Multiple organ metastases of pulmonary epithelioid haemangioendothelioma and a review of the literature

Abstract

This report describes a case of pulmonary epithelioid haemangioendothelioma presented as multi-organ metastases including lung, liver and bone in a 56-year-old woman with an initial diagnosis made with thoracoscopic wedge biopsy. The diagnosis is confirmed through immunohistochemistry. This is a rare disease, with approximately 90 cases described in the English literature and approximately 10 cases reported in China. The case of PEH presented as multiple pulmonary nodules and metastasing to liver and bone is rare in the English literature. The rarity of this condition, the lack of clear standards for treatment, and the partial-to-complete spontaneous regression of EHE seen in some patients up to 15 years from initial detection makes it difficult to decide on the most appropriate treatment. This report may contribute to the data on clinical findings and natural history of this rare tumor.

Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules

“…Epithelioid hemangioendothelioma can arise from many organs, including lungs, liver, bone, and soft tissue, simultaneously or sequentially. When this occurs, it may be difficult to determinate if the tumor is multicentric from the beginning or there is a primary lesion with metastases to the other tissues [4]. Around 120 cases have been reported in literature [2, Table 2], only five of them were in Italian patients [5, 15–18].…”

“…The diagnosis of PEH is made on the basis of the histopathological features and confirmed using immunohistochemical staining for endothelial cell markers, such as against factor VIII-related von Willebrand antigen, CD31 or CD34 [2, 4, 13, 20, 21]. …”

Pulmonary epithelioid hemangioendothelioma misconceived as pulmonary metastasis of other malignancies

“…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.…”

Lung lobar collapse as the first manifestation of pulmonary epithelioid haemangioendothelioma diagnosed with fibreoptic bronchoscopy

“…According to a December 2008 literature review of 221 reported cases, the median age at the time of diagnosis is 41 years, and 66% are females 14. Recently, there was an increase in the prevalence of PEH, likely due to improvement in diagnostic competencies 15…”

“…CD31 has been used to confirm the diagnosis of vascular tumours, as it is found in neoplasms developed from endothelial cells 3 14. Biopsies are usually obtained through thoracoscopy.…”