Pulmonary epithelioid hemangioendothelioma presenting with vertebral metastases: a case report

Abstract

IntroductionEpithelioid hemangioendothelioma is a rare vascular tumor that has an epithelioid and histiocytoid appearance, originates from vascular endothelial or pre-endothelial cells and comprises less than 1% of all vascular tumors. It was described for the first time in 1975 as pulmonary epithelioid hemangioendothelioma, because initially it was believed to be an aggressive form of bronchoalveolar cell carcinoma with a remarkable propensity to invade adjacent blood vessels and small airways. Only a few cases have been reported in the literature to date. Tumor cells expressing Fli-1 and CD31 have been identified as relatively specific endothelial markers. Epithelioid hemangioendothelioma may affect multiple organs and may vary considerably in its clinical and radiological presentation. More than 50% to 76% of pulmonary epithelioid hemangioendothelioma patients are asymptomatic. They are usually incidentally diagnosed on the basis of abnormal chest radiography during routine physical examinations. Hematologic and gastrointestinal disorders and weakness or numbness may also be observed, in addition to respiratory symptoms, in cases of disseminated pulmonary epithelioid hemangioendothelioma. Pain and swelling, pathological fractures, spine compression or paresthesia, loss of muscular strength and paraplegia may be present when bone metastases occur. Because of the rarity of this disease, there is no standard for treatment.Case presentationA 46-year-old Caucasian woman presented to our institution in November 2009 with metastases of pulmonary epithelioid hemangioendothelioma from the L3 and L4 vertebrae. A course of radiotherapy at a dosage of 3,000cGy delivered in individual doses of 200cGy/day for 5 days/wk to the L3 and L4 vertebrae led to the disappearance of the patient’s lumbar pain without any detectable side effects. Percussion of the patient’s vertebral spine was negative, and no radiological progression of bone disease was found at her 1-year follow-up examination.ConclusionSince epithelioid hemangioendothelioma was first correctly defined, several research groups have reported their experiences with epithelioid hemangioendothelioma irradiation. Further studies are needed to establish a standard radiation dose to be used for such a complex and extremely rare disease. In our present case, a radiotherapy dosage of 3,000cGy delivered in individual doses 200cGy/day for 5 days/wk allowed us to reach our goals: local pain control with good tolerance and better quality of life by the 1-year follow-up examination.

Metastatic epithelioid haemangioendothelioma masquerading as periodontal pathosis: a case report

“…Oral epithelioid haemangioendothelioma is a rare neoplasm that can mimic traumatic ulceration, pyogenic granuloma, and fibroepithelial polyp. Its estimated prevalence is less than one in a million, 1 and available data is scarce. Epidemiology, presentation, and clinical course vary, which reinforces the need for careful inspection of such lesions.…”

Epithelioid hemangioendothelioma of the spine: case report and review of the literature

“…Interferon 2α or carboplatin plus etoposide have been used for patients with widespread disease but the evidence is not clear (28). No definitive conclusions about the use of chemotherapy and radiotherapy can be made until larger studies can be analyzed.…”

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

“…A 6400 cGy adjuvant RT was also performed against an axillary form of EHE, resulting in the absence of lymph nodes metastases but pleural and pulmonary widespread [18]. In our experience, at our Institute we observed systemic progression of EHE after 4-month-PEG-IFN-α therapy in spite of stable pulmonary disease; then, lumbar pain control with good tolerance and better quality of life at 1-year-follow-up from a symptomatic, normofractionated RT onto the L3-L5 vertebral tract, consolidated outcome through Ifosfamide and Epirubicin chemotherapy soon after RT, and surgical removal of EHE spleen lesions, showing 1-year survival [1].…”

“…A 6400 cGy adjuvant RT was also performed against an axillary form of EHE, resulting in the absence of lymph nodes metastases but pleural and pulmonary widespread [18]. In our experience, at our Institute we observed systemic progression of EHE after 4-month-PEG-IFN-α therapy in spite of stable pulmonary disease; then, lumbar pain control with good tolerance and better quality of life at 1-year-follow-up from a symptomatic, normofractionated RT onto the L3-L5 vertebral tract, consolidated outcome through Ifosfamide and Epirubicin chemotherapy soon after RT, and surgical removal of EHE spleen lesions, showing 1-year survival [1].All this considered, despite the rarity of this vascular tumor and its extremely complex and heterogeneous patterns of presentation, actually we are capable of performing more treatment lines, combined in a multimodal strategy or sequencially adopted, so that we are able to reach good outcomes in most of cases. In this regard, taking into account the EHE radiobiological characteristics, no doubt evidence is all in favour of RT obtaining local pain control with good tolerance and better quality of life at least one-year-follow up.…”

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IntroductionWe have already discussed about dail and liebow describing pulmonary epithelioid hemangioendothelioma (PEH) as an aggressive bronchoalveolar cell carcinoma with a remarkable propensity to invade adjacent blood vessels and small airways, the following Weiss and Enzinger description of epithelioid hemangioendothelioma (EHE) as a vascular bone and soft tissues tumor showing intermediate malignancy between hemangioma and angiosarcoma, and the final confirmation from Weldon-Linne of a factor-VIII-related antigen onto malignant cells [1]. The recent World Health Organization (WHO 2002) classification has described EHE as lesions that fall into the category of locally aggressive tumors with metastatic potential [2,3].

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