Hepatic angiomyolipoma: mutation analysis and immunohistochemical pitfalls in diagnosis

Zhang, Yan; Grenert, James P.; Joseph, Nancy M.; Ren, Chuanli; Chen, Xin; Shafizadeh, Nafis; Kakar, Sanjay

doi:10.1111/his.13509

Cited by 18 publications

(16 citation statements)

References 41 publications

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“…Due to the challenge in diagnosis of hepatic angiomyolipoma with non-specific clinical characteristics and imaging features, so that histological examination and immunohistochemistry staining are considered as the gold standard for HAML diagnosis [15] . In our case, severe anemia was occurred before the patient's admission due to the giant size of the tumor and not related to percutaneous biopsy.…”

Section: Discussionmentioning

confidence: 99%

Giant hepatic angiomyolipoma presenting with severe anemia: A surgical case report and review of literature

Nguyen

Luong

et al. 2022

International Journal of Surgery Case Reports

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Section: Discussionmentioning

confidence: 99%

Giant hepatic angiomyolipoma presenting with severe anemia: A surgical case report and review of literature

Nguyen

Luong

et al. 2022

International Journal of Surgery Case Reports

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“…Some studies suggest that epithelioid type HAML may mimic HCC or hepatocellular adenoma on pathological specimens. 11 Epithelioid type HAML are a type of HAML composed almost solely of epithelioid cells, blood vessels and few adipocytes. 12 These epithelioid type HAML are thought to have the most malignant course of behavior with recurrence or metastasis.…”

Section: Discussionmentioning

confidence: 99%

Hepatic angiomyolipoma: an international multicenter analysis on diagnosis, management and outcome

Klompenhouwer

Dwarkasing

Doukas

et al. 2020

HPB

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Background: Hepatic angiomyolipoma (HAML) may easily be misdiagnosed as a malignancy. The study aim was to assess diagnostic dilemmas, clinical management and outcome of this rare tumor.Methods: This retrospective international multicenter study included all patients with pathologically proven HAML diagnosed between 1997 and 2017. Data on patient characteristics, diagnostic work-up, management and follow-up were analyzed.Results: Thirty-eight patients were included, 32 female. Median age was 56yrs (i.q.r. 43-64) and median HAML-diameter was 57.5 mm (i.q.r. 38.5-95.3). Thirty patients had undergone CT and 27/38 MRI of the liver, diagnostic biopsy was performed in 19/38. Initial diagnosis was incorrect in 15/38 patients, of which 13 were thought to have malignancy. In 84% biopsy resulted in a correct preoperative diagnosis. Twenty-nine patients were managed with surgical resection, 4/38 with surveillance and 3/38 with liver transplantation. Recurrence after resection occurred in two cases. No HAML related deaths or progression to malignancy were documented. Conclusion:HAML diagnosis proved problematic even in hepatobiliary expertise centers. Biopsy is indicated and may provide valuable additional information when HAML diagnosis is considered on cross-sectional imaging, especially when surgical resection imposes a risk of complications. Conservative management with regular imaging follow-up might be justified when biopsy confirms (classic type) HAML.

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“…Consequently, these authors developed a provisional classification of PEComas with increasing aggressive potential (Table 2 ). Cases of HAML with aggressive behavior are reported in Table 3 [ 8 - 24 ]. Regarding HAML, it is mainly the epithelioid type that confers a risk of aggressive behavior[ 34 ].…”

Section: Prognosismentioning

confidence: 99%

“…Hepatic AML (HAML) poses a veritable diagnostic challenge in radiological terms, especially when fat content is low, because this type of tumor may appear as a hypervascular tumor associated with a washout phase that mimics other, more common hypervascular hepatic tumors, such as hepatocellular carcinoma[ 4 - 7 ]. The natural course of HAML is mostly benign, although several cases have been reported to exhibit aggressive behavior with metastasis or recurrence after surgery[ 8 - 24 ], or spontaneous rupture[ 8 , 25 - 33 ]. These rare but dramatic observations unavoidably compound the complexity of managing patients with HAML.…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: A literature review

Calame¹,

Tyrode²,

Weil³

et al. 2021

WJG

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First reported in 1976, hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumor occurring mostly in middle-aged women. Diagnosis of the liver mass is often incidental on abdominal imaging due to the frequent absence of specific symptoms. Nearly 10% of HAMLs are associated with tuberous sclerosis complex. HAML contains variable proportions of blood vessels, smooth muscle cells and adipose tissue, which renders radiological diagnosis hazardous. Cells express positivity for HMB-45 and actin, thus these tumors are integrated into the group of perivascular epithelioid cell tumors. Typically, a HAML appears on magnetic resonance imaging (or computed tomography scan) as a hypervascular solid tumor with fatty areas and with washout, and can easily be misdiagnosed as other liver tumors, particularly hepatocellular carcinoma. The therapeutic strategy is not clearly defined, but surgical resection is indicated for symptomatic patients, for tumors showing an aggressive pattern ( i.e. , changes in size on imaging or high proliferation activity and atypical epithelioid pattern on liver biopsy), for large (> 5 cm) biopsy-proven HAML, and if doubts remain on imaging or histology. Conservative management may be justified in other conditions, since most cases follow a benign clinical course. In summary, the correct diagnosis of HAML is challenging on imaging and relies mainly on pathological findings.

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Hepatic angiomyolipoma: mutation analysis and immunohistochemical pitfalls in diagnosis

Cited by 18 publications

References 41 publications

Giant hepatic angiomyolipoma presenting with severe anemia: A surgical case report and review of literature

Giant hepatic angiomyolipoma presenting with severe anemia: A surgical case report and review of literature

Hepatic angiomyolipoma: an international multicenter analysis on diagnosis, management and outcome

Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: A literature review

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