J Liver Cancer.  2021 Mar;21(1):76-80. 10.17998/jlc.21.1.76.

Huge Hepatic Angiomyolipoma Mimicking Low Grade Hepatocellular Carcinoma

• Hwang HS
• Han DH

• KMID: 2514237
• DOI: http://doi.org/10.17998/jlc.21.1.76

Abstract

A 41-year-old man was diagnosed with a huge symptomatic liver mass and was referred to our hospital for liver biopsy and further evaluation. He presented with right upper quadrant tenderness. Enhanced abdominal computed tomography and magnetic resonance imaging revealed a 12.5-cm relatively well-defined heterogeneous enhancing mass in the right inferior liver with a large exophytic component containing a fat component and progressive delayed enhancement. The patient underwent right inferior sectionectomy. The pathological diagnosis was confirmed as angiomyolipoma, 12.3×9.2×5.0 cm in size, with tumor necrosis in 20% of the tissue. Hepatic angiomyolipoma is known as a benign tumor, but in our case, because of the large tumor size and coagulative necrosis, this tumor had malignant potential; surgical resection was deemed to be appropriate, and close follow-up monitoring was essential postoperatively.

Keyword

Hepatic angiomyolipoma; Hepatocellular carcinoma

Figure

• 

  Figure 1 Abdominal computed tomography revealed a 12.5-cm sized relatively well-defined heterogeneous enhancing mass in the right inferior liver with a large exophytic component containing a fat component (A, B).

• 

  Figure 2 On magnetic resonance imaging, the hepatic mass showed progressive delayed enhancement; the last two arterial phase images revealed the presence of early draining veins around the tumor (arrows), which is a typical imaging finding of angiomyolipoma (A–H). AP, arterial phase; PP, portal phase; PDFF, proton density fat fraction.

• 

  Figure 3 (A) Gross image of specimen with dilated veins (arrowheads) on liver surface. (B) Resected specimen with heterogeneous fatty and hemorrhagic components (arrowheads).

• 

  Figure 4 Histopathological findings of angiomyolipoma; (A) thick-walled vessels (white arrow, portal vein) and thin-walled vessels (black arrow) are fused at the boundary between normal liver tissue (N) and the tumor (T) (×40, hematoxylin and eosin [H&E] stain). (B–D) The pleomorphism of histological features of hepatic angiomyolipoma is showed (×100, H&E stain) (B, adipose tissues; C, foam cells; and D, smooth muscle cells).

Reference

1. Wang CP, Li HY, Wang H, Guo XD, Liu CC, Liu SH, et al. Hepatic angiomyolipoma mimicking hepatocellular carcinoma: magnetic resonance imaging and clinical pathological characteristics in 9 cases. Medicine (Baltimore). 2014; 93:e194.

2. Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, et al. Management of hepatic angiomyolipoma: a systematic review. Liver Int. 2017; 37:1272–1280.

3. Nguyen TT, Gorman B, Shields D, Goodman Z. Malignant hepatic angiomyolipoma: report of a case and review of literature. Am J Surg Pathol. 2008; 32:793–798.

4. Sato K, Ueda Y, Tachibana H, Miyazawa K, Chikazawa I, Kaji S, et al. Malignant epithelioid angiomyolipoma of the kidney in a patient with tuberous sclerosis: an autopsy case report with p53 gene mutation analysis. Pathol Res Pract. 2008; 204:771–777.

5. Ishak KG. Mesenchymal tumor of the liver. Okuda K, Peters RL, editors. Hepatocellular carcinoma. New York: Wiley Medical;1976. p. 247–304.

6. Chang JC, Lee YW, Kim HJ. Preoperative diagnosis of angiomyolipoma of the liver. Abdom Imaging. 1994; 19:546–548.

7. Blumgart R, Payne M, Rhodes A. Angiomyolipoma of the liver. Clin Radiol. 1987; 38:329–330.

8. Zhong DR, Ji XL. Hepatic angiomyolipoma-misdiagnosis as hepatocellular carcinoma: a report of 14 cases. World J Gastroenterol. 2000; 6:608–612.

9. Kiuchi R, Sakaguchi T, Kitajima R, Furuhashi S, Takeda M, Hiraide T, et al. Hepatic angiomyolipoma with early drainage veins into the hepatic and portal vein. Clin J Gastroenterol. 2019; 12:361–366.

10. Saito Y, Shimada M, Utsunomiya T, Morine Y, Imura S, Ikemoto T, et al. Hepatic epithelioid angiomyolipoma with arterioportal venous shunting mimicking hepatocellular carcinoma: report of a case. J Med Invest. 2013; 60:262–266.

11. Nonomura A, Mizukami Y, Kadoya M, Matsui O, Shimizu K, Izumi R. Angiomyolipoma of the liver: its clinical and pathological diversity. J Hepatobiliary Pancreat Sur. 1996; 3:122–132.

12. Nonomura A, Mizukami Y, Kadoya M. Angiomyolipoma of the liver: a collective review. J Gastroenterol. 1994; 29:95–105.

13. Yang CY, Ho MC, Jeng YM, Hu RH, Wu YM, Lee PH. Management of hepatic angiomyolipoma. J Gastrointest Surg. 2007; 11:452–457.