BJU International 2001 87 (9), 903

R. Takazawa, S. Kawakami, Y. Kageyama, K. Kihara and H. Oshima

Department of Urology and Reproductive Medicine, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan

Comment 

References 

Authors 

A 19-year-old man presented with a 1-month history of appetite loss and pollakiuria. His external genitalia were normal except for an apparently absent left testis; a right testis of 4 × 2 × 2 cm was normally positioned. A hard, fixed mass was palpated bimanually in the midline of the lower abdomen. Ultrasonography and CT showed a 10 cm mass which occupied the pelvic cavity, compressed the urinary bladder and bilateral lower ureters, and caused bilateral hydronephro-ureter. Blood examination revealed elevated serum creatinine (16 mg/L) and LDH (604 U/L) levels. His karyotype was 46,XY. Bilateral nephrostomies were placed and the patient's renal function returned to normal; a needle biopsy of the tumour revealed pure seminoma. No distant metastasis was detected. Two courses of systemic chemotherapy (cisplatinum 25 mg and etoposide 150 mg, 5 days) and external irradiation to the tumour (28 Gy in 14 fractions over 24 days) led to an 80% reduction in tumour size and rapid normalization of the elevated serum LDH levels. CT and MRI showed remaining soft-tissue structure continuing from the tumour to the right inguinal canal. At laparotomy, the uterus, vagina and left Fallopian tube were herniated into the right inguinal canal. The right Fallopian tube was not recognisable (Fig. 1). The left round ligament was attached to the back of the right pubic bone. The tumour located in the recto-uterine pouch was resected en bloc together with the left Fallopian tube and the uterus, which was severed at the cervix, preserving the right seminiferous vas which opened at the fornix of the vagina. Pathologically, no cancer cells were found in the tumour. Well-developed left seminal tract and dysgenetic testicular tissue were identified (Fig. 2). Six-months after surgery his serum testosterone level was within normal limits, while LH and FSH levels remained high. Semen analysis showed azoospermia. The patient had no evidence of disease after more than a year of follow-up.

Case report 

References 

Authors 

Mixed gonadal dysgenesis (MGD) is characterized by asymmetric gonadal development, with a testis on one side and a streak gonad, gonadal tumour, or none at all on the other side. Müllerian structures (Fallopian tube, uterus and vagina) are always present because of insufficient anti-Müllerian hormone production by the malformed gonad [1]. The present patient had a testis and seminoma with persistent Müllerian structures, compatible with MGD. The Müllerian structures herniated into the contralateral inguinal canal, which mimicked so-called uterine inguinal hernia [2]. The patient was raised as a male and had a normal adolescence, suggesting that testicular hormone production had always been satisfactory, as the endocrinological analyses confirmed. As the testis of an adult with MGD usually shows no advanced stages of spermatogenesis, azoospermia might not be caused by the gonotoxic effect of the chemotherapeutic agents in the present case. Undescended malformed gonads in MGD are highly susceptible to developing various types of malignant tumours. To the best of our knowledge, only one case of seminoma has been reported previously [3]. The successful treatment of the present patient indicates that seminoma arising from a malformed gonad in MGD responds as well to the standard therapies as that from the testis.

Case report 

Comment 

Authors 

Case report 

Comment 

R. Takazawa, MD, Urologist.

S. Kawakami, MD, Urologist.

Y. Kageyama, MD, PhD, Associate Professor.

K. Kihara, MD, PhD, Professor & Chairman.

H. Oshima, MD, PhD, Professor Emeritus.