16.6 cm and on the left
measured 32.6 cm x 19.6 cm x 13.8 cm. Only three cases
of bilateral massive renal angiomyolipomatosis have
been mentioned in the literature [4-6] . In comparison
with those cases, our case happens to be the second
biggest.
Most angiomyolipomas can be managed
conservatively, particularly if they are asymptomatic.
Nephron conservation is of great importance in
tuberous sclerosis, as the tumors are often bilateral.
Partial nephrectomy is ideal for masses with a diameter
smaller than 3 cm, and partial nephrectomy may be
possible in masses with a diameter smaller than 5 cm
that do not reach the hilum. Lesions greater than 3.5
- 4 cm size are at great risk of serious spontaneous
hemorrhage. Renal arterial embolization can be used to
control the hemorrhage. When the lesions are greater
than 10 cm, preferred treatment is partial nephrectomy
or selective arterial embolisation [12,15] . Our patient was
posted for selective arterial embolization and if needed
nephrectomy. Unfortunately the patient had cardiac
arrest most likely due to hypovolemic shock as a result
of continuing hemorrhage from the angiomyolipoma
and died in spite of attempted resuscitation. Of the three
cases of bilateral massive renal angiomyolipamatosis
mentioned in literature, two patients presented with
hemorrhage; one of these patient who had nephrectomy
survived [4] , while the other patient who did not
undergo surgery died due to massive hemorrhage [5] .
In general, individuals with mild form of disease live
well with good life expectancy, while those with severe
form of disease have serious disabilities. However,
with appropriate management most individuals have
normal life expectancy [17] . Lesions large than 3.5 - 4 cm
are associated with catastrophic hemorrhage leading
to death unless appropriately treated [12, 5] . The leading
causes of death include renal failure, brain tumor,
status epilepticus, bronchopneumonia and lymhangiomyomatosis
[18] .
CONCLUSION
Renal angiomyolipomas associated with
tuberous sclerosis are distinctly different from those
without tuberous sclerosis. Patients with renal
angiomyolipomas associated tuberous sclerosis
present at a younger age, are, more likely to be
symptomatic, have large bilateral tumors that are more
likely to grow and therefore more frequently require
surgery. Bilateral massive angiomyolipomatosis is a
very rare entity, with only three case reports in medical
literature; all the three being associated with tuberous
sclerosis. Angiomyolipomas can have varying clinical
presentations including life threatening massive
hemorrhage. Poor communication as a result of mental
retardation in these patients can delay the diagnosis,
resulting in increased morbidity and mortality. Thus a
high index of suspicion is needed in mentally retarded
patients with poor communication skills, especially for
prompt intervention for spontaneous hemorrhage.
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