Abstract
Angiomyolipoma (AML) is a rare mesenchymal neoplasm of the tumor, composed of a varying heterogeneous mixture of three tissue components: blood vessels, smooth muscle and adipose cells. Hepatic AML may demonstrate a marked histological diversity. We herein present one case of hepatic AML exhibiting prominent inflammatory cells in the background, which happened in a 61-year-old Chinese female patient, without signs of tuberous sclerosis. Histologically, the striking feature was the infiltration of numerous inflammatory cells in the background, including small lymphocytes, plasma cells, and eosnophils. The tumor cells were spindled and histiocytoid in shape, with slightly eosinophilic cytoplasm, and arranged along the vessels or scattered among the inflammatory background. Sinusoid structure was obviously seen in the tumor. Mature adipocytes and thick-walled blood vessels were focally observed at the boundaries between the tumor and surrounding liver tissues. The tumor cells were positive immunostaining for HMB-45, Melan-A, and smooth muscle actin. The inflammatory AML should be distinguished from other tumors with inflammatory background such as inflammatory myofibroblastic tumor and follicular dendritic cell tumor and deserves wider recognition for its occurrence as a primary hepatic tumor.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1828633072762370
Highlights
Hepatic angiomyolipoma is a rare, benign, hepatic mesenchymal neoplasm found in both males and females, and most commonly in adult females
Other unusual architectural patterns such as pelioid and inflammatory ones were usually present as focal finding, but sometimes they may exist as a pure pattern [4,8,9] which makes it difficult to distinguish with other hepatic tumors
The immunophenotype (CD21, CD35, S100, smooth muscle actin (SMA) focal +, ALK-) overthrows the diagnosis of follicular dendritc cell (FDC) tumor, IPT and inflammatory myofibroblastic tumor (IMT), so we reviewed this case carefully and found some scattered adipocytes and thick-walled blood vessels at the boundaries between the tumor and surrounding liver tissues (As shown in Figure. 1I)
Summary
Hepatic angiomyolipoma is a rare, benign, hepatic mesenchymal neoplasm found in both males and females, and most commonly in adult females. The liver represents the second most frequent site of involvement [1] Similar to those in the kidney, hepatic AML consists of a mixture of myoid cells, adipose tissue and thick-walled vessels. Immunohistochemistry The immunohistochemical study showed that the histiocytoid cells were faintly positive for AE1/AE3 (Figure 2A and B), strongly diffuse positive for vimentin (Figure 2C), HMB-45 (Figure 2D), Melan-A (Figure 2E), focally positive for smooth muscle actin (SMA) (Figure 2F), and occassionally positive for CD68 (Figure 2G) They were strictly negative for CD21 (Figure 2H), S100 (Figure 2I), ALK (Figure 2J) CD1α, Hepar-1, CD35, CD10, CD23, CD117, DOG-1, synaptophysin and chromogranin A (data not shown).
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