Klinefelter Syndrome with Poor Risk Extragonadal Germ Cell Tumor
نویسندگان
چکیده
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.
منابع مشابه
Klinefelter Syndrome and medical treatment: hypogonadism and beyond.
Klinefelter syndrome (KS), though described more than 70 years ago, still imposes significant diagnostic challenges. Based on data from epidemiological studies, KS is associated with increased morbidity and mortality. Although the pathophysiology and etiology behind these observations are as yet not well understood, a significant contribution of hypogonadism, central to the syndrome, is traditi...
متن کاملDose-intensive chemotherapy with syngeneic peripheral blood stem cell support for poor risk germ cell tumor of extragonadal origin: a case report.
A variety of regimens of high-dose chemotherapy with hematopoietic stem cell support for poor risk germ cell tumors have been established. However, a series of chemotherapy steps carried out prior to the harvest sometimes leads to an insufficient number of peripheral blood stem cells. Here, we report a case of a patient who successfully underwent high-dose chemotherapy for the treatment of poor...
متن کاملRecovery from Choriocarcinoma Syndrome Associated with a Metastatic Extragonadal Germ Cell Tumor Hemorrhage
A germ cell tumor is the most common form of malignancy in early male life, and can be classified as either seminomatous or nonseminomatous. Choriocarcinoma, comprised of nonseminomatous germ cells, is the most aggressive type of germ cell tumor and characteristically metastasizes to the retroperitoneal lymph nodes and less frequently to the lungs, liver, bone or brain [Shibuya et al., 2009;48:...
متن کاملShould extragonadal germ cell tumors be included in studies of families with testicular germ cell tumors?
BACKGROUND Family history is among the few established risk factors for testicular germ cell tumor (TGCT). Approximately 1.4% of newly diagnosed TGCT patients report a positive family history of TGCT. Sons and siblings of TGCT patients have four- to six fold and eight- to tenfold increase in TGCT risk, respectively. In twins of men with TGCT the relative risk of testicular cancer is 37.5 (12.3-...
متن کاملGlioblastoma multiforme associated with klinefelter syndrome.
A 54-year-old man with Klinefelter syndrome presented with glioblastoma multiforme manifesting as a 2-week history of motor weakness of the bilateral extremities. Magnetic resonance imaging showed multiple heterogeneously enhanced tumors in the bilateral frontal lobes. Angiography showed no tumor stain or arteriovenous shunt. The tumor was partially removed through a right craniotomy. The histo...
متن کامل