Multiple Angiomyolipoma of the Liver

Nonomura, Akitaka; Mizukami, Yuji; Kadoya, Masumi; Takayanagi, Nobutatsu; Hirono, Teisuke

doi:10.1097/00004836-199504000-00018

Cited by 25 publications

(24 citation statements)

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“…They are probably more closely related to the hepatic sinusoidal endothelium and occur in many primary benign or malignant liver tumors [4]. Most hepatic AMLs are solitary, as in our seven cases; however, multiple AMLs of the liver have been described [4,17,18], resulting in an overall multiplicity of 6.7 % [4]. Association with tuberous sclerosis (TS) ranges from 5.8 % to 10 % [3,4,6] and goes together with a higher incidence of multiplicity [19].…”

Section: Discussionmentioning

confidence: 78%

Correlation of MRI and CT findings with histopathology in hepatic angiomyolipoma

et al. 2001

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Hepatic angiomyolipomas are rare and often mimic other liver tumors. The aim of our study was to describe the CT and MRI findings and to correlate imaging features with histopathology. The CT and/or MR images were available for retrospective analysis in seven patients. Patients had non-enhanced as well as enhanced CT (n = 6) or MRI (n = 4) before and after administration of Gd-DTPA (n = 2) or MnDPDP, a liver specific contrast agent, (n = 3). In three patients CT and MRI did not detect fat, and in two patients the angiomyolipomas were also histopathologically devoid of fat. Vascularity ranged from hypervascular (n = 4) with arteriovenous shunts (n = 1) to equal (n = 1) or less (n = 2) postcontrast enhancement compared with the normal liver parenchyma. No uptake of the liver specific contrast agent, MnDPDP, was observed (n = 3). Predominantly, CT and MRI did not include angiomyolipoma in the differential diagnosis, and the initial histopathological evaluation was inconclusive in more than half the cases. Hepatic angiomyolipomas frequently manifest as solitary well-circumscribed heterogeneous masses in patients with no underlying liver disease or elevation of serum tumor markers. If present, the demonstration of intratumoral fat is helpful in the diagnosis of angiomyolipoma. The final diagnosis can be obtained by immunohistochemistry.

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Section: Discussionmentioning

confidence: 78%

Correlation of MRI and CT findings with histopathology in hepatic angiomyolipoma

et al. 2001

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“…Truly multiple AMLs (more than two lesions) along with multiple renal AMLs have only been described in patients with tuberous sclerosis (2,10,11). Two patients, each with two hepatic AMLs, have been reported from Japan without any clinical evidence of tuberous sclerosis (12,13). This is the first report of a patient with multiple hepatic AMLs without neurologic manifestations or any radiologic features suggestive of tuberous sclerosis.…”

Section: Discussionmentioning

confidence: 91%

“…Multiple AMLs are uncommon and usually occur in tuberous sclerosis patients (2,10,11). There have been only two reported cases of multiple hepatic AMLs in patients without tuberous sclerosis (12,13); both patients were Japanese and each had only two hepatic lesions. To the best of our knowledge, cases with more than two AMLs of the liver in patients without tuberous sclerosis have not been reported to date.…”

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confidence: 99%

Multiple Angiomyolipomata of the Liver: A Case Report

et al. 2002

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Angiomyolipoma (AML) is a rare benign tumor that occurs most commonly in the kidney. Occasionally it may be found in the liver. Lesions in the liver are usually solitary. Multiple AMLs are extremely rare and are typically seen in patients with tuberous sclerosis. We now report an unusual case of a 46-year-old woman with multiple hepatic AMLs. There were more than 15 lesions distributed predominantly in the right hepatic lobe. The tumors ranged from 0.2 to 6 cm in size and consisted of a variable admixture of proliferating blood vessels, adipose tissue, and smooth muscle. There was no clinical evidence of tuberous sclerosis in this patient. Polymerase chain reaction amplification of the highly polymorphic human androgen receptor gene (HU-MARA) was performed and the pattern of X chromosome inactivation was analyzed. Three of the five representative AML nodules showed a preferential loss of one of the two HUMARA alleles indicating a clonal proliferation with involvement of different alleles. Histologic examination of the corresponding lesions showed clonal lesions to be predominantly composed of epithelioid myoid cells while the polyclonal lesions were predominantly composed of adipose tissue. While the histologic diagnosis of AML in a surgical resection specimen is often straightforward, the radiographic, cytologic and intraoperative interpretation of a case with multiple lesions presents a considerable challenge.

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“…2 They are usually solitary, but rare cases of multiple hepatic angiomyolipoma have been reported. 12,13 Typically, the aspirates display smooth muscle cells with cigar-shaped nuclei, prominent endothelial cells in vessels, and some mature fat cells. The amount of these components varies depending on their relative predominance.…”

Section: Discussionmentioning

confidence: 99%

Hepatic angiomyolipoma: Report of two cases with emphasis on smear cytomorphology and the use of cell block with immunohistochemical stains

Lin

Eng

et al. 2004

Diagnostic Cytopathology

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Angiomyolipoma is a benign mesenchymal lesion that occurs most commonly in the kidney. Its occurrence in the liver is relatively rare. We report two cases of hepatic angiomyolipoma diagnosed by ultrasound-guided, needle aspiration biopsy. One of the patients was a previously healthy 62-yr-old woman who presented with a hepatic mass incidentally during a healthy examination. The other patient was a 67-yr-old man, a victim of chronic hepatitis C, who was found to have a hepatic tumor in the right lobe during regular follow-up. Cytologic preparations of the two hepatic masses showed some mature adipocytes admixed with clusters of variable-sized mesenchyme-like cells with fibrillar cytoplasm and indistinct cytoplasmic borders. Some of the cells were round to oval and others were spindle shaped with oval, cigar-shaped or elongated nuclei. The nuclear chromatin was fine and some cells showed round nucleoli. No thick-walled blood vessels were noted in the cytological smears. In the cell block preparations, a mixture of thick-walled blood vessels, mature adipose tissue, and bundles of variable-sized smooth muscle cells were noted. The spindle or epithelioid smooth muscle cells were positive for vimentin, alpha-smooth muscle actin, and human melanoma black (HMB)-45 but negative for desmin and S100 protein in immunohistochemical stains. The purpose of the current study is to describe cytological features of this lesion with differential diagnosis because pathologists may be called on to render a diagnosis on needle aspiration. HMB-45 has been proved to be a sensitive marker for the smooth muscle component of angiomyolipoma. Cell block preparations assisted with immunohistochemical staining should be emphasized for definite diagnosis. Although a surgical procedure may still be necessary once a diagnosis of angiomyolipoma is made, treatment may be tailored for a less aggressive procedure than for malignant hepatic nodules.

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Multiple Angiomyolipoma of the Liver

Cited by 25 publications

References 0 publications

Correlation of MRI and CT findings with histopathology in hepatic angiomyolipoma

Correlation of MRI and CT findings with histopathology in hepatic angiomyolipoma

Multiple Angiomyolipomata of the Liver: A Case Report

Hepatic angiomyolipoma: Report of two cases with emphasis on smear cytomorphology and the use of cell block with immunohistochemical stains

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