PAIN IN THE SIDEResident(s): Paul Haste, MD

Attending(s): Dan Wertman, MD

Program/Dept(s): Indiana University School of Medicine

CHIEF COMPLAINT & HPI

Chief Complaint

Hypotension

History of Present Illness

55 year old woman presenting with hypotension and anemia. She reports recent seat belt injury with left flank pain which has persisted for the past week

RELEVANT HISTORY

Past Medical History Bilateral renal angiomyolipomas requiring prior transfusions and right sided

embolizations Glaucoma Depression

Past Surgical History Multiple right renal embolizations

Medications Citalopram

Allergies NKDA

DIAGNOSTIC WORKUP – NON INVASIVE IMAGING

Axial and coronal images from CT abdomen demonstrate a large, hemorrhagic left renal angiomyolipoma (yellow arrows).

An angiomyolipoma is also evident in the right kidney, with evidence of prior embolizations (white arrows).

DIAGNOSIS

Retroperitoneal bleed secondary to left renal angiomyolipoma hemorrhage.

QUESTION

At what size should resection and/or embolization of an angiomyolipoma be considered due to the increased risk of hemorrhage? (click on one of the following answers)

A. 3 cmB. 4 cmC. 5 cmD. 6 cmE. 7 cm

CORRECT!

At what size should resection and/or embolization of an angiomyolipoma be considered due to the increasing risk of hemorrhage? (click on one of the following answers)

A. 3 cmB. 4 cmC. 5 cmD. 6 cmE. 7 cm

CONTINUE WITH CASE

SORRY, THAT’S INCORRECT.

At what size should resection and/or embolization of an angiomyolipoma be considered due to the increasing risk of hemorrhage? (click on one of the following answers)

A. 3 cmB. 4 cmC. 5 cmD. 6 cmE. 7 cm

CONTINUE WITH CASE

INTERVENTION - EMBOLIZATION

Left renal arteriogram demonstrates multiple large, hypervascular tumors (arrows)

INTERVENTION - EMBOLIZATION

Figure A: Upper pole arteriogram prior to embolizationFigure B: Following upper pole embolization. The arrow points to an embolization coil in an upper pole renal artery.

A B

INTERVENTION - EMBOLIZATION

Left lower pole renal arteriogram, following embolization of upper pole renal artery with particles and coils.

The lower pole renal artery was not embolized as it supplied the only functioning portion of the kidney. More than 80% of tumor was devascularized after embolization.

QUESTION

What syndrome is classically associated with bilateral angiomyolipomas?

A. Von-Hippel LindauB. McCune-AlbrightC. Osler-Rendu-WeberD. Klippel-TrenaunayE. Tuberous sclerosis complex

CORRECT!

What syndrome is classically associated with bilateral angiomyolipomas?

A. Von-Hippel LindauB. McCune-AlbrightC. Osler-Rendu-WeberD. Klippel-TrenaunayE. Tuberous sclerosis complex

CONTINUE WITH CASE

SORRY, THAT’S INCORRECT.

What syndrome is classically associated with bilateral angiomyolipomas?

A. Von-Hippel LindauB. McCune-AlbrightC. Osler-Rendu-WeberD. Klippel-TrenaunayE. Tuberous sclerosis complex

CONTINUE WITH CASE

SUMMARY & TEACHING POINTS

• 55 y/o woman presenting with hypotension from a hemorrhaging left angiomyolipoma who underwent particle/coil embolization.

• Post embolization arteriography showed devascularization of >80% of the tumors with sparing of the functional left lower pole kidney.

• Patient was discharged with outpatient follow-up scheduled.

• On CT or MR, the characteristic imaging finding of angiomyolipoma (AML) is a mass that contains macroscopic fat . It is usually well-marginated and is comprised predominantly of fat density (-30 to -100 HU). A renal mass with fat density is nearly diagnostic of an AML. Roughly 5% of AMLs will not have fat and therefore cannot be distinguished by imaging. Calcification is almost never present in an AML, and if seen, renal cell carcinoma should be considered.

• Bilateral angiomyolipomas are associated with tuberous sclerosis complex.

• Resection or embolization of angiomyolipomas 4cm or greater should be considered, due to an increased risk of hemorrhage.