Myomatous hepatic angiomyolipoma: imaging findings in 14 cases with radiological–pathological correlation and review of the literature

Zhu, Zhaohui; Yang, Lifen; Xi, Zhao; Dq, Luo; Zhang, Hong Tao; Cw, Zhou

doi:10.1259/bjr.20130712

Cited by 9 publications

(6 citation statements)

References 23 publications

(31 reference statements)

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“…These features are absent in patients with HCC [9]. Furthermore, tumor capsule, liver cirrhosis, bile duct dilatation and lymphadenopathy, which are usually present in HCC, are absent in HAML [9]. These distinguishing features significantly contributed in considering angiomyolipoma in our patient preoperatively.…”

Section: Discussionmentioning

confidence: 73%

“…Zhu et al compared imaging findings of 14 cases with existing literature and noted that HAML usually have an early draining vein in the arterial phase and tortuous vessels in the tumor. These features are absent in patients with HCC [9]. Furthermore, tumor capsule, liver cirrhosis, bile duct dilatation and lymphadenopathy, which are usually present in HCC, are absent in HAML [9].…”

Section: Discussionmentioning

confidence: 97%

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Hepatic angiomyolipoma presenting with chronic epigastric pain: A case report

Perez¹,

Yacapin²

2016

Int J Hepatobiliary Pancreat Dis

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 97%

Hepatic angiomyolipoma presenting with chronic epigastric pain: A case report

Perez¹,

Yacapin²

2016

Int J Hepatobiliary Pancreat Dis

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“…These PEC cells have no normal corollary and are thought to be the precursor of a group of lesions that can be found in many different organs [30]. In 2002, the classification of soft tissue tumors released by the World Health Organization; HAML was classified as perivascular epithelioid cell tumors (PEComas) [31].…”

Section: Discussionmentioning

confidence: 99%

Utility of Immunohistochemistry in the Diagnosis of a Case of Hepatic Angiomyolipoma from a Tertiary Referral Centre for Liver Diseases

NA¹,

MM²

2016

J Hepatol Gastroint Dis

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A single case of hepatic Angiomyolipoma (AML) over 25 years in a tertiary referral center for liver diseases is being presented. A 56-year old gentleman presented with abdominal pain, fullness and weight loss. A large left lobe mass in a background of non-cirrhotic liver was radiologically inscribed. The risk of rupture of together with the possibility of malignancy seedlings were the restraints against CT guided liver biopsy. Left lateral segmentectomy was the consulting surgical team's decisive maneuver. Gross pathological examination, histological evaluation and immunohistochemical analysis were performed to determine the nature of tumor. Results revealed normal serum levels of alpha-fetoprotein, CA 19.9 and carcinoembryonic antigen. Grossly the tumor exhibited variable consistency. Histologically, the tumor displayed three main constituents intermingled together; blood vessels, myoid cells and fat. Immunohistochemistry revealed positive immune reaction for CD34, alpha smooth muscle actin and S-100 in tumor tissue but negative for anti-human hepatocyte antibody.

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“…Similar to the more common kidney AML, hAML consists of variable proportions of mature-appearing adipose cells, thick-walled vessels and epithelioid or spindleshaped smooth muscle-like cells, most frequently of epithelioid appearance. 5,6 This variable composition may complicate diagnosis, leading to misdiagnoses of other benign and malignant liver tumours, including hepatocellular carcinoma (HCC) and other hypervascular liver tumours. [7][8][9][10][11][12][13] In addition, the lipomatous type of hAML can be misdiagnosed as lipoma or liposarcoma and the angiomatous type as hepatic haemangioma.…”

Section: Introductionmentioning

confidence: 99%

“…Hepatic AML show a female to male ratio of 4:1, and occur mainly in adults aged 30–65 years. Similar to the more common kidney AML, hAML consists of variable proportions of mature‐appearing adipose cells, thick‐walled vessels and epithelioid or spindle‐shaped smooth muscle‐like cells, most frequently of epithelioid appearance 5,6 . This variable composition may complicate diagnosis, leading to misdiagnoses of other benign and malignant liver tumours, including hepatocellular carcinoma (HCC) and other hypervascular liver tumours 7–13 .…”

Section: Introductionmentioning

confidence: 99%

TSC2 inactivation, low mutation burden and high macrophage infiltration characterise hepatic angiomyolipomas

Giannikou,

Klonowska,

Tsuji

et al. 2023

Histopathology

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AimsAlthough TSC1 or TSC2 inactivating mutations that lead to mTORC1 hyperactivation have been reported in hepatic angiomyolipomas (hAML), the role of other somatic genetic events that may contribute to hAML development is unknown. There are also limited data regarding the tumour microenvironment (TME) of hAML. The aim of the present study was to identify other somatic events in genomic level and changes in TME that contribute to tumorigenesis in hAML.Methods and resultsIn this study, we performed exome sequencing in nine sporadic hAML tumours and deep‐coverage targeted sequencing for TSC2 in three additional hAML. Immunohistochemistry and multiplex immunofluorescence were carried out for 15 proteins to characterise the tumour microenvironment and assess immune cell infiltration. Inactivating somatic variants in TSC2 were identified in 10 of 12 (83%) cases, with a median allele frequency of 13.6%. Five to 18 somatic variants (median number: nine, median allele frequency 21%) not in TSC1 or TSC2 were also identified, mostly of uncertain clinical significance. Copy number changes were rare, but detection was impaired by low tumour purity. Immunohistochemistry demonstrated numerous CD68+ macrophages of distinct appearance from Küpffer cells. Multiplex immunofluorescence revealed low numbers of exhausted PD‐1+/PD‐L1+, FOXP3+ and CD8+ T cells.ConclusionhAML tumours have consistent inactivating mutations in TSC2 and have a low somatic mutation rate, similar to other TSC‐associated tumours. Careful histological review, standard IHC and multiplex immunofluorescence demonstrated marked infiltration by non‐neoplastic inflammatory cells, mostly macrophages.

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Myomatous hepatic angiomyolipoma: imaging findings in 14 cases with radiological–pathological correlation and review of the literature

Cited by 9 publications

References 23 publications

Hepatic angiomyolipoma presenting with chronic epigastric pain: A case report

Hepatic angiomyolipoma presenting with chronic epigastric pain: A case report

Utility of Immunohistochemistry in the Diagnosis of a Case of Hepatic Angiomyolipoma from a Tertiary Referral Centre for Liver Diseases

TSC2 inactivation, low mutation burden and high macrophage infiltration characterise hepatic angiomyolipomas

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