A 30-year-old woman with primary cardiac angiosarcoma and pulmonary metastases initially misdiagnosed as a granulomatous process died 11 months after presentation despite combination chemotherapy.
Case Report (n=1)
No
Metastatic pulmonary angiosarcoma from a primary cardiac source is highly heterogeneous and challenging to diagnose, often requiring adequate surgical tissue sampling for definitive histopathology.
Abstract Angiosarcoma is a malignant soft tissue sarcoma of endothelial cell origin that arises from blood vessels or lymphatic vessels, and the lung is the most frequent site of metastasis. Metastatic pulmonary angiosarcoma exhibits highly heterogeneous clinical, imaging, and pathological features, with an extremely poor prognosis. A 30-year-old woman presented to our hospital with a 3-month history of hemoptysis and a 3-day history of nausea and vomiting. Imaging showed multiple bilateral pulmonary nodules and consolidations with associated hypermetabolism in these nodules, mediastinal lymphadenopathy, bilateral pleural effusions, and a large pericardial effusion. Pericardiocentesis yielded hemorrhagic fluid with elevated tumor markers (CA125, CYFRA 21-1, NSE) but negative cytology. CT-guided core biopsy revealed focal necrosis surrounded by hyperchromatic spindle and epithelioid cells. Immunoprofile: CD20 and CD3 (scattered+), CD56(-), CD68/KP1 (histiocytes+), pan-CK(+), EBER(-), Granzyme B(-), Ki-67 (scattered+), Perforin(+), TIA-1 (rare+), CD31(+), CD4/8 (subset+), IgG(+), IgG4(-), MUM-1(-); special stains for organisms negative. Based on the integration of prior clinical datas, initial pathological assessment suggested a granulomatous process. To establish a definitive diagnosis, the patient proceeded to video-assisted thoracoscopic surgery for wedge resection of the lung and pericardial window formation. Pathology revealed multifocal granulomatoid lesions in the left upper lobe, with perivascular growth and vascular endothelial hyperplasia. Further assessment confirmed a multifocal mesenchymal malignancy with necrosis and angio-centric features. Immunohistochemistry was positive for CD31, CD34, ERG, and F8; Smooth muscle actin showed focal positivity in tumor cells. The Ki-67 proliferation index was approximately 50%. The morphological and immunohistochemical findings were consistent with a final diagnosis of multifocal angiosarcoma. Echocardiography revealed a solid mass adjacent to the right atrial wall near the atrioventricular groove. The final diagnosis was primary cardiac angiosarcoma with multifocal pulmonary metastases. The patient received two cycles of combination chemotherapy with albumin-bound paclitaxel, gemcitabine and Endostar, and the patient’s hemoptysis improved with stable follow-up echocardiography. Based on telephone follow-up, the patient did not receive further chemotherapy and died 11 months after initial presentation. Twenty-two cases of metastatic pulmonary angiosarcoma from the literature were reviewed. For cases with recurrent hemoptysis, multiple pulmonary nodules or thin-walled cysts, and “halo sign” or ground-glass opacities, metastatic pulmonary angiosarcoma should be highly suspected. Histopathology remains the cornerstone for a definitive diagnosis. However, diagnostic confirmation can be challenging with early-stage lesions or small biopsy specimens due to sampling limitations and tumor heterogeneity. Obtaining an adequate tissue sample, for instance via surgical resection, is crucial for enhancing diagnostic accuracy. This abstract is funded by: This study was supported by China Postdoctoral Science Foundation (Grant Numbers: 2022M720778, 2024T170162, 2024M750545), National Natural Science Foundation of China (Grant Numbers: 82130001, 82200089, 82203517), Excellent Postdoctoral Foundation of Zhongshan Hospital, Fudan University (Grant Numbers: 2023ZYYS-006, 2023ZYYS-008), Shanghai Science and Technology Committee Foundation (Grant Number: 20DZ2261200), Scientific Research Development Fund of Zhongshan Hospital, Fudan University (for Discipline Construction, Grant Number: 2024XKPT23-RC4), National Key Research and Development Program of China (Grant Number: 2024ZD0529704).
Mao et al. (Fri,) conducted a case report in Primary cardiac angiosarcoma with multifocal pulmonary metastases (n=1). Combination chemotherapy (albumin-bound paclitaxel, gemcitabine, Endostar) was evaluated. A 30-year-old woman with primary cardiac angiosarcoma and pulmonary metastases initially misdiagnosed as a granulomatous process died 11 months after presentation despite combination chemotherapy.