2026年 · 第106卷第36期

The patient, a 50-year-old woman, visited the Department of Respiratory and Critical Care Medicine at the Affiliated Hospital of Hebei University on May 5, 2026, due to "cough and expectoration for more than half a month". There was no obvious cause of cough or expectoration, no fever, and no history of chronic diseases such as hypertension or diabetes in the first half of the month before admission. Physical examination: The breathing sounds in both lungs were rough, no dry or wet rales were heard, and the heart rhythm was uniform. The patient's outpatient chest plain CT scan showed multiple solid nodules of varying sizes in both lungs, suggesting a high possibility of metastasis. Upon admission, neuron-specific enolase was 27.60 μg/L (reference value: 0~16.30) and hemoglobin was 104 g/L (reference value: 115~150).¹⁸Fluorine-fluorodeoxyglucose positron emission computed tomography (¹⁸F-FDG PET/CT) showed soft tissue density mass shadows in the right ventricle, with a maximum cross-section of approximately 4.7 cm × 2.8 cm × 2.6 cm, a CT value of 26 HU, high metabolism, and a maximum standard uptake (SUVmax) of 21.8 (Figure 1, indicated by arrow); Multiple solid nodules of varying sizes were seen in both lungs, with high metabolism and SUVmax of 3.9–13.8 (Figure 2, indicated by arrows). The CT value of the right ventricular lesion after enhancement was 35 HU (Figure 3, indicated by arrows). CT-guided percutaneous pulmonary nodule biopsy was performed, and the pathology showed epithelioid hemangioendothelioma (as shown in Figure 4, HE staining, ×100). Immunohistochemistry: cytokeratin (CK) (partial+), Calretinin (-), CD31 (+), ETS-related gene (ERG) (+), S-100 (-), proliferating cell nuclear antigen (Ki-67) (15%+), CD68 (-), CD1a (-), thyroid transcription factor-1 (TTF-1) (-), P40 (-), integrase interacting protein 1 (INI-1) (+), Friend leukemia integration virus 1 (FLI-1) (+), CK7 (partial+). According to the imaging and pathological examination results, the diagnosis was cardiac and pulmonary epithelioid hemangioendothelioma (EHE). After 2 cycles of targeted combined chemotherapy, the lesions in the right ventricle and both lungs shrank slightly, and the efficacy was evaluated as stable disease (SD). EHE is a rare vascular tumor, and there are few reports of EHE involving the heart and lungs at the same time at home and abroad. Cardiac EHE is rare, most patients have no obvious symptoms, and it often occurs in the right atrium. The imaging manifestations are non-specific, the enhancement is mild, and PET/CT shows high metabolism. The confirmed diagnosis requires pathological examination. The typical manifestation of pulmonary EHE is multiple solid nodules of varying sizes in the lungs, mainly distributed in the middle and lower lung fields, with light to high metabolism. The treatment of EHE is mainly surgery, and cases that lose the opportunity for surgery can be treated with radiotherapy, chemotherapy or targeted therapy. The overall prognosis of EHE is poor, easy to recur and metastasize, and the survival time of patients with EHE involving the heart and lungs is significantly shortened. The postoperative survival time of cardiac tumors is 4 to 10 months.