Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2023 Oct 14;2023(10):rjad556.
doi: 10.1093/jscr/rjad556. eCollection 2023 Oct.

Hepatic angiomyolipoma, misdiagnosed as hepatocellular carcinoma

Bárbara M Marinho  1 , António G Canha  2 , Donzília S Silva  2 , Ana P Rodrigues  3
Affiliations
Case Reports

Hepatic angiomyolipoma, misdiagnosed as hepatocellular carcinoma

Bárbara M Marinho et al. J Surg Case Rep. .

Abstract

Perivascular epithelioid cell neoplasm (PEComa) is a rare type of tumor, and hepatic PEComa is even rarer. Its preoperative diagnosis is difficult, given the absence of specific clinical manifestations, often constituting an accidental finding, and the lack of a gold standard for identification using imaging studies. Instead, the diagnosis of hepatic PEComa is based on morphological and immunohistochemical features. We describe a case of an asymptomatic hepatic PEComa, angiomyolipoma type, which appeared in a middle-aged woman with chronic liver disease, during her follow-up and screening. Given the patient's context, human immunodeficiency virus-positive with chronic hepatitis C, and the similarities between the two tumors, the hepatic lesion was interpreted as hepatocellular carcinoma. The patient underwent surgical excision of the tumor, and the positive immunohistochemical staining for human melanoma black 45 and Melan A made the definitive diagnosis. In the absence of aggressiveness tumor markers, surveillance was decided. We also provide a literature review of these tumors.

PubMed Disclaimer

Conflict of interest statement

None declared.

Figures

Figure 1
Figure 1
Ultrasound showing a hypoechoic nodule, vaguely heterogeneous, located in segment VII, subcapsular, and with ~22 mm in diameter.
Figure 2
Figure 2
CT scan showing, in addition to signs of chronic liver disease, the presence of a solid subcapsular nodule of segment VII, measuring 23 mm, with hypervascular behavior in the arterial phase.
Figure 3
Figure 3
CT scan showing the same mass as shown in Fig. 2 but with washout in the portal venous phase.
Figure 4
Figure 4
Liver parenchyma partially occupied by a neoplastic proliferation with expansive borders.
Figure 5
Figure 5
The neoplastic cells are organized in small bundles. The cells are spindled to oval, with eosinophilic and granular cytoplasm and with mild nuclear atypia.
Figure 6
Figure 6
There is heterogeneous positivity for smooth muscle markers.
Figure 7
Figure 7
The neoplastic cells are also positive for HMB45, demonstrating the typical myo-melanocytic differentiation.
See this image and copyright information in PMC

References

    1. Ding GH, Liu Y, Wu MC, Yang GS, Yang JM, Cong WM. Diagnosis and treatment of hepatic angiomyolipoma. J Surg Oncol 2011;103:807–12. - PubMed
    1. Klompenhouwer AJ, Dwarkasing RS, Doukas M, Pellegrino S, Vilgrain V, Paradis V. et al. Hepatic angiomyolipoma: an international multicenter analysis on diagnosis, management and outcome. Hpb [Internet] 2020;22:622–9. - PubMed
    1. Attard A, Piscopo N, Schembri J, Buhagiar T, Cortis K, Ellul P. A rare case of PEComa of the liver. GE Port J Gastroenterol 2021;28:217–21. - PMC - PubMed
    1. Khaja F, Carilli A, Baidas S, Sriharan A, Norford S. PEComa: a perivascular epithelioid cell tumor in the liver - a case report and review of the literature. Case Rep Med 2013;2013:4–8. - PMC - PubMed
    1. Khan HM, Katz SC, Libbey NP, Somasundar PS. Hepatic PEComa: a potential pitfall in the evaluation of hepatic neoplasms. BMJ Case Rep 2014;2014:bcr2014204122–5. - PMC - PubMed

Publication types