Pulmonary Epithelioid Hemangioendothelioma: Advances in Treatment Options despite a Rare Vascular Tumor

We have already discussed about dail and liebow describing epithelioid hemangioendothelioma (PEH) as an aggressive cell carcinoma with a remarkable propensity to invade blood vessels and small airways, the following Weiss and description of epithelioid hemangioendothelioma (EHE) as a bone and soft tissues tumor showing intermediate malignancy hemangioma and angiosarcoma, and the final confirmation Weldon-Linne of a factor-VIII-related antigen onto malignant [1]. The recent World Health Organization (WHO 2002) has described EHE as lesions that fall into the category of aggressive tumors with metastatic potential [2,3]. The etiology EHE is still a dilemma, as several clonal abnormalities in tumor , and different angiogenic stimulators may act as promoters of cell proliferation [4-8]. A new etiopathogenetic hypothesis a causal relationship between chronic Bartonella infection and development of this rare vascular tumor: if this was confirmed, it be plausible that eradicating the bacterial infection or Bartonella-induced angiogenic and proliferative cell could slow the tumor progression and improve patient [9]. to its rarity (EHE represents less than 1% of all vascular ), because of EHE clinical presentation is as heterogeneous as its localization can be, there is no standard for treatment and few options are available. As the most common presentations liver alone (21%), liver plus lung (18%), lung alone (12%) and bone (14%), the available literature focuses above all on these three [10]. The general issue seems to be that, when bone or lesions are small and limited in number, surgical, curative (that is amputation, en-bloc resection, wedge resection) good outcomes [11,12]. Locally advanced hepatic EHE seems benefit from transplantation, with good results [13]. However, a surgical resection is not usually feasible: Pinet et al. reported case of an aggressive form of pleural EHE resulting in complete after treatment with carboplatin plus etoposide [14], as well bilateral multicentric PEH seems to respond well to interferon 2α, some partial spontaneous regression [7]. Nonetheless, when a fracture occurs, a great alternative to plate-and-screws stabilization is radiofrequency ablation: by creating small, controlled, thermal injuries of bone, the extent of resection be reduced [15]. On the other side, with the aim of controlling desease given the recurrence of EHE, radiation therapy (RT) is for localized EHE, while chemotherapy is preferred for disease. In any case, the beneficial effect of both is still not [14,16-19]. Given the vascular origin of this tumor, the use antiangiogenetic molecules like thalidomide and lenalidomide might achieve good outcomes (partial response and several diseases), even if using bevacizumab and nanoparticle albuminbound (nab-paclitaxel) produced higher percentages of disease [20]. RT has proven to be ineffective for PEH of the tumor’s radiobiological characteristics (slow growth of tumor cells), while a good local control has been obtained in EHE bone [19, 21] Since EHE has been correctly defined, several research have dealt with EHE bone irradiation, in order to define the and the dose distribution achieving the greatest results for disease control, together with the greatest tolerance to the and the lowest incidence of side effects [19,21-24]. A 6400 adjuvant RT was also performed against an axillary form of EHE, in the absence of lymph nodes metastases but pleural and widespread [18]. In our experience, at our Institute we systemic progression of EHE after 4-month-PEG-IFN-α in spite of stable pulmonary disease; then, lumbar pain control good tolerance and better quality of life at 1-year-follow-up from symptomatic, normofractionated RT onto the L3-L5 vertebral tract, outcome through Ifosfamide and Epirubicin soon after RT, and surgical removal of EHE spleen , showing 1-year survival [1]. this considered, despite the rarity of this vascular tumor and its complex and heterogeneous patterns of presentation, we are capable of performing more treatment lines, combined a multimodal strategy or sequencially adopted, so that we are able to good outcomes in most of cases. In this regard, taking into the EHE radiobiological characteristics, no doubt evidence is in favour of RT obtaining local pain control with good tolerance better quality of life at least one-year-follow up. The literature certain discordance about the radiation dose to be used for this , maybe attributable to the very small number of patients and the limited survival that EHE typically shows. As a result, further are needed to answer the question.