Pulmonary epithelioid hemangioendothelioma

Abstract

We report two cases of pulmonary epithelioid hemangioendothelioma (PEH). Both patients presented with multiple bilateral pulmonary nodules, <10 mm diameter, on computed tomography (CT). Multiple pulmonary metastases were considered, but no primary malignant lesion was detected by other imaging modalities including (18)F-fl uorodeoxyglucose positron emission tomography ((18)F-FDG-PET)/CT. Moreover, the nodules did not show increased uptake of (18)F-FDG. We performed pulmonary wedge resections by video-assisted thoracoscopic surgery (VATS). Histological and immunohistochemical analysis revealed PEH in both. Positivity for the monoclonal antibody MIB-1 in the tumor cells was 5% in the fi rst case and 5%-10% in the second case. Slow tumor progression was detected with CT in the second case. Although (18)F-FDG PET/CT is effective for screening other malignant lesions, it does not appear to be of direct use in the diagnosis and surgical planning of PEH. Pathological diagnosis by VATS is the most effective method. MIB-1 positivity should be analyzed as to whether it is a prognostic factor of PEH.

New Insights about Pulmonary Epithelioid Hemangioendothelioma: Review of the Literature and Two Case Reports

“…Radiotherapy has proven to be ineffective for PEH because of the tumor's radiobiological characteristics, particularly the slow growth of tumor cells [ 37 ]. Surgery when tumor resection is feasible is usually considered the treatment of choice [ 80 , 81 ]. However, a complete surgical resection is usually not feasible.…”

“…However, a complete surgical resection is usually not feasible. Chemotherapy/immunostimulants in patients with disseminated disease and lung transplantation can also be an option [ 28 , 58 , 59 , 61 , 81 – 85 ]. Although there are a small number of reports about the effectiveness of chemotherapy, a general standard of chemotherapy is usually accepted for PEH [ 8 ].…”

Pulmonary Epithelioid Hemangioendothelioma in a Patient with Crohn’s Disease

“…It is more often diagnosed in women and in younger patients, with a median age of 35 years [ 6 – 8 ]. Imaging is not sufficient for diagnosis, but lesions often appear as either unilateral or bilateral perivascular nodules generally <2 cm in diameter [ 6 , 9 ]. Histological characteristics include nodules with hypocellular sclerotic or necrotic center.…”

Watch the Mime Carefully! A Refractory Interstitial Lung Disease

“…PEH usually affects young/middle-aged people, and its incidence is two times higher in women than in men and its prognosis is unpredictable [ 3 ]. Patients can remain stable for a long time or experience rapid disease progression until exitus, and the life expectancy may range from 1 to 30 years [ 4 ]. PEH is mainly incidentally discovered in young women, and some patients may present symptoms such as chest pain, pleuritic pain, cough, dyspnea, or rarely hemoptysis [ 5 ].…”