Renal angiomyolipomas (AML) are benign neoplasms, arising
from perivascular epithelioid cells. They are very rare
tumors and are strongly associated with tuberous sclerosis.
Their incidence in the general population is very low, approximately
0.3-2.4%, and were described as first time in 1880 by
Bourneville and Brissard. Most patients with renal AML are
asymptomatic. The dreaded complication is spontaneous
retroperitoneal hemorrhage. Due to that, prophylactic surgery
is recommended to prevent hemorrhage in patients with
tumor size larger than 8 cm in diameter. Here we describe
a young woman who presented with severe abdominal
pain and was found to have underlying renal angiomyolipoma
complicated with active hemorrhage.
A 21-year-old woman presented with sudden onset right
flank and right lower quadrant abdominal pain that began
when she woke up that morning. She described the pain as
sharp, knife like, non-radiating, 7/10 in intensity, aggravated
by minimal movement and relieved by lying still, associated
with nausea and 1 episode of non-bloody vomiting. She also
reported an episode of syncope earlier that day, when she
lost consciousness for few seconds. She denied hitting her head, seizure like activity or post syncopal confusion. Patient
denied abdominal injury, recent illness, foreign travel, fever,
chills, change in bowel or bladder habits and vaginal discharge.
She had no significant past medical or surgical history.
She was not taking any medications. She was never married
or pregnant. Her menstrual cycles were irregular. Last menstrual
period was 20 days prior to admission. She reported
being sexually active and used condoms for protection. She
denied any significant family history.
On physical examination, patient was afebrile and vitally stable.
She had severe tenderness to palpation in the right upper
and lower abdomen and right flank. Normoactive bowel
sounds were present. Attending to blood tests, complete
blood count and comprehensive metabolic panel were within
normal limits except for a normochromic normocytic anemia
with hemoglobin of 11.6 g/dl and minimal leukocytosis (WBC
11.1 u/kL). Urine analysis and urine pregnancy test were negative.
Chest x ray was unremarkable. CT scan of abdomen and
pelvis with contrast (Figure I) was obtained, which revealed a
17.5 x 13.8 x 13.1 cm retroperitoneal mass arising from the
right kidney.
The mass was composed of fatty and soft tissue components
along with blood products. A differential diagnosis of ruptured
angiomyolipoma and malignant tumors such as liposarcoma
were considered. Considering patient’s young age, acuity of
symptom onset and the fact that the mass arised from the
kidney, the diagnosis of renal angiomyolipoma was determined
to be more likely. The patient had no stigmata of tuberous
sclerosis on physical examination. Initially, we decided
to proceed with conservative management but within a time
span of 12 hours, patient’s hemoglobin decreased to 9.5 g/dl.
Interventional radiologist was consulted for the management
of ongoing bleeding. Emergency arteriogram with super selective
embolization of AML with alcohol and coil was performed
through the lower pole of right renal artery. A CT guided biopsy
of right renal lesion was also performed. Histological examination
confirmed our diagnosis of renal angiomyolipoma.
The patient was monitored in Intensive care unit for few more
days and was subsequently discharged as she improved. She
is planned to undergo elective resection of angiomyolipoma
at a future date.
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