Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor
Epithelioid hemangioendothelioma is a very rare vascular neoplasm, which is often multifocal or metastatic at diagnosis. Most frequently arises in the liver, followed by the lung and bones. The authors present a case of a liver transplant recipient who developed a pattern of hepatic cholestasis asso...
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| Format: | Article |
| Language: | English |
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Karger Publishers
2023-05-01
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| Series: | GE: Portuguese Journal of Gastroenterology |
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| Online Access: | https://beta.karger.com/Article/FullText/529157 |
| _version_ | 1827918953641410560 |
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| author | Margarida Gonçalves Helena Pessegueiro Judit Gandara José Ramón Vizcaíno Vitor Lopes Sofia Ferreira |
| author_facet | Margarida Gonçalves Helena Pessegueiro Judit Gandara José Ramón Vizcaíno Vitor Lopes Sofia Ferreira |
| author_sort | Margarida Gonçalves |
| collection | DOAJ |
| description | Epithelioid hemangioendothelioma is a very rare vascular neoplasm, which is often multifocal or metastatic at diagnosis. Most frequently arises in the liver, followed by the lung and bones. The authors present a case of a liver transplant recipient who developed a pattern of hepatic cholestasis associated with the appearance of a proliferative hepatic lesion with infiltrative growth. Histological examination and immunohistochemical study were compatible with the diagnosis of epithelioid hemangioendothelioma. Pulmonary micronodules were detected and lung metastases were hypothesized. Therefore, bronchoscopy was performed, which turned out to be normal, and cytology was negative for neoplastic cells. After a multidisciplinary discussion, liver re-transplantation was decided. After 8 years of follow-up, the patient is clinically stable, with no graft dysfunction, no neoplastic recurrence, and dimensional stability of the pulmonary micronodules. Patients with organ transplant have higher risk of developing carcinoma compared to the general population. The development of cancer is a multifactorial process and little is known about the etiology of epithelioid hemangioendothelioma. No standard treatment strategy has been defined yet, and the natural course of the disease is heterogenous and the individual prognosis unpredictable. Complete surgical resection is offered to patients with unifocal disease, and those with unresectable disease should be evaluated for orthotopic liver transplantation. |
| first_indexed | 2024-03-13T03:50:52Z |
| format | Article |
| id | doaj.art-7c39420e70964ee49751474ea2b7b7e4 |
| institution | Directory Open Access Journal |
| issn | 2341-4545 2387-1954 |
| language | English |
| last_indexed | 2024-03-13T03:50:52Z |
| publishDate | 2023-05-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | GE: Portuguese Journal of Gastroenterology |
| spelling | doaj.art-7c39420e70964ee49751474ea2b7b7e42023-06-22T13:45:57ZengKarger PublishersGE: Portuguese Journal of Gastroenterology2341-45452387-19542023-05-011510.1159/000529157529157Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare TumorMargarida Gonçalves0https://orcid.org/0000-0002-2285-0159Helena Pessegueiro1https://orcid.org/0000-0003-2072-8763Judit Gandara2José Ramón Vizcaíno3Vitor Lopes4Sofia Ferreira5https://orcid.org/0000-0003-3796-9635Gastroenterology Department, Braga Hospital, Braga, PortugalLiver Transplant Unit, Centro Hospitalar Universitário do Porto, Porto, PortugalLiver Transplant Unit, Centro Hospitalar Universitário do Porto, Porto, PortugalPathology Department, Centro Hospitalar Universitário do Porto, Porto, PortugalLiver Transplant Unit, Centro Hospitalar Universitário do Porto, Porto, PortugalLiver Transplant Unit, Centro Hospitalar Universitário do Porto, Porto, PortugalEpithelioid hemangioendothelioma is a very rare vascular neoplasm, which is often multifocal or metastatic at diagnosis. Most frequently arises in the liver, followed by the lung and bones. The authors present a case of a liver transplant recipient who developed a pattern of hepatic cholestasis associated with the appearance of a proliferative hepatic lesion with infiltrative growth. Histological examination and immunohistochemical study were compatible with the diagnosis of epithelioid hemangioendothelioma. Pulmonary micronodules were detected and lung metastases were hypothesized. Therefore, bronchoscopy was performed, which turned out to be normal, and cytology was negative for neoplastic cells. After a multidisciplinary discussion, liver re-transplantation was decided. After 8 years of follow-up, the patient is clinically stable, with no graft dysfunction, no neoplastic recurrence, and dimensional stability of the pulmonary micronodules. Patients with organ transplant have higher risk of developing carcinoma compared to the general population. The development of cancer is a multifactorial process and little is known about the etiology of epithelioid hemangioendothelioma. No standard treatment strategy has been defined yet, and the natural course of the disease is heterogenous and the individual prognosis unpredictable. Complete surgical resection is offered to patients with unifocal disease, and those with unresectable disease should be evaluated for orthotopic liver transplantation.https://beta.karger.com/Article/FullText/529157liver transplantationepithelioid hemangioendotheliomacancer |
| spellingShingle | Margarida Gonçalves Helena Pessegueiro Judit Gandara José Ramón Vizcaíno Vitor Lopes Sofia Ferreira Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor GE: Portuguese Journal of Gastroenterology liver transplantation epithelioid hemangioendothelioma cancer |
| title | Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor |
| title_full | Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor |
| title_fullStr | Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor |
| title_full_unstemmed | Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor |
| title_short | Epithelioid Hemangioendothelioma in a Liver Transplant Recipient: A Case Report of an Extremely Rare Tumor |
| title_sort | epithelioid hemangioendothelioma in a liver transplant recipient a case report of an extremely rare tumor |
| topic | liver transplantation epithelioid hemangioendothelioma cancer |
| url | https://beta.karger.com/Article/FullText/529157 |
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