PMC5925158_01
Epithelioid hemangioendothelioma arising within the spleen. (a) Axial MRI scan showing liver hemangiomata (white masses) and vascular splenic lesions. (b) Gross specimen following splenectomy. White arrows correspond to imaging findings in (a). (c) H&E staining demonstrated a highly cellular, atypical complex vascular lesion with rare mitotic figures. (d) MIB-1 staining illustrates a proliferation index of approximately 10%. (e) Immunophenotyping with CD31.
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These lesions were followed with serial MRI studies, and while the liver lesions remained stable in size and enhancement, follow-up imaging at two years demonstrated an interval enlargement in the splenic lesion to 7.1 x 5.0 x 6.3 cm, with four additional new nodules within the spleen (Figure 1(a), white arrows). The dominant lesion had heterogenous enhancement compared to the liver hemangiomata (white lesions within the liver in Figure 1(a)). Gross pathology demonstrated an 8.0 x 5.8 x 4.5 cm lesion with a mottled appearance and focal hemorrhagic tissue which nearly replaced the splenic parenchyma (Figure 1(b)), which was consistent with preoperative imaging (Figure 1(a); white arrows correlate to similar areas in gross specimen in Figure 1(b)). Histological assessment demonstrated a highly cellular, atypical complex vascular lesion with rare mitotic figures on H&E staining (Figure 1(c)). MIB-1 staining illustrates a proliferation index of approximately 10% (Figure 1(d)). Immunophenotyping with CD31 (Figure 1(e)) and CD34 (not shown) confirmed the presence of vascular endothelium.
Epithelioid hemangioendothelioma in explant following liver transplantation. (a) Axial CT scan showing liver hemangioma (dark mass) and no obvious additional lesions. Red arrow indicates known residual hemangioma following left lobectomy. (b) Gross specimen following hepatectomy and liver transplantation. White arrows indicate multifocal EHE which was not apparent on preoperative imaging studies. Red arrow indicates corresponding lesion from (a), which demonstrated microscopic EHE within hemangioma on microscopic examination (not pictured).
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She ultimately underwent liver transplantation in early 2017, with evidence of multifocal HEHE in the explanted liver (Figure 2).