Case Report # 1 Submitted by:Keith Pettibon Faculty reviewer:Sandra Oldham, MD Date accepted:24 August 2010 Radiological Category:Principal Modality (1): Principal Modality (2): Fluoroscopy CTVascular Radiology

Case History Ms. W is a 63 year old white female who presented to her outside physician with symptoms of pneumonia and fluid around her lungs. As part of her evaluation, she had a CT scan of the abdomen and pelvis.

Radiological Presentations

Case History Ms. W denied any symptoms related to the mass. She denied any pain, hematuria, or dysuria.

Percutaneous Biopsy MRI Sonogram Lab work Which one of the following is your choice for the appropriate diagnosis? Test Your Diagnosis

Angiomyolipoma Renal Cell Carcinoma Liposarcoma Wilms Tumor Lipoma Findings: Differential: Findings and Differentials

Angiomyolipoma Renal Cell Carcinoma – usually does not contain fat Liposarcoma Wilms Tumor Lipoma Findings: Differential: Findings and Differentials

Angiomyolipoma Renal Cell Carcinoma – usually does not contain fat Liposarcoma – no defect in renal parenchyma Wilms Tumor Lipoma Findings: Differential: Findings and Differentials

Angiomyolipoma Renal Cell Carcinoma – usually does not contain fat Liposarcoma – no defect in renal parenchyma Wilms Tumor – very uncommon in adults Lipoma Findings: Differential: Findings and Differentials

Percutaneous biopsy performed was consistent with angiomyolipoma. Angiomyolipoma Renal Cell Carcinoma Liposarcoma Wilms Tumor Lipoma Findings: Differential: Findings and Differentials

The CT shows a 7 cm. heterogenous, fat containing mass. The presence of fat in a renal mass is usually considered diagnostic for angiomyolipoma (although renal lipoma, liposarcoma, and renal cell carcinoma engulfing perinephric fat are other rare possibilities). Discussion

The CT shows a 7 cm. heterogenous, fat containing mass. The presence of fat in a renal mass is usually considered diagnostic for angiomyolipoma (although renal lipoma, liposarcoma, and renal cell carcinoma engulfing perinephric fat are other rare possibilities). 95% of angiomyolipomas contain enough fat to be detected by CT scan. Discussion

The CT shows a 7 cm. heterogenous, fat containing mass. The presence of fat in a renal mass is usually considered diagnostic for angiomyolipoma (although renal lipoma, liposarcoma, and renal cell carcinoma engulfing perinephric fat are other rare possibilities). 95% of angiomyolipomas contain enough fat to be detected by CT scan. Angiomyolipomas are benign hamartomas composed of vascular components, smooth muscle components and fat. Discussion

The CT shows a 7 cm. heterogenous, fat containing mass. The presence of fat in a renal mass is usually considered diagnostic for angiomyolipoma (although renal lipoma, liposarcoma, and renal cell carcinoma engulfing perinephric fat are other rare possibilities). 95% of angiomyolipomas contain enough fat to be detected by CT scan. Angiomyolipomas are benign hamartomas composed of vascular components, smooth muscle components and fat. Smaller angiomyolipomas are often asymptomatic and are usually very slow growing. Discussion

Angiomyolipomas become at risk for spontaneous hemorrhage at 4 cm. Discussion

Angiomyolipomas become at risk for spontaneous hemorrhage at 4 cm. 80% develop spontaneously, however 20% are associated with tuberous sclerosis. Discussion

When associated with tuberous sclerosis, patient often has multiple angiomyolipomas along with renal cystic disease. 80% of people with tuberous sclerosis develop angiomyolipomas. They are grow more rapidly than the sporadic form. Discussion

When associated with tuberous sclerosis, patient often has multiple angiomyolipomas along with renal cystic disease. 80% of people with tuberous sclerosis develop angiomyolipomas. They are grow more rapidly than the sporadic form. Other findings in tuberous sclerosis include: seizures, mental retardation, ash leaf spots on skin, cortical and retinal hamartomas, cardiac rhabdomyomas. Discussion

Treatment Options include nephrectomy, active surveillance and selective embolization. Discussion

Treatment Options include nephrectomy, active surveillance and selective embolization. Ms. W was referred to the interventional radiology department for selective embolization. Discussion

Radiological Presentations

From study published in Journal of Vascular and Interventional Radiology, patients underwent selective embolization for 30 AMLs. 10 of these patients had TS. The other 9 were sporadic. Recurrence (defined as increase of tumor size by 2 cm or repeated symptoms over the following 6 months-11 years) occurred in 6 of the patients with TS but there was no recurrence in the sporadic patients. Conclusion: Embolization is an effective treatment option for angiomyolipomas however lifelong surveillance is important, especially in patients with TS. Discussion

Zagoria, Ronald J., Ronald J. Zagoria, Julia R. Fielding, and Wiliams W. Mayo-Smith. Genitourinary Imaging: Case Review. Philadelphia: Mosby, Print. Kothary, Nishita. “Renal Angiomyolipoma: Long-tern Results after Arterial Embolization.” Journal of Vascular and Interventional Radiology 16.1 (2005): Israel, Gary. “CT Differentiation of Large Exophytic Renal Angiomyolipomas and Perirenal Liposarcomas.” American Journal of Radiology 179 (2002): References