Objective: Based on a large series of histopathologically confirmed hepatic angiomyolipomas, we retrospectively studied the typical diagnostic features of hepatic angiomyolipoma and proposed a treatment strategy for this disease.
Materials And Methods: From December 1997 to December 2007, 74 consecutive patients who received definitive treatment for hepatic angiomyolipoma, at a single tertiary center, were studied.
Results: There was a marked female predominance (54 females vs. 20 males) and the mean age was 42 years. Forty patients had no symptoms and the tumors were detected incidentally during a medical check-up. From this study, we proposed the typical diagnostic features of hepatic angiomyolipoma to be the absence of risk factors for malignancy, normal tumor marker levels, and typical imaging features on ultrasound (USG), abdominal contrast computed tomography (CT), or magnetic resonance imaging (MRI). Only 23% of patients could have been diagnosed before surgery using these features. One patient (1.4%) had a malignant angiomyolipoma, and died with distant metastases 14 months after surgery. After a median follow-up of 64 months, there was no recurrence in the other 73 patients.
Conclusion: Patients with typical diagnostic features suggestive of hepatic angiomyolipoma could be observed with regular surveillance. Definitive treatment should be performed when the tumor has symptoms/complications, when the tumor is enlarging, or when a malignant lesion cannot be ruled out.
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http://dx.doi.org/10.1016/j.asjsur.2011.11.005 | 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!