Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

Abstract

Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.

Pulmonary epithelioid hemangioendothelioma misconceived as pulmonary metastasis of other malignancies

“…According to the literature, approximately 248 cases of PEH have been reported [5] and some cases have been diagnosed based on findings of a solitary lung mass or multiple pulmonary nodules with no other primary malignancy [6,7]. This is the first report of multiple pulmonary nodules found in a patient diagnosed with both colon cancer and breast cancer, which were misidentified as metastasis.…”

“…Some studies have reported a 5-year survival probability of 60% (range, 47-71%) [13] and median survival of asymptomatic PEH patients was 180 months [5]. The prognostic factors of PEH have not yet been established either.…”

“…Several studies reported that lack of these symptoms was associated with a lower risk for cancer progression [5,12].…”

“…Surgery is the approach of choice, and is indicated by the presence of a single pulmonary nodule, or multiple unilateral nodules when the number of lesions is limited [10]. Various chemotherapies have been reported and certain patients with bilateral multiple nodules may benefit from chemotherapy and immunotherapy [5,8]. Because PEH is a vascular tumor, the use of antiangiogenic therapy may be suggested.…”

“…Several studies have reported various prognostic factors. Some prognostic factors of PEH such as hemorrhagic symptoms (pleural effusion, alveolar hemorrhage, anemia, and hemoptysis) can reveal markers of vascular aggressiveness [5,12].…”