Klin Onkol 2021; 34(3): 235-240. DOI: 10.48095/ccko2021235.
Background: At present, lung cancer ranks among the most frequent malignant diseases. However, according to literature data, mesenchymal lung tumors are very rare, representing less than 0.5% of all malignant lung tumours. Epithelioid hemangioendothelioma of the lungs belongs to this group of uncommon entities. Case report: We present a case of a 45-year-old male with a history of increasing dyspnoea and abdominal and back pains, developing over the past several months. Vertebral lesions were found on imaging studies. PET/ CT following 18F-fluorodeoxyglucose administration (FDG) showed a large FDG-positive malignant infiltration affecting the thorax, abdominal cavity, and bones. The extension and other characteristics of the mass on PET/ CT did not correspond to any of the common oncologic diseases. With its spread in a plaque-like form predominantly in the right hemithorax and on the surface of the liver, the disease closely resembled malignant mesothelioma. The primary tumour origin could not be clearly identified on PET/ CT scans but they allowed us to choose a suitable site to obtain tissue for pathologic examination. Based on a CT-guided bone biopsy of the 7th right rib, the diagnosis was concluded as epithelioid hemangioendothelioma. Despite an early initiation of systemic treatment, the patient succumbed to the disease only 15 days after the diagnosis, due to superior vena cava syndrome and progressive pleural effusion leading to respiratory insufficiency. Conclusions: Given the extremely low prevalence of epithelioid hemangioendothelioma and its heterogeneous manifestation, it is impossible to base the diagnosis solely on disease symptoms, laboratory findings, and imaging modalities. In this respect, pathologic examination has a crucial role. For the same reason, there is a lack of recommendations for the standard-of-care systemic therapy of metastatic disease.
