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Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed malignancy of vascular origin. We describe a case of a 36-year-old, who presented with a burning sensation in his chest on exercise and was believed to have bilobar intrahepatic cholangiocarcinoma. After receiving chemothe...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
AME Publishing Company
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9468985/ https://www.ncbi.nlm.nih.gov/pubmed/36300157 http://dx.doi.org/10.21037/tgh-20-310 |
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author | Kawka, Michal Mak, Sau Qiu, Shengyang Gall, Tamara M. H. Jiao, Long R. |
author_facet | Kawka, Michal Mak, Sau Qiu, Shengyang Gall, Tamara M. H. Jiao, Long R. |
author_sort | Kawka, Michal |
collection | PubMed |
description | Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed malignancy of vascular origin. We describe a case of a 36-year-old, who presented with a burning sensation in his chest on exercise and was believed to have bilobar intrahepatic cholangiocarcinoma. After receiving chemotherapy with partial response, the patient underwent staged resection with modified associating liver partition with portal vein ligation for staged hepatectomy (ALPPS) procedure—laparoscopic radiofrequency-assisted ALPPS (RALPPS). Histological examination of the stage 1 specimen revealed HEHE deposits in the left lobe of the liver. The patient proceeded to have stage 2 open right hepatectomy with concurring histology. Ninety-day follow-up computed tomography scan showed almost complete resolution of HEHE lesions. Low incidence, non-specific clinical and radiological characteristics all contribute to high HEHE misdiagnosis rate. Histological analysis can be used for confirmation of the diagnosis; however, specialist staining is required, which is not routinely performed. Multiple treatments are available against HEHE, but only liver resection and liver transplantation are potentially curative. ALPPS and its modifications (such as RALPPS) offer an alternative from conventional two-stage hepatectomy for patients with extensive bilobar HEHE, who are at risk of developing post-hepatectomy liver failure or patients with anomalous hepatic vasculature anatomy which makes portal vein embolisation (PVE) challenging. |
format | Online Article Text |
id | pubmed-9468985 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | AME Publishing Company |
record_format | MEDLINE/PubMed |
spelling | pubmed-94689852022-10-25 Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report Kawka, Michal Mak, Sau Qiu, Shengyang Gall, Tamara M. H. Jiao, Long R. Transl Gastroenterol Hepatol Case Report Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed malignancy of vascular origin. We describe a case of a 36-year-old, who presented with a burning sensation in his chest on exercise and was believed to have bilobar intrahepatic cholangiocarcinoma. After receiving chemotherapy with partial response, the patient underwent staged resection with modified associating liver partition with portal vein ligation for staged hepatectomy (ALPPS) procedure—laparoscopic radiofrequency-assisted ALPPS (RALPPS). Histological examination of the stage 1 specimen revealed HEHE deposits in the left lobe of the liver. The patient proceeded to have stage 2 open right hepatectomy with concurring histology. Ninety-day follow-up computed tomography scan showed almost complete resolution of HEHE lesions. Low incidence, non-specific clinical and radiological characteristics all contribute to high HEHE misdiagnosis rate. Histological analysis can be used for confirmation of the diagnosis; however, specialist staining is required, which is not routinely performed. Multiple treatments are available against HEHE, but only liver resection and liver transplantation are potentially curative. ALPPS and its modifications (such as RALPPS) offer an alternative from conventional two-stage hepatectomy for patients with extensive bilobar HEHE, who are at risk of developing post-hepatectomy liver failure or patients with anomalous hepatic vasculature anatomy which makes portal vein embolisation (PVE) challenging. AME Publishing Company 2022-10-25 /pmc/articles/PMC9468985/ /pubmed/36300157 http://dx.doi.org/10.21037/tgh-20-310 Text en 2022 Translational Gastroenterology and Hepatology. All rights reserved. https://creativecommons.org/licenses/by-nc-nd/4.0/Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) . |
spellingShingle | Case Report Kawka, Michal Mak, Sau Qiu, Shengyang Gall, Tamara M. H. Jiao, Long R. Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
title | Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
title_full | Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
title_fullStr | Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
title_full_unstemmed | Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
title_short | Hepatic epithelioid hemangioendothelioma (HEHE)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
title_sort | hepatic epithelioid hemangioendothelioma (hehe)—rare vascular malignancy mimicking cholangiocarcinoma: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9468985/ https://www.ncbi.nlm.nih.gov/pubmed/36300157 http://dx.doi.org/10.21037/tgh-20-310 |
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