Klinefelter Syndrome with Poor Risk Extragonadal Germ Cell Tumor

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• 作者：Jeremy A. Konheim ; ^{Jeremy.konheim@gmail.com} ; Jonathan A. Israel ; Scott E. Delacroix
• 关键词：Extragonadal germ cell tumor ; Klinefelter syndrome ; Growing teratoma syndrome ; Myelodysplasia
• 刊名：Urology Case Reports
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：10
• 期：Complete
• 页码：1-3
• 全文大小：734 K
• 卷排序：10

文摘

Germ cell tumors are the most common malignancy in men aged 15-35 years old, with a small percentage presenting in an extragonadal location. These tumors are seldom identified in the gastrointestinal tract. There is increased risk of extragonadal germ cell tumors (EGCT) in men with Klinefelter syndrome (KS). We report a rare case of a 37-year-old male with KS and EGCT discovered in the duodenum and pelvis. After treatment with Bleomycin-Etoposide-Cisplatin (BEP), he developed growing teratoma syndrome (GTS) and myelodysplasia. Despite surgical excision of the pelvic growing teratoma, he unfortunately died secondary to complications of severe bone marrow suppression.