Hepatic epithelioid haemangioendothelioma: difficult name, difficult diagnosis?

Abstract

Summary Two patients with epithelioid haemangioendotheliomata of the liver are described. Both presented with abdominal pain and malaise, with hepatomegaly and a variable degree of hepatocellular dysfunction. Diagnosis was delayed in both cases, each patient undergoing a protracted series of investigations including repeated liver biopsies. The major obstacles to early diagnosis were a lack of clinical awareness of the condition and difficulties in interpretation of the liver histology: the widespread sclerosis in the tumour tissue is easily mistaken for a post-necrotic or cirrhotic process. The key to the diagnosis is the demonstration of cells containing Factor-VIII-related antigen confirming the endothelial origin of the tumour. One patient died within three months of presentation and the other after 18 months. The tumour may, therefore, be more aggressive than earlier reports seem to suggest. It seems likely that the tumour is being under-diagnosed and although no specific therapy has been shown to be of value, a greater awareness of the condition, resulting in a more prompt diagnosis, should save patients from undergoing unnecessary investigation.

Epithelioid hemangioendothelioma of the liver

“…A total of 35 cases treated by orthotopic hepatic transplantation have been reported in the literature. 14,17,46,48,90 Metastatic spread at the time of surgery does not correlate with survival and has not been considered a contraindication to surgery in two series. 17,27 The role of adjuvant chemotherapy, radiotherapy, and/or treatment with interferon-␣-2 remains unclear.…”

Central amygdala mineralocorticoid receptors modulate alcohol self-administration

“…Alternatively, while the timing of MR antagonist effects on alcohol self-administration (within minutes) suggest that they are non-genomically mediated (Karst et al, 2005, Khaksari et al, 2007, the effect of MR tone on alcohol drinking may be mediated, partially or in full, by genomic MR action as a ligand-dependent transcription factor (Fuller et al, 2000, Ruhs et al, 2017. Notably, the ASO knockdown utilized in these studies was transient and resulted in a similar behavioral effect as MR antagonism on self-administration, while knockdown studies finding opposite results to antagonism utilize genomic manipulation of MR (Cobden et al, 1988, Lai et al, 2007, Rozeboom et al, 2007, Brinks et al, 2009, Ter Horst et al, 2012. Differences in the impact of MR expression and antagonism on alcohol self-administration could be further clarified by characterizing MR expression patterns within the CeA of animals with high and low MR expression, or by assessing alcohol self-administration in animals with genomically altered CeA MR expression.…”

Epithelioid Hemangioendothelioma of the Liver: A Report of Two Cases

“…Fewer than 100 case of primary hepatic EHEs have been reported in the medical literature (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). The patients ranged in age from 12 co 86 years ( mean 41.5) with a female: malc ratio of 2: 1.…”

“…Negative staining for mucin and bile, negative immunochemical staining for CEA and alpha-fetoprotein and the presence of many thin filaments and Weibel-Palade bodies, ultrastructurally, are other useful differentiating features (2,10). However, for practical purposes, it is the identification of the tumour cells' vascular origin, based on their immunohistochemical reacttv1ty for vWF:Ag and Ulex europeaus I leccin, that is key to the correct diagnosis (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). Positive staining of the neoplastic cells tends to be variable and may be seen within the cytoplasm, intracytoplasmic vacuoles and/or within vascular lumina.…”

“…Primary hepatic EHE appears to be rare, based on the number of cases reported in the medical literature. However, because of the diagnostic difficulties it can presentas has been demonstrated by the frequency of initial misdiagnosis -it may be a more common tumour than reported (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). The etiology is uncertain.…”