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

BACKGROUND

Angiomyolipoma (AML) is a solid benign neoplasm with mesenchymal features. The clinical signs and symptoms of hepatic angiomyolipoma are nonspecific, and treatment strategy is variable.

PRESENTATION

A 35-years-old male patient has admitted to the hospital with symptoms of severe anemia. Abdominal multi-slice computed tomography (MSCT) and Gadoxetic acid (GA)-enhanced magnetic resonance imaging (MRI) revealed a heterogeneous 23 × 17 cm-in-sized tumor with heterogeneous enhancement and increased angiogenesis. Percutaneous hepatic biopsy was proceeded and in immunohistochemistry, tumor cells responded positively to the HMB-45, SMA, and Glutamine stains, the CD-34 stain was positive for blood vessels as well as Ki-67 sporadically positive, but the Heppar1, S-100, CK stains reacted negative. The final pathologic result was consistent with the primary hepatic angiomyolipoma. The final surgical management was extended right hepatectomy with ligation of the right hepatic artery (RHA) and vein (RHV), as well as the middle hepatic vein (MHV), one month after portal embolization as well as to enlarge the remnant liver volume.

DISCUSSION

A hepatic angiomyolipoma (HAML) primary origin is relatively rare, the clinical manifestations are variable and non-specific. Histological examination and immunohistochemistry staining are considered as the gold standard for HAML diagnosis. HAML are commonly expressed benign behaviors, but HAML cases with malignant behaviors were reported in a cumulative incidence. Radical surgery must be still the most effective and major treatment approach.

CONCLUSION

The present case being the first case with initial syndrome of severe anemia accounted in the English literature. Giant angiomyolipoma is composed of blood vessels and could lead to extensive internal tumoral hemorrhage. We here present a report of this case with had primary hepatic angiomyolipoma with clinical picture of severe anemia.