Dig Dis Sci DOI 10.1007/s10620-016-4039-z

CORRESPONDENCE

Radiographic and Histologic Findings of a Rare Liver Tumor-Hepatic Epithelioid Hemangioendothelioma Noel Yarze1 • Joseph C. Yarze2,3

Received: 31 December 2015 / Accepted: 10 January 2016 Ó Springer Science+Business Media New York 2016

To the Editor A 53-year-old male with a history of COPD presented for evaluation of an abnormal abdominal CT scan. Surgical history was significant for repair of a left diaphragmatic hernia several months earlier. The patient was asymptomatic, and the liver biochemical tests and serum alphafetoprotein were normal. At that time, a CT scan also showed a large liver lesion. The lesion was located in the right lobe of the liver and was described as being heterogeneous with areas of calcification. It measured 13.9 9 8.2 cm. An MRI scan revealed a heterogeneous T2 hyper intense and T1 hypointense 14 cm 9 9.5 cm mass in the inferior portion of the right liver lobe (see Fig. 1). The mass was lobulated and it enhanced in an irregular and delayed fashion. Multiple subtle, smaller satellite lesions were seen. Capsular retraction was noted within the right liver lobe anterolaterally. Liver biopsy revealed a tumor that had fairly evenly distributed small epithelioid cells present within abundant eosinophilic to myxoid stroma. Some of these cells had small intracytoplasmic vacuoles, resembling intracytoplasmic lumina. The nuclei were small and cytologically bland. The neoplasm and stroma infiltrated the sinusoids and separated hepatocytes, making the junction between the neoplasm and the benign hepatic

& Joseph C. Yarze [email protected] 1

Ross University School of Medicine, Edison, NJ, USA

2

Gastroenterology Associates of Northern New York, Glens Falls, NY, USA

3

Clinical Associate Professor of Medicine, Albany Medical College, Albany, NY, USA

parenchyma indistinct. This histologic appearance and infiltrative pattern were characteristic of epithelioid hemangioendothelioma (HEHE). To verify this presumptive diagnosis, immunohistochemical staining for CD31 and CD34 was performed and it highlighted the neoplastic cells, confirming the diagnosis. HEHE is a very rare malignant vascular tumor of endothelial origin, which was initially described in 1982 [1]. HEHE has an unknown etiology and an incidence between 0.1 and 1 per 100,000 individuals. The natural history is variable, and the clinical manifestations of HEHE vary from being asymptomatic to causing nonspecific symptoms to liver failure. Lung, peritoneum, lymph nodes, and bone are the most common sites of extra-hepatic involvement. Radiologic imaging studies may be helpful, and MRI may detect findings suggestive of the lesion [2]. The diagnosis, however, ultimately depends on histopathologic staining of the cells combined with immunochemistry. Specifically, HEHE tumors have immunochemical reactivity to endothelial markers including vimentin, factor VIII, CD 10, CD31, and CD34 [3]. Current management options include medical therapy with anti-angiogenic agents (thalidomide and bevacizumab), chemoradiotherapy, liver resection, liver transplantation, or a watch-and-wait strategy [4, 5]. After numerous discussions, our patient adamantly refused all but an observational approach, and he agreed to undergo a repeat liver MRI in 1 year. In conclusion, HEHE is a rare vascular neoplasm with a highly variable clinical presentation and natural history. As in our case, the definitive diagnosis rests upon histopathologic staining of biopsy tissue combined with immunohistochemical testing. Numerous treatment options have been described; however, due to the rarity of the tumor and its unpredictable natural history, it is not possible to assess the effectiveness of these therapies in a systematic fashion.

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References 1. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50:970–981. 2. Lyburn ID, Torreggiani WC, Harris AC, et al. Hepatic epithelioid hemangioendothelioma: sonographic, CT, and MR imaging appearances. Am J Roentgenol. 2003;180:1359–1364. 3. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer. 1999;85:562–582. 4. Mehrabi A, Kashfi A, Fonouni H, et al. Primary malignant hepatic epithelioid hemangioendothelioma. A comprehensive review of the literature with emphasis on the surgical therapy. Cancer. 2006;107:2108–2121. 5. Gurung S, Hua F, Zhang WW, Gu YH. Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum, omentum and mesentery: a case report. Int J Clin Exp Pathol. 2015;8:5883–5889.

Fig. 1 T2-weighted axial MRI revealing a hyperintense, lobulated liver lesion with subtle capsular retraction of the right liver lobe anterolaterally

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Radiographic and Histologic Findings of a Rare Liver Tumor-Hepatic Epithelioid Hemangioendothelioma.

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