International Journal of Innovative Research in Medical Science (IJIRMS) Volume 05, Issue 02, February 2020, https://doi.org/10.23958/ijirms/vol05-i02/828 www.ijirms.in 84 Case report Pazopanib in Pulmonary Epithelioid Hemangioendothelioma: Report of a Case Ibtihal Ahalli 1 , Karima Oualla 1 , Loubna Hejjane 1 , Raihana Boujarnija 1 , Kaouthar Messoudi 1 , Adil Mai 2 , Fatima Zahrae Er Reggad 3 , Lamiaa Amaadour 1 , Zineb Benbrahim 1 , Samia Arifi 1 , Badr Alami 2 , Laila Chbani 3 , Nawfel Mellas 1 1 Department of Medical Oncology, Hassan II University Hospital, Fez, Morocco 2 Radiology Department, University Hospital Hassan II, Fez, Morocco 3 Anatomical Pathology and Cytology Laboratory, University Hospital Hassan II, Fez, Morocco Corresponding author: Ibtihal Ahalli; ahalli.ibtihal@gmail.com Received 31 December 2019; Accepted 25 January 2020; Published 21 February 2020 Abstract Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of borderline or low-grade malignancy, Vascular endothelial growth factor (VEGF) and the VEGF receptor were found on PEH tumor cells, suggesting that target therapies that block VEGFR have a logical base in this rare malignancy. We report a 25 years old woman with a pulmonary hemangioendothelioma, treated with pazopanib, the only anti angiogenic registered agent for sarcoma, with clinical improvement of symptoms and durable stabilization for more than two years of lung tumor. Introduction Pulmonary epithelioid hemangioendothelioma (EHE) is a rare disease, the prevalence of EHE is reported to be less than one in 1 million [1] . It was originally published the first time by Dail and Lie- bow in 1975, and it was taken as an intravascular bronchial- alveolar carcinoma [2] . In 1982 it was differentiated from bronchioalveolar carcinoma by Weiss and Enzinger [3,4] . It is a rare vascular tumor of low to intermediate malignancy. It affects preferably lung, liver and soft tissues. The usual presentation is incidental in middle-aged women who have single or multiple pulmonary nodules. The imaging diagnosis is complex and granulomatous or metastatic diseases should be excluded. Histology is similar to angiosarcoma and epithelioid sarcoma among others [9] . The electron microscopy techniques used by Weldon-Linne et al, as well as the immunohistochemical techniques by means of the detection of specific tumor markers for factor VIII and CD34, have facilitated the diagnosis [5] . The 2015 WHO Classification of Tumors of the Lung, Pleura, Thymus and Heart, has a significant change involving recognition of usefulness of WWTR1-CAMTA1 fusions in diagnosis of pulmonary EHE. The new information regarding this tumor is recognition of a translocation involving the WWTR1 and CAMTA1 genes and prognostic factors [6] . This update was based on the recent recognition that a recurrent t (1;3) (p36.3; q25) chromosomal translocation is characteristic of EH [7] . Prognosis remain unclear, although indicators of poor prognosis include the presence of respiratory symptoms, male gender, older age and multi-organ disease [1] . There is no standard treatment for PEH but curative resection is the preferred treatment option where possible, for metastatic or inoperable disease, the treatment options are not well established. The aim of this work is to report the rarity of pulmonary epithelioid hemangioendothelioma, describe the clinical, radiological, and pathological features, in addition to the therapeutic management. Case presentation A 25-year-old woman, history of celiac disease since childhood stabilized by a gluten-free diet, non-smoker, the main symptom was a sharp pain in the chest radiating to the left shoulder. The physical examination was normal. A chest CT scan revealed the presence of a lung mass in the parenchyma of the left superior lobe, with necrotic component, occluding the left bronchus, invading the left pulmonary artery 5mm from its origin in the trunk of the pulmonary artery, comes into contact with the arch of the aorta and the left superior pulmonary vein but without signs of invasion, and coexisting multiple nodules scattered in both lungs (Fig. 4). Abdomino-pelvic CT scan did not show distant metastases. Surgical lung biopsy which was performed thoracoscopically and histological examination of the nodules revealed an epithelioid cell with eosinophilic cytoplasm and intracytoplasmic vacuolization (Fig. 1,2).