Case report reveals a rare angiosarcoma diagnosis in a 34-year-old female, highlighting the challenges of early detection.
Angiosarcoma is a rare and aggressive malignant tumor originating from endothelial cells lining blood vessels and lymphatic channels. While most commonly arising in the skin and soft tissues, it can metastasize to multiple organs, with the lungs being the most frequent site. Primary ovarian angiosarcoma is exceedingly rare, comprising less than 1% of ovarian malignancies and complicating diagnosis. The case below describes an extensive evaluation initially aimed at identifying a benign tumor syndrome, which ultimately revealed angiosarcoma.A 34-year-old female with a history of cesarean section and ovarian cystectomy for a benign dermoid cyst presented with intractable nausea and vomiting for one day. She reported dyspnea on exertion and a productive cough with yellow sputum. Chest X-ray and CT scan revealed a large right-sided and small left-sided pleural effusion. Thoracentesis removed 1.5 L of exudative, serosanguinous fluid. Repeat CT chest, abdomen and pelvis due to persistent nausea, vomiting, and dyspnea showed reaccumulation of pleural effusions, ascites, and a 6.4 × 8.2 cm predominantly soft-tissue lesion in the cul-de-sac, suggestive of malignant transformation of a mature cystic teratoma or liposarcoma. Tumor markers, including carcinoembryonic antigen and cancer antigen 125, were elevated. Given the imaging findings and persistent thoracic abnormalities, it was unclear whether the pleural effusions were secondary to a subdiaphragmatic process. A chest tube was placed, and the patient was monitored for possible PleurX catheter placement.Gynecologic oncology performed an exploratory laparotomy with excisional biopsy of an 8 cm irregular, friable white-tan mass from the left adnexa. The chest tube output decreased and was removed. Considering the triad of ovarian tumor, ascites, and pleural effusion—with effusion resolution after mass removal and negative cytology for malignant cells—Meigs’ syndrome was initially favored. However, days later she had re-accumulation of pleural effusions, underwent video-assisted thoracoscopic surgery, and had a PleurX catheter placed. Cytopathology from the pleural biopsy ultimately revealed angiosarcoma.The etiology of angiosarcoma remains unclear, though chronic lymphedema, radiation exposure, and environmental carcinogens are associated risk factors. Owing to its rapid progression and high metastatic potential, early diagnosis and prompt multimodal treatment—surgical resection, radiation therapy, and chemotherapy—are critical. Despite aggressive management, prognosis remains poor due to the tumor’s highly malignant nature. This abstract is funded by: None
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