Hepatic angiomyolipoma (AML) is a rare stromal tumor composed of variable admixtures of thick-walled vessels, smooth muscles and adipose tissue. One of the specific radiological findings of hepatic AML is an early drainage vein noted via enhanced computed tomography (CT). We report a case of hepatic AML showing early drainage veins into both the hepatic and portal vein. The case involved a 46-year-old woman who was referred to our hospital because of a giant hepatic tumor. CT revealed well-enhanced 14 cm and 1 cm tumors in the left and right lobes, respectively. Magnetic resonance imaging demonstrated the existence of adipose tissues in the larger tumor. Hepatic arteriography revealed early drainage veins draining into both the hepatic and portal vein. Based on a diagnosis of hepatic AML, left hepatectomy and partial hepatectomy were performed. Pathology revealed both tumors as hepatic AML based on human melanoma black-45 immuno-positivity. Hepatic AML with early drainage veins into both the hepatic and portal vein is rare. The dilated and retrogressive vein drains the abundant arterial blood flow of the tumor. The finding of early drainage veins into not only the hepatic vein but also the portal vein should be helpful for diagnosing hepatic AMLs.
Hepatic angiomyolipoma Early drainage vein Hepatic vein Portal vein
This is a preview of subscription content, log in to check access.
Compliance with ethical standards
Conflict of interest
Ryota Kiuchi, Takanori Sakaguchi, Ryo Kitajima, Satoru Furuhashi, Makoto Takeda, Takanori Hiraide, Yoshifumi Morita, Takasuke Ushio, Rei Ishikawa, Satoshi Baba and Hiroya Takeuchi declare that we they have no conflict of interest.
All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.
Informed consent was obtained from all patients for being included in the study.
Ishak K. Mesenchymal tumors of the liver. In: Okuda K, Peters RL, editors. Hepatocellular carcinoma. New York: Wiley; 1976. pp. 228–304.Google Scholar
Zheng RQ, Kudo M. Hepatic angiomyolipoma: identification of an efferent vessel to be hepatic vein by contrast-enhanced harmonic ultrasound. Br J Radiol. 2005;78:956–60.CrossRefGoogle Scholar
Jeon TY, Kim SH, Lim HK, et al. Assessment of triple-phase CT findings for the differentiation of fat-deficient hepatic angiomyolipoma from hepatocellular carcinoma in non-cirrhotic liver. Eur J Radiol. 2010;73:601–6.CrossRefGoogle Scholar
Yoshioka M, Watanabe G, Uchinami H, et al. Hepatic angiomyolipoma: differential diagnosis from other liver tumors in a special reference to vascular imaging - importance of early drainage vein. Surg Case Rep. 2015;1:11.CrossRefPubMedCentralGoogle Scholar
Saito Y, Shimada M, Utsunomiya T, et al. Hepatic epithelioid angiomyolipoma with arterioportal venous shunting mimicking hepatocellular carcinoma: report of a case. J Med Investig. 2013;60:262–6.CrossRefGoogle Scholar
Tsui WM, Colombari R, Portmann BC, et al. Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants. Am J Surg Pathol. 1999;23:34–48.CrossRefGoogle Scholar
Martignoni G, Pea M, Reghellin D, et al. PEComas: the past, the present and the future. Virchows Arch. 2008;452:119–32.CrossRefGoogle Scholar
Weeks DA, Malott RL, Arnesen M, et al. Hepatic angiomyolipoma with striated granules and positivity with melanoma–specific antibody (HMB-45): a report of two cases. Ultrastruct Pathol. 1991;15:563–71.CrossRefGoogle Scholar
Makhlouf HR, Ishak KG, Shekar R, et al. Melanoma markers in angiomyolipoma of the liver and kidney: a comparative study. Arch Pathol Lab Med. 2002;126:49–55.Google Scholar
Kudo M, Okuno T, Tomita S, et al. Hepatic angiomyolipoma pre-operatively diagnosed by imaging. J Gastroenterol Hepatol. 1993;8:483–8.CrossRefGoogle Scholar
Ahmadi T, Itai Y, Takahashi M, et al. Angiomyolipoma of the liver: significance of CT and MR dynamic study. Abdom Imaging. 1998;23:520–6.CrossRefGoogle Scholar
Yan F, Zeng M, Zhou K, et al. Hepatic angiomyolipoma: various appearances on two-phase contrast scanning of spiral CT. Eur J Radiol. 2002;41:12–8.CrossRefGoogle Scholar
Yoshimura H, Murakami T, Kim T, et al. Angiomyolipoma of the liver with least amount of fat component: imaging features of CT, MR, and angiography. Abdom Imaging. 2002;27:184–7.CrossRefGoogle Scholar
Wang S, Kuai X, Meng X, et al. Comparison of MRI features for the differentiation of hepatic angiomyolipoma from fat-containing hepatocellular carcinoma. Abdom Imaging. 2014;39:323–33.CrossRefGoogle Scholar
Iwao Y, Ojima H, Onaya H, et al. Early venous return in hepatic angiomyolipoma due to an intratumoral structure resembling an arteriovenous fistula. Hepatol Res. 2014;44:700–6.CrossRefGoogle Scholar
Murakami T, Nakamura H, Hori S, et al. Angiomyolipoma of the liver. Ultrasound, CT, MR imaging and angiography. Acta Radiol. 1993;34:392–4.CrossRefGoogle Scholar
Dalle I, Sciot R, de Vos R, et al. Malignant angiomyolipoma of the liver: a hitherto unreported variant. Histopathology. 2000;36:443–50.CrossRefGoogle Scholar
Mizuguchi T, Katsuramaki T, Nobuoka T, et al. Growth of hepatic angiomyolipoma indicating malignant potential. J Gastroenterol Hepatol. 2004;19:1328–30.CrossRefGoogle Scholar
Deng YF, Lin Q, Zhang SH, et al. Malignant angiomyolipoma in the liver: a case report with pathological and molecular analysis. Pathol Res Pract. 2008;204:911–8.CrossRefGoogle Scholar
Nguyen TT, Gorman B, Shields D, et al. Malignant hepatic angiomyolipoma: report of a case and review of literature. Am J Surg Pathol. 2008;32:793–8.CrossRefGoogle Scholar
Kamimura K, Oosaki A, Sugahara S, et al. Malignant potential of hepatic angiomyolipoma: case report and literature review. Clin J Gastroenterol. 2010;3:104–10.CrossRefGoogle Scholar
Fukuda Y, Omiya H, Takami K, et al. Malignant hepatic epithelioid angiomyolipoma with recurrence in the lung 7 years after hepatectomy: a case report and literature review. Surg Case Rep. 2016;2:31.CrossRefPubMedCentralGoogle Scholar
Makhlouf HR, Remotti HE, Ishak KG. Expression of KIT (CD117) in angiomyolipoma. Am J Surg Pathol. 2002;26:493–7.CrossRefGoogle Scholar
Parfitt JR, Bella AJ, Izawa JI, et al. Malignant neoplasm of perivascular epithelioid cells of the liver. Arch Pathol Lab Med. 2006;130:1219–22.Google Scholar
Folpe AL, Mentzel T, Lehr H-AA, et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29:1558–75.CrossRefGoogle Scholar