Introduction: Hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours (PEComas). It is typically composed of blood vessels, smooth muscle, and adipose cells, and shows strong immunoreactivity for HMB-45.
Presentation Of The Case: A 57-year-old woman was referred to our hospital with an unclear liver lesion. A fine needle biopsy revealed a suspicion of hepatic angiomyolipoma with extramedullary haematopoiesis. Preoperative imaging revealed a tumour 17 cm in diameter in the left liver lobe segments II and III. A lobectomy of the left lobe segments II and III was performed. The pathological diagnosis of hepatic angiomyolipoma was obtained.
Discussion: Variations in the predominance of the tissue components in HAML impedes diagnosis based on imaging alone. The most promising evidence of HAML is the histological identification of lipomatous, myomatous, and angiomatous tissue combined with immunohistochemical positivity for HMB-45. Although the tumour is considered benign, some cases have been described with malignant behaviour. Surgical resection should be considered in case of symptoms, inconclusive biopsy, or growth in follow-up. Other surgical indications may include aggressive patterns such as vascular invasion, p53 immunoreactivity, or rapidly proliferating tumour cells.
Conclusion: HAML is a rare liver tumour. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed according to oncological criteria.
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http://dx.doi.org/10.1016/j.ijscr.2020.11.045 | DOI Listing |
Clin J Gastroenterol
January 2025
Department of Gastroenterology and Hepatology, Osaka University Graduate School of Medicine, Suita, Japan.
A 55-year-old man with tuberous sclerosis complex (TSC) was diagnosed with left renal angiomyolipoma (AML), a group of perivascular epithelioid cell tumors called PEComas. He had received the mTOR inhibitor everolimus, which resulted in a complete response. However, a left renal mass relapsed in two years, followed by the occurrence of a hepatic mass five months later.
View Article and Find Full Text PDFHistopathology
December 2024
Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Aims: The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors.
Methods And Results: In total, 132 patients were included.
Sci Rep
December 2024
Department of Radiology, Seoul National University Hospital, 101 Daehangno, Jongno-gu, Seoul, 03080, Korea.
Ultrasound (US) is a widely used technique for liver disease but has limitations in distinguishing tumors. This study evaluates the clinical efficacy of fluctuational imaging (FLI), a new US method that detects the fluttering sign in liver tumors. We conducted a prospective exploratory study with 120 participants diagnosed with liver tumors through histopathology or standard imaging.
View Article and Find Full Text PDFJ Investig Med High Impact Case Rep
December 2024
Gastroenterology Department, Mohamed Taher Maamouri University Hospital, Nabeul, Tunisia.
Hepatic angiomyolipoma (HAML) is an uncommon primary liver tumor with limited reported cases in the literature. It is composed of varying amounts of fat, smooth muscle, and blood vessels, typically non-malignant proliferation. It usually presents diagnostic challenges due to diverse imaging characteristics.
View Article and Find Full Text PDFJ Ultrasound
November 2024
Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy.
Perivascular epithelioid cell neoplasms (PEComas) and epithelioid angiomyolipomas (EAMLs) are two different denominations for the same "mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells". Hepatic PEComa/EAML is a very rare neoplasm, and only 29 case reports of hepatic PEComa and 25 of hepatic EAML have been reported in the current literature. A clear female predominance with a mean age at diagnosis of 42.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!