Clinical benefit of paclitaxel/carboplatin plus bevacizumab with zoledronic acid in pulmonary epithelioid hemangioendothelioma complicated by hypertrophic pulmonary osteoarthropathy and cardiac tamponade: a case report.

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm lacking a standard systemic therapy. Pulmonary EHE (PEH) may be aggressive if accompanied by serosal effusion and has occasionally been reported in association with hypertrophic pulmonary osteoarthropathy (HPOA). Here, we report a case of a 33-year-old woman who had never smoked and presented with chronic cough, arthralgia, and digital clubbing. Imaging revealed a left upper lobe mass with mediastinal invasion, lymphadenopathy, and pericardial effusion. The patient developed cardiac tamponade that required emergent pericardial drainage. Histopathology from the transbronchial biopsy and mediastinal lymph node sampling showed an epithelioid endothelial tumor positive for CD31, D2-40, factor VIII, and ERG with nuclear CAMTA1 expression, confirming EHE. Bone scintigraphy and ankle magnetic resonance imaging revealed symmetric periosteal-predominant abnormalities consistent with HPOA. Zoledronic acid was administered for HPOA, and paclitaxel/carboplatin plus bevacizumab was initiated with palliative intent. Follow-up imaging during treatment showed marked reduction of the pericardial and pleural effusions, with improvement in arthralgia and activities of daily living. Although the benefit was transient, this case suggests that systemic chemotherapy in combination with bevacizumab may have contributed not only to temporary effusion control in advanced PEH but also to relief of PEH-associated HPOA, while concomitant zoledronic acid may also have supported symptom improvement.