Case Report # 1

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

2. 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.

3. Radiological Presentations

4. Radiological Presentations

5. Radiological Presentations

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

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

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

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

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

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

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

13. 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).

14. 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.

15. 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.

16. 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.

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

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

19. 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.

20. 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.

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

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

23. Radiological Presentations

24. Radiological Presentations

25. Discussion From study published in Journal of Vascular and Interventional Radiology, 2005 19 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.

26. Zagoria, Ronald J., Ronald J. Zagoria, Julia R. Fielding, and Wiliams W. Mayo-Smith.Genitourinary Imaging: Case Review. Philadelphia: Mosby, 2007. Print.Kothary, Nishita. “Renal Angiomyolipoma: Long-tern Results after Arterial Embolization.” Journal of Vascular and Interventional Radiology 16.1 (2005): 45-50. Israel, Gary. “CT Differentiation of Large Exophytic Renal Angiomyolipomas and Perirenal Liposarcomas.” American Journal of Radiology 179 (2002): 769-73. References