A Young Female with a Hepatic Epithelioid Hemangioendothelioma

Abstract

Background: Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of <0.1 cases per 100,000 people worldwide.

Case description: A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team's findings, liver transplantation was indicated as the only curative treatment.

Conclusion: Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis.

Learning points: Hepatic epithelioid hemangioendothelioma is a rare vascular tumour that is often misdiagnosed and mismanaged.This case emphasises the critical importance of interdisciplinary teamwork and the use of non-invasive and invasive techniques to achieve a definitive diagnosis.

Keywords: Hepatic epithelioid hemangioendothelioma; liver biopsy; vascular tumour.

Figure 1

Representative photomicrographs of hepatic epithelioid haemangioendothelioma. Tumour consisted of cords and nests of epithelioid cells in densely hyalised stroma. Tumour cells have moderate amounts of eosinophilic cytoplasm and round nuclei with some nucleoli. The tumour cell showed moderate cellular atypia. The mitotic index was 3 mitosis/10 high-powered fields. Haematoxylin-eosin staining: A) optical microscope (OM) magnification ×40; B) OM magnification ×100; C) OM magnification ×200; D) OM magnification ×400.

Figure 2

Representative photomicrographs of hepatic epithelioid haemangioendothelioma. Immunohistochemical staining method, CD31 staining. The tumour cells were diffusely and strongly CD31 positive. OM magnification ×200.

Figure 3

Representative photomicrographs of hepatic epithelioid haemangioendothelioma. Immunohistochemical staining method, CD34 staining. The tumour cells were diffusely CD34 positive. OM magnification ×200.

Figure 4

Magnetic resonance imaging shows multiple nodules with a poorly vascularised periphery and an avascular central part.

Figure 5

Contrast-enhanced ultrasound shows liver S7 nodule which accumulates contrast media isovascularly to the liver parenchyma, and with a markedly hypovascular/avascular central part.