DOI: 10.12890/2024_004529
A young female with a hepatic epithelioid hemangioendothelioma
Authors
natali6221@gmail.com
Keywords
Hepatic epithelioid hemangioendothelioma, liver biopsy, vascular tumourAbstract
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.
References
- Virarkar M, Saleh M, Diab R, Taggart M, Bhargava P, Bhosale P. Hepatic hemangioendothelioma: an update. World J Gastrointest Oncol 2020;12:248–266.
- Din NU, Rahim S, Asghari T, Abdul-Ghafar J, Ahmad Z. Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases. Diagn Pathol 2020;15:120.
- Ullah K, Dogar AW, Uddin S, Jan Z, Abbas SH, Shoaib A. Hepatic hemangioendothelioma – a rare case report. Int J Surg Case Rep 2021;87:106424.
- Kou K, Chen YG, Zhou JP, Sun XD, Sun DW, Li SX, et al. Hepatic epithelioid hemangioendothelioma: update on diagnosis and therapy. World J Clin Cases 2020;8:3978–3987.
- Lakkis Z, Kim S, Delabrousse E, Jary M, Nguyen T, Mantion GB, et al. Metronomic cyclophosphamide: an alternative treatment for hepatic epithelioid hemangioendothelioma. J Hepatol 2013;58:1254–1257.
- Requena L, Kutzner H. Hemangioendothelioma. Semin Diagn Pathol 2013;30:29–44.
- Fan YH, Tang HN, Zhou JP, Fang Q, Li SX, Kou K, et al. Fast-growing epithelioid hemangioendothelioma of the liver: a case report. Medicine (Baltimore) 2020;99:e22077.
- Merriam P, Nathenson MJ. Liver transplantation for hepatic epithelioid hemangioendothelioma. Cancer 2021;127:3714–3716.
- Lai Q, Feys E, Karam V, Adam R, Klempnauer J, Oliverius M, et al. European Liver Intestine Transplant Association (ELITA). Hepatic epithelioid hemangioendothelioma and adult liver transplantation: proposal for a prognostic score based on the analysis of the ELTR-ELITA Registry. Transplantation 2017;101:555–564.
- Frezza AM, Ravi V, Lo Vullo S, Vincenzi B, Tolomeo F, Chen TW, et al. Systemic therapies in advanced epithelioid haemangioendothelioma: a retrospective international case series from the World Sarcoma Network and a review of literature. Cancer Med 2021;10:2645–2659.
