BACKGROUND: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor which has an intermediate aggressive behavior. Although the value of liver transplantation (LT) is well established, its place in the management of HEHE is still unclear. The aim of this study is to confirm, based on a very large patient cohort, the value of LT in the management of HEHE and to identify risk factors for post-LT recurrence. METHODS: The outcome of 149 transplant recipients with HEHE recorded in the European Liver Transplant Registry during the period November 1984 to May 2014 was analyzed. Median post-LT follow-up was 7.6 years (interquartile range, 2.8-14.4). RESULTS: Cox regression analysis showed that macrovascular invasion (hazard ratio [HR], 4.8; P < 0.001), pre-LT waiting time of 120 days or less (HR, 2.6; P = 0.01) and hilar lymph node invasion (HR = 2.2; P = 0.03), but not pre-LT extrahepatic disease, were significant risk factors for recurrence. These findings, which were also confirmed in a propensity score analysis, allowed the development of a HEHE-LT score enabling stratification of patients in relation to their risk of tumor recurrence. Patients with a score of 2 or less had a much better 5-year disease-free survival compared to those having a score of 6 or higher (93.9% vs 38.5%; P < 0.001). CONCLUSIONS: The analysis of this (largest in the world) HEHE adult liver recipient cohort clearly confirms the value of LT in the treatment of this rare disorder and also permits identification of patients at risk of posttransplant recurrence. Posttransplant follow-up should take the HEHE-LT score into account. Extrahepatic disease localization is reconfirmed not to be a contraindication for LT.
- MeSH
- algoritmy MeSH
- časové faktory MeSH
- dospělí MeSH
- epiteloidní hemangioendoteliom diagnóza mortalita chirurgie MeSH
- hodnocení rizik MeSH
- invazivní růst nádoru MeSH
- Kaplanův-Meierův odhad MeSH
- lidé středního věku MeSH
- lidé MeSH
- lokální recidiva nádoru MeSH
- lymfatické metastázy MeSH
- metody pro podporu rozhodování * MeSH
- multivariační analýza MeSH
- nádory jater diagnóza mortalita chirurgie MeSH
- neoadjuvantní terapie MeSH
- prediktivní hodnota testů MeSH
- přežití po terapii bez příznaků nemoci MeSH
- přežívání štěpu MeSH
- proporcionální rizikové modely MeSH
- registrace MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- seznamy čekatelů MeSH
- tendenční skóre MeSH
- transplantace jater * škodlivé účinky mortalita MeSH
- výběr pacientů MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- Geografické názvy
- Evropa MeSH
BACKGROUND/AIM: Hepatic epithelioid haemangio-endothelioma (HEHE) is a very rare malignant tumor of vascular origin and uncertain biological behaviour that is difficult to diagnose using preoperative radiology diagnostic techniques. PATIENTS AND METHODS: The authors present here two patients with HEHE of different extent. The first patient had a generalised form of disease, with involvement of the liver, lungs and bones and was treated with a combination of bevacizumab and capecitabine. The second patient had a localised form of disease involving the liver and this was resolved using a combination of liver resection and radiofrequency ablation. In both patients, the radiology work-up before surgery was non-specific and metastases of another malignant process were considered. The definitive histological diagnosis was made by the pathologist on the basis of immunohistochemical analysis that demonstrated the presence of CD31, CD34 and calmodulin-binding transcription activator 1 (CAMTA 1). RESULTS: Both patients remain in an overall good condition 27 and 5 months respectively following treatment for HEHE. CONCLUSION: Preoperative radiological diagnosis of HEHE is difficult and immunohistochemical examination of the tumor tissue sample remains the key diagnostic tool. Radical surgical resection or liver transplantation is the method of choice in patients with localised liver involvement.
- MeSH
- bevacizumab aplikace a dávkování MeSH
- capecitabinum aplikace a dávkování MeSH
- dospělí MeSH
- epiteloidní hemangioendoteliom diagnóza farmakoterapie patologie chirurgie MeSH
- hepatektomie MeSH
- játra patologie chirurgie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory jater diagnóza farmakoterapie patologie chirurgie MeSH
- nádory kostí diagnóza farmakoterapie patologie sekundární MeSH
- nádory plic diagnóza farmakoterapie patologie sekundární MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Primary hepatic epitheloid heamangioendothelioma (EHE) is a rare neoplasm of vascular origin. We present a retrospective study of 6 patients with EHE treated by liver transplantation that were monitored clinically for more than 3 years and had protocol biopsy samples taken at 1, 3, 5, 7, and 10 years posttransplant. None of the patients suffered from any form of viral or autoimmune hepatitis before or after the transplantation. Two patients had lung metastases detected by preoperative imaging. All tumours showed factor VIII, CD31, and CD34 strong positive staining. In 5 of the 6 transplant recipients the protocol graft biopsies showed chronic non-specific hepatitis with slowly progressive periportal fibrosis that appeared during the 3rd post-transplant year. The septal fibrosis was diagnosed in the 6th and 10th year after transplantation. Liver tests did not reflect either the presence or the degree of inflammation or fibrosis and have remained normal. In retrospect, we consider that our recipients most probably developed alloantigen dependent inflammatory and fibrotic damage to their liver grafts. All six recipients are still alive for a median survival time of 95.1 month (range 44 months to 132 months), with good graft function, and without recurrence of the tumor. The lung metastases in 2 of the 6 patients have remained unchanged for 10 and 12 years retrospectively.
- MeSH
- biopsie metody využití MeSH
- chronická hepatitida diagnóza etiologie komplikace MeSH
- dospělí MeSH
- epiteloidní hemangioendoteliom chirurgie MeSH
- financování organizované MeSH
- homologní transplantace metody trendy využití MeSH
- imunohistochemie metody využití MeSH
- lidé MeSH
- přežití po terapii bez příznaků nemoci MeSH
- retrospektivní studie MeSH
- transplantace jater metody škodlivé účinky využití MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- Publikační typ
- tabulky MeSH
- MeSH
- dospělí MeSH
- epiteloidní hemangioendoteliom chirurgie ultrasonografie MeSH
- jehlová biopsie MeSH
- lidé MeSH
- nádory jater diagnóza terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
