Case Reports: Primary Yolk Sac Tumor of the Prostate in a Patient With Klinefelter's Syndrome

Primary yolk sac (endodermal sinus) tumor of the prostate is extremely rare with only 2 cases reported in the literature. We describe a case of primary yolk sac tumor of the prostate in a man with Klinefelter's syndrome. Treatment included 4 courses of combination chemotherapy followed by retroperitoneal lymph node dissection, cystoprostatectomy and ileal conduit urinary diversion. We review the association of Klinefelter's syndrome with extragonadal germ cell tumor along with the management of this rare disease. The existence and pathogenesis of extragonadal germ cell tumor have been well established since the first report in 1927 by Prym.' Lachman et al reported a 30 to 40 times greater incidence of Klinefelter's syndrome among patients with mediastinal germ cell tumors than the incidence of Klinefelter's syndrome in the general population.' Yolk sac (endodermal sinus) tumor is a rare type of extragonadal germ cell tumor that was first recognized in 1959 by Teilum? Two cases of primary yolk sac tumor of the prostate gland have been described in the To our knowledge we report the first case of primary yolk sac tumor of the prostate in a patient with Klinefelter's syndrome. CASE REPORT