Endodermal sinus tumors are the second most common germ cell malignancy, representing 20% of these tumors. They manifest with increased abdominal volume, pain, torsion or rupture of the tumor. They are usually unilateral, large size and manifest with an elevation of alpha-fetus protein (AFP) between 20 and 1000 ng/ml. They show extremely fast growth. Advanced stages have higher recurrence rates. Survival for these types of tumors increased significantly with the use of chemotherapy regimens.
The case presents a young woman with advanced disease, whose first manifestation was thrombosis of the right jugular, subclavian and axillary veins.
Twenty–year–old Caucasian healthy woman hospitalized due to cervical pain and right upper limb edema, diagnosed with right jugular, subclavian and axillary vein thrombosis. CT images showed bilateral pleural effusion, massive ascites, a large, expansive solid cystic lesion in the pelvic cavity and multiple solid peritoneal nodules. Tumor markers showed negative Beta HCG, AFP 1119.3 ng/ml, CA 125 284.0 IU/ml, CA19–9 10UI/ml and CEA 1.0 ng/ml. She underwent radical surgery and drained eight liters of ascites. Peritoneal carcinomatosis and a solid lesion in the left ovary measuring 17 × 16 × 9 cm were evidenced. Anatomic pathological result: malignant neoplasm suggestive of a yolk sac tumor of the left ovary compromising the sac fundus, diaphragmatic dome, parietal, pelvic and vesical peritoneum, subhepatic nodule, cecal appendix and two of the 67 isolated lymph nodes. Positive ascitic fluid for malignancy. FIGO T3N1M1 staging. Immunohistochemistry compatible with endodermal sinus tumor. Five cycles of BEPx3–4 were performed. AFP after chemotherapy 2.8 ng/ml. Imaging exams without evidence of disease.
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