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A Case of Polyorchidism with Testicular Teratoma
0022-5347 /80/1246-0930$02.00/0 Vol. 124, December Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1980 by The Williams & Wilkins Co.
A CASE OF POLYORCHIDISM WITH TESTICULAR TERATOMA K. W. M. SCOTT From the Department of Pathology, The Royal Hospital, Wolverhampton, England
ABSTRACT
A patient with duplication of the testis who presented initially with torsion of a right inguinal testis is described. Subsequently, a malignant teratoma of the trophoblastic type (choriocarcinoma) developed in the scrotal testis on the right side. This scrotal testis was impalpable initially but a normal testis was present on the left side. The patient died of cerebral and pulmonary metastases of the teratoma. ously in the literature 1 and it is known that 1 or both of the duplicated testes may be undescended. 2 Torsion of the smaller testis, as in this case, has been described. 3 It is also known that each of the duplicated testes may be small, which presumably explains why the second testis was not palpable on the right side of the scrotum when this patient presented initially. To the best of my knowledge, no case of testicular tumor associated with duplication of the testis has been reported previously. It has long been recognized that testicular tumors, especially seminoma, are more common in patients with an ectopic testis. There is a 20 to 40-fold increase in the incidence of testicular malignancy in patients with cryptorchidism compared to those with normal testes. 4 Several authors have shown that orchiopexy does not always prevent malignant change in a cryptorchid testis. 5- 8 Johnson observed that 20 per cent of the cryptorchidassociated testicular malignancies arose in the normally descended contralateral scrotal testis. 9 Gehring and associates found 29 patients with unilateral cryptorchidism in a series of 529 testicular malignancies. 4 Of these 29 patients 22 had ipsilateral and 7 (24 per cent) had contralateral tumors. In 3 of their patients who had had orchiectomy 1 teratoma and 2 seminomas developed subsequently in the contralateral testis. The unusual feature in the present case is that the teratoma arose neither in the cryptorchid testis nor in the contralateral normally descended testis but in an ipsilateral reduplicated testis. Sohval proposed that testicular dysgenesis in cryptorchid testes may account for the increased susceptibility of such testes to malignant change. 10 Johnson postulated that dysgenetic areas in the contralateral normally descended testis may explain the increased incidence of malignancy in that testis. 9 As with ectopic testes, duplicated testes are thought to have dysplastic or undifferentiated testicular tissue within them, which may be the site of origin of any subsequent tumor. In the present case the tumor arose in the testis that was presumed to be intrascrotal throughout life, which is noteworthy with regard to the theories of etiology of testicular neoplasms. Willis stated that ectopia is not necessarily the direct cause of the tumor formation but probably only 1 expression of a gonadal or embryonic anomaly predisposing to development of a subsequent tumor. 11 The present unusual case wouldtend to support this theory. It also is noteworthy that the patient had a hernia as well as a cryptorchid testis on the right side. In their series of 529 testicular tumors Gehring and associates found that 19 patients (3.52 per cent) had had previous hemiorrhaphies. 4 The tumors that subsequently developed were ipsilateral in 12 patients, contralateral in 2 and bilateral in 5. The fact that the tumor in the present case subsequently developed on the same side as the hernia but in a descended testis is more in keeping with the suggestion of Sohval 10 that the hernia is a manifestation of the dysgenesis rather than the theory of Johnson9 that trauma, as may occur at herniorrhaphy, may predispose to the develop-
An 11-year-old mentally subnormal boy presented with a painful swelling in the right groin of recent onset. No testis could be palpated in the scrotum on the right side but the left testis was noted to be in the normal position. Exploration of the right groin revealed a testis in the superficial inguinal pouch, which had undergone torsion, and an associated hernial sac. Orchiectomy and hemiorrhaphy were performed. Convalescence was uneventful. The excised testis was 2.5 cm. and histology showed an immature testis and epididymis, which exhibited early hemorrhagic infarction (fig. 1). The patient was rehospitalized when he was 18 years old. He had collapsed and was stuperous but conscious. Headache, violent limb movements and a mute state developed 24 hours before hospitalization. On examination the eyes deviated to the right side and both plantar responses were upgoing. The scrotum was swollen on the right side, and there were irregular masses palpable in the mid abdomen and crepitations over the left mid zone of the chest. A chest x-ray showed an opacity in the left mid lung zone, ultrasonography of the abdomen revealed para-aortic masses and an electroencephalogram demonstrated a possible cerebral lesion on the right side. The patient died suddenly before further procedures could be performed. At autopsy a 10 cm. mass was confirmed in the right side of the scrotum and there was a healed scar in the right inguinal region. The scrotal swelling was owing to an 8 cm. tumor, apparently in the right testis and almost completely replacing it (fig. 2). A compressed rim of testicular tissue was present at 1 pole and the spermatic cord was attached to the mass. The left testis was in the scrotum and, although it appeared rather small (2.5 cm. in diameter), it was otherwise normal. Numerous enlarged para-aortic lymph nodes were present, a 6 cm. tumor was seen in the middle zone of the left lung and the brain showed 4 separate tumor deposits, each 2 to 3 cm. There was extensive hemorrhage in 1 of the tumor deposits. Histological examination of the tissues showed a malignant teratoma of the trophoblastic type (choriocarcinoma) in the right testis (fig. 3). Syncytioblastic and cytotrophoblastic elements were present, and secondary deposits of a similar tumor were confirmed in the para-aortic lymph nodes, left lung and brain. In the compressed testicular tissue around the tumor on the right side there were recognizable seminiferous tubules with spermatogenesis (fig. 4). Histology of the left testis showed slightly reduced spermatogenesis, some fibrosis and mild Leydig cell hyperplasia. DISCUSSION
There have been 37 cases of polyorchidism reported previAccepted for publication March 21, 1980. 930
POLYORCHIDISM WITH TESTICULAR TERATOMA
FIG. 1. Photomicrograph of right inguinal testis shows immature seminiferous tubules surrounded by red blood cells and fibrin owing to infarction. H & E, reduced from X32.
931
FIG. 3. Photomicrograph of testicular tumor. Tumor cells are pleomorphic and giant cells form ill-defined villous as seen in trophoblastic teratoma (choriocarcinoma). H & E, reduced from X80.
FIG. 4. Photomicrograph of surviving rim of testicular tissue around teratoma. Seminiferous tubule can be seen containing cells at all stages of spermatogenesis, including spermatozoa. There is some fibrosis in surrounding tissues. H & E, reduced from X80. FIG. 2. Right testis with tumor bisected. Compressed rim of testicular tissue can be seen around top right of mass and spermatic cord is attached.
ment of a neoplasm. This case also is unusual in that the duplication occurred on the right side of the scrotum. Butz and Croushore stated that 21 of the previously described 36 cases were on the left side. I Also, spermatogenesis was present in the left testis and in the surviving rim of the testis on the right side around the tumor. Spermatogenesis was present in only 15 of the 36 cases described previously. I Mr. J. A. MacDougall assisted in the preparation of this paper. REFERENCE
1. Butz, R. E. and Croushore, J. H.: Polyorchidism. J. Urol., 119: 289, 1978. 2. Williams, D. I.: Paediatric Urology. London: Butterworth & Co., p. 462, 1968. 3. Wilson, W. A. and Littler, J.: Polyorchidism; report of 2 cases with
torsion. Brit. J. Surg., 41: 302, 1953. 4. Gehring, G. G., Rodriguez, F. R. and Woodhead, D. M.: Malignant degeneration of cryptorchid testis following orchiopexy. J. Urol., 112: 354, 1974. 5. Cunningham, J. H.: New growths developing in undescended testicles. J. Urol., 5: 471, 1921. 6. Sumner, W. A.: Malignant tumor of testis occurring 29 years after orchiopexy: case report and review of the literature. J. Urol., 81: 150, 1959. 7. Dow, J. A. and Mostofi, F. K.: Testicular tumors following orchiopexy. South. Med. J., 60: 193, 1967. 8. Johnson, D. E., Woodhead, D. M., Pohl, D.R. and Robison, J. R.: Cryptorchidism and testicular tumorigenesis. Surgery, 63: 919, 1968. 9. Johnson, D. E.: Testicular Tumors. New York: Medical Examination Publishing Co., Inc., pp. 34-35, 1972. 10. Sohval, A. R.: Testicular dysgenesis as an etiologic factor in cryptorchidism. J. Urol., 72: 693, 1954. 11. Willis, R. A.: Pathology of Tumours, 4th ed. London: Butterworth & Co., p. 570, 1967.
THE JOURNAL OF UROLOGY
Copyright© 1980 by The Williams & Wilkins Co.
A CASE OF POLYORCHIDISM WITH TESTICULAR TERATOMA K. W. M. SCOTT From the Department of Pathology, The Royal Hospital, Wolverhampton, England
ABSTRACT
A patient with duplication of the testis who presented initially with torsion of a right inguinal testis is described. Subsequently, a malignant teratoma of the trophoblastic type (choriocarcinoma) developed in the scrotal testis on the right side. This scrotal testis was impalpable initially but a normal testis was present on the left side. The patient died of cerebral and pulmonary metastases of the teratoma. ously in the literature 1 and it is known that 1 or both of the duplicated testes may be undescended. 2 Torsion of the smaller testis, as in this case, has been described. 3 It is also known that each of the duplicated testes may be small, which presumably explains why the second testis was not palpable on the right side of the scrotum when this patient presented initially. To the best of my knowledge, no case of testicular tumor associated with duplication of the testis has been reported previously. It has long been recognized that testicular tumors, especially seminoma, are more common in patients with an ectopic testis. There is a 20 to 40-fold increase in the incidence of testicular malignancy in patients with cryptorchidism compared to those with normal testes. 4 Several authors have shown that orchiopexy does not always prevent malignant change in a cryptorchid testis. 5- 8 Johnson observed that 20 per cent of the cryptorchidassociated testicular malignancies arose in the normally descended contralateral scrotal testis. 9 Gehring and associates found 29 patients with unilateral cryptorchidism in a series of 529 testicular malignancies. 4 Of these 29 patients 22 had ipsilateral and 7 (24 per cent) had contralateral tumors. In 3 of their patients who had had orchiectomy 1 teratoma and 2 seminomas developed subsequently in the contralateral testis. The unusual feature in the present case is that the teratoma arose neither in the cryptorchid testis nor in the contralateral normally descended testis but in an ipsilateral reduplicated testis. Sohval proposed that testicular dysgenesis in cryptorchid testes may account for the increased susceptibility of such testes to malignant change. 10 Johnson postulated that dysgenetic areas in the contralateral normally descended testis may explain the increased incidence of malignancy in that testis. 9 As with ectopic testes, duplicated testes are thought to have dysplastic or undifferentiated testicular tissue within them, which may be the site of origin of any subsequent tumor. In the present case the tumor arose in the testis that was presumed to be intrascrotal throughout life, which is noteworthy with regard to the theories of etiology of testicular neoplasms. Willis stated that ectopia is not necessarily the direct cause of the tumor formation but probably only 1 expression of a gonadal or embryonic anomaly predisposing to development of a subsequent tumor. 11 The present unusual case wouldtend to support this theory. It also is noteworthy that the patient had a hernia as well as a cryptorchid testis on the right side. In their series of 529 testicular tumors Gehring and associates found that 19 patients (3.52 per cent) had had previous hemiorrhaphies. 4 The tumors that subsequently developed were ipsilateral in 12 patients, contralateral in 2 and bilateral in 5. The fact that the tumor in the present case subsequently developed on the same side as the hernia but in a descended testis is more in keeping with the suggestion of Sohval 10 that the hernia is a manifestation of the dysgenesis rather than the theory of Johnson9 that trauma, as may occur at herniorrhaphy, may predispose to the develop-
An 11-year-old mentally subnormal boy presented with a painful swelling in the right groin of recent onset. No testis could be palpated in the scrotum on the right side but the left testis was noted to be in the normal position. Exploration of the right groin revealed a testis in the superficial inguinal pouch, which had undergone torsion, and an associated hernial sac. Orchiectomy and hemiorrhaphy were performed. Convalescence was uneventful. The excised testis was 2.5 cm. and histology showed an immature testis and epididymis, which exhibited early hemorrhagic infarction (fig. 1). The patient was rehospitalized when he was 18 years old. He had collapsed and was stuperous but conscious. Headache, violent limb movements and a mute state developed 24 hours before hospitalization. On examination the eyes deviated to the right side and both plantar responses were upgoing. The scrotum was swollen on the right side, and there were irregular masses palpable in the mid abdomen and crepitations over the left mid zone of the chest. A chest x-ray showed an opacity in the left mid lung zone, ultrasonography of the abdomen revealed para-aortic masses and an electroencephalogram demonstrated a possible cerebral lesion on the right side. The patient died suddenly before further procedures could be performed. At autopsy a 10 cm. mass was confirmed in the right side of the scrotum and there was a healed scar in the right inguinal region. The scrotal swelling was owing to an 8 cm. tumor, apparently in the right testis and almost completely replacing it (fig. 2). A compressed rim of testicular tissue was present at 1 pole and the spermatic cord was attached to the mass. The left testis was in the scrotum and, although it appeared rather small (2.5 cm. in diameter), it was otherwise normal. Numerous enlarged para-aortic lymph nodes were present, a 6 cm. tumor was seen in the middle zone of the left lung and the brain showed 4 separate tumor deposits, each 2 to 3 cm. There was extensive hemorrhage in 1 of the tumor deposits. Histological examination of the tissues showed a malignant teratoma of the trophoblastic type (choriocarcinoma) in the right testis (fig. 3). Syncytioblastic and cytotrophoblastic elements were present, and secondary deposits of a similar tumor were confirmed in the para-aortic lymph nodes, left lung and brain. In the compressed testicular tissue around the tumor on the right side there were recognizable seminiferous tubules with spermatogenesis (fig. 4). Histology of the left testis showed slightly reduced spermatogenesis, some fibrosis and mild Leydig cell hyperplasia. DISCUSSION
There have been 37 cases of polyorchidism reported previAccepted for publication March 21, 1980. 930
POLYORCHIDISM WITH TESTICULAR TERATOMA
FIG. 1. Photomicrograph of right inguinal testis shows immature seminiferous tubules surrounded by red blood cells and fibrin owing to infarction. H & E, reduced from X32.
931
FIG. 3. Photomicrograph of testicular tumor. Tumor cells are pleomorphic and giant cells form ill-defined villous as seen in trophoblastic teratoma (choriocarcinoma). H & E, reduced from X80.
FIG. 4. Photomicrograph of surviving rim of testicular tissue around teratoma. Seminiferous tubule can be seen containing cells at all stages of spermatogenesis, including spermatozoa. There is some fibrosis in surrounding tissues. H & E, reduced from X80. FIG. 2. Right testis with tumor bisected. Compressed rim of testicular tissue can be seen around top right of mass and spermatic cord is attached.
ment of a neoplasm. This case also is unusual in that the duplication occurred on the right side of the scrotum. Butz and Croushore stated that 21 of the previously described 36 cases were on the left side. I Also, spermatogenesis was present in the left testis and in the surviving rim of the testis on the right side around the tumor. Spermatogenesis was present in only 15 of the 36 cases described previously. I Mr. J. A. MacDougall assisted in the preparation of this paper. REFERENCE
1. Butz, R. E. and Croushore, J. H.: Polyorchidism. J. Urol., 119: 289, 1978. 2. Williams, D. I.: Paediatric Urology. London: Butterworth & Co., p. 462, 1968. 3. Wilson, W. A. and Littler, J.: Polyorchidism; report of 2 cases with
torsion. Brit. J. Surg., 41: 302, 1953. 4. Gehring, G. G., Rodriguez, F. R. and Woodhead, D. M.: Malignant degeneration of cryptorchid testis following orchiopexy. J. Urol., 112: 354, 1974. 5. Cunningham, J. H.: New growths developing in undescended testicles. J. Urol., 5: 471, 1921. 6. Sumner, W. A.: Malignant tumor of testis occurring 29 years after orchiopexy: case report and review of the literature. J. Urol., 81: 150, 1959. 7. Dow, J. A. and Mostofi, F. K.: Testicular tumors following orchiopexy. South. Med. J., 60: 193, 1967. 8. Johnson, D. E., Woodhead, D. M., Pohl, D.R. and Robison, J. R.: Cryptorchidism and testicular tumorigenesis. Surgery, 63: 919, 1968. 9. Johnson, D. E.: Testicular Tumors. New York: Medical Examination Publishing Co., Inc., pp. 34-35, 1972. 10. Sohval, A. R.: Testicular dysgenesis as an etiologic factor in cryptorchidism. J. Urol., 72: 693, 1954. 11. Willis, R. A.: Pathology of Tumours, 4th ed. London: Butterworth & Co., p. 570, 1967.