Teaching File Case - Patient: 10556Peer Reviewed and Certified - Approved by: Stephanie A Bernard - 2007-01-22 21:53:06-05
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Malignant Germ Cell Tumor (mixed) The anterior mediastinum can harbor primary germ cell neoplasms as well as germ cell metastases from primary testicular tumors. This patient had a subsequent scrotal ultrasound (images not available) showing coarse testicular calcifications. This could be a serendipitous finding or represent a “burnt–out” testicular neoplasm.
PET CT evaluation of the patient showed abnormal FDG activity only in the mediastinum. For now, this case is being approached as a primary mediastinal mixed germ cell tumor.
The peak occurrence is age 20-40 predominantly in males (M:F = 9:1). When these tumors occur in children they are equally distributed among males/females. They are associated with Klinefelter syndrome (47 XXY chromosomal abnormality) in 20% of patients. In Klinefelter syndrome, they are typically found in young boys with precocious puberty.
Presenting symptoms include chest pain, dyspnea, cough, weight loss, and fever. In 90%+ of patients there are symptoms of superior vena cava syndrome. Laboratory analysis typically demonstrates elevated serum levels of LDH (60%), hCG (30%), and alpha-fetoprotein (80%).
Treatment is based on cisplatin-based chemotherapy and tumor resection when possible with a long-term survival approaching 50%.
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