Successful treatment of metastatic hepatic epithelioid hemangioendothelioma with thalidomide: a case report

<p>Abstract</p> <p>Introduction</p> <p>Hepatic epithelioid hemangioendothelioma is a rare malignancy arising from the vascular endothelial cells within the liver. Historically, the disease is characterized as being poorly responsive to both chemotherapy and radiotherapy, with liver resection or transplantation the treatment of choice when feasible. For patients with advanced disease, reports of long-term therapeutic benefits from conventional cytotoxic treatments are very limited. Owing to the rarity of this malignancy, there is no structured therapeutic research, but a small number of cases have been reported to respond well to treatment with inhibitors of angiogenesis. Thalidomide was originally developed as an anti-emetic but is a potent inhibitor of vascular neogenesis, and could offer potential in the treatment of hepatic epithelioid hemangioendothelioma by blocking the proliferation of the malignant vascular endothelial cells.</p> <p>Case presentation</p> <p>We describe the case of a Caucasian British woman who presented at the age of 53 years with a hepatic mass, malignant lymphadenopathy and pulmonary metastases, which were confirmed as hepatic epithelioid hemangioendothelioma on biopsy. After unproductive treatment with interferon, our patient was started on thalidomide 400 mg daily. She has been successfully managed on this therapy for the past seven years, and has remained asymptomatic, with radiologically stable disease and minimal treatment-related side effects.</p> <p>Conclusion</p> <p>At present, there is no standard therapy for advanced hepatic epithelioid hemangioendothelioma. Our case supports the role for thalidomide and potentially other inhibitors of vascular neogenesis in the treatment of patients with metastatic hepatic epithelioid hemangioendothelioma.</p

Differential expression of epithelial sodium channel subunit mRNAs in rat skin

Three subunits (alpha, beta, gamma) of the amiloride-sensitive epithelial sodium channel have been recently characterized. The channel subunits have significant homologies with the Caenorhabditis elegans mec-4, mec-10 and deg-1 genes, which are involved in control of cell volume and mecanotransduction. These subunits are coexpressed at equivalent levels in the renal collecting duct and the distal colon epithelium which are high resistance sodium transporting epithelia. We have investigated whether these subunits were expressed, at the mRNA level, in transporting as well as non transporting epithelial cells of rat skin. In full-thickness abdominal skin only alpha and gamma subunit mRNAs were detected, while all three subunit mRNAs were present in sole skin, as demonstrated by RNase-protection assay. Furthermore, the level of expression of each subunit varied with the epithelial cell type as demonstrated by in situ hybridization: epidermal and follicular keratinocytes express mostly alpha and gamma subunits (while beta was low); a prevalence of beta and gamma was observed in sweat glands. Thus, it appeared that two out of the three subunit mRNAs predominated in each epithelial structure. In addition, mRNAs of the alpha, beta and gamma subunits of the amiloride-sensitive sodium channel were expressed at a higher level in large suprabasal epidermal keratinocytes (which undergo terminal differentiation) than in small proliferative basal keratinocytes

Hemangioendotelioma epitelióide de pleura Epithelioid hemangioendothelioma of the pleura

Relata-se o caso de um paciente exposto profissionalmente a asbesto por dez anos e portador de um tumor pleural muito raro, o hemangioendotelioma epitelióde. O paciente apresentava derrame pleural serohemorrágico, sem evidência de células neoplásicas e com predomínio de linfócitos. A biópsia pleural por agulha revelou processo inflamatório crônico inespecífico, com áreas de tecido mixóide. A videotoracoscopia mostrou nódulos difusos nas pleuras parietal e visceral. A biópsia revelou neoplasia mesenquimal e eram semelhantes às áreas focais observadas na primeira biópsia. O estudo imunohistoquímico demonstrou a presença dos marcadores vasculares CD31, CD34 e Fator VIII, caracterizando a origem vascular do tumor. O paciente foi tratado com cisplatina e ectoposide, tendo o óbito ocorrido três meses após o diagnóstico.<br>Epithelioid hemangioendothelioma (EHE), a very uncommon pleural tumor, was diagnosed in a 61-year-old man with work-related exposure to asbestos. Serohemorrhagic pleural effusion was diagnosed in the work-up of this patient, whose complaints were chest pain and weight loss. A lymphocytic predominance was present in the effusion, but no malignant cells were seen; pleural needle biopsy disclosed only a non-specific inflammatory process. Video thoracoscopy revealed nodules in parietal and visceral pleurae. A biopsy revealed a mesenchymal neoplasm; vascular markers CD 31, CD 34 and VIII factor were present; therefore, diagnosis of HE was accepted. The tumor was not responsive to cisplatin or etoposide and the patient died 3 months after the diagnosis