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Malignant Disease in the Undescended Testis
MALIGNANT DISEASE IN THE UNDESCENDED TESTIS OSCAR
B.
MURRAY AND EARL E. EWERT
THE presence in the hospital at the same time of two physicians with malignant disease of the undescended testis has prompted us to review our cases of tumors of the undescended testis and to examine our statistics in regard to the number of cases of undescended testicles in the last five years. There is some understandable variation throughout the lit(;rature1 , 3. 4 in the reported incidence of testicular malignancy. The general statistical data show an incidence of 0.5 to 0.6 per cent in relation to malignant disease of all other organs. The Memorial Hospital in New York, however, quoted an incidence of 2.09 per cent with regard to all other malignant tumors in males. In reviewing the literature it is definitely established that the incidence of malignant disease of the testes is markedly accelerated in those that are undescended. In one series of 694 cases of undescended testes, 11 per cent were malignant. Cancer of the testes associated with ectopy in over 11 per cent of the recorded caSeS indicates a correlation 48 times greater than expected by chance association. In patients with cancer of one testis and unilateral cryptorchidism, 97.5 per cent of the tumors are in the ectopic testis. In bilateral cryptorchidism with unilateral testicular cancer, a tumor of the second testis developed in 24 per cent, whereas when both testes were in the scrotum a second testicular tumor developed in but 0.7 per cent of the cases. The frequency of bilateral involvement of the ectopic testis is 32 times that of the scrotal testis. In view of these facts, we Believe that the ectopic testes must be regarded as a potentially malignant tumor. It would seem from the literature of unilateral cryptorchidism that if the testis cannot be re. placed in the scrotum it should be removed. At the Lahey Clinic during the last five years there have been 50 cases of ectopic testicles. During this same period 14 patients were seen with testicular tumor and of these 14, the tumor was in the undescended testis in 4 cases, an incidence of over 28 per cent. Although the scrotal testis is readily accessible to examination, an average period of fifteen months elapsed from the time the patient first noticed the swelling until he presented himself for treatment. Perhaps because of the painless enlargement of the testicle as the most common presenting symptom, this long delay is explainable. These patients are most often seen between the ages of 20 and 50, which is during the years of greatest sexual activity. The diagnosis of malignant disease must be based on an ever709
710
OSCAR B. MURRAY, EARL E. EWERT
present suspicion whenever any type of swelling, usually painless, occurs in this scrotum. The Aschheim-Zondek test is helpful only when positive and does not exclude the presence of malignant disease when negative. It has also been demonstrated that there is no close correlation between the amount of hormone in the urine and the structure of the testicular tumor. One exception, perhaps, is the chorionepithelioma. This condition produces a gonadotropic hormone, often in great amounts. The amount of hormone in the urine after removal of the tumor and x-ray treatment can be used at times as a criterion both as to the efficacy of the therapy and to determine the need for further therapy while the patient is under close observation. We wish to report the 4 patients with tumors of the undescended testis. REPORT OF CASES CASE I.-A 35-year-old physician stated that he had had a right inguinal hernia repaired when he was five years of age at which time the right testis was transfixed to the inguinal canal. It had remained just distal to the external ring
Fig. 254. (Case 1).-The only focus of embryonal carcinoma found is shown (low power). since the time of operation. He was quite definite of the fact that the testicle was in the scrotum prior to operation. He stated that for the past few months there had been some pain and tenderness in the ectopic testis. This was particularly prominent when he stood or when pressure was. applied to the testis.
MALIGNANT DISEASE IN UNDESCENDED TESTIS
711
The right testis could be palpated in the inguinal canal just distal to the external ring. There was no enlargement of the inguinal nodes and no masses palpable in the abdomen. The consistency and the size of the testis seemed to be within normal limits. A right orchiectomy was carried out at this time and the testis was found to be in the mid inguinal canal. It was grossly normal in appearance and size. The pathologic report revealed atrophy of the testicular elements with a small focus of embryonal carcinoma. Serial sections showed no extension beyond the original focus (Figs. 254 and 255).
Fig. 255 (Case 1).-High power magnification of the same field shown in Figure 254.
We believe this case is one of the earliest malignancies of the testes seen. An Aschheim-Zondek test was not done in this case as malignancy was not suspected. CASE 2.-A physician, aged 48, entered the clinic for examination because of a symptomless mass in the left pelvis. There was a history of bilateral cryptorchidism with removal of the right testis at an earlier date. Physical examination was negative except for the palpable mass. Laparotomy was carried out, with removal of the mass; frozen sections at this time revealed carcinoma. The microscopic examination showed embryonal carcinoma with lymphoid stroma and a few atrophic testicular tubules. Grossly, this mass was found to be about 15 cm. in diameter, lying free in the pelvis except for an attachment to the left spermatic cord and an attachment to the lower portion of the ileum by an adhesion.
712
OSCAR B. MURRAY, EARL E. EWERT
CASE 3.-A man, aged 32, was seen at the clinic because of abdominal pain and a palpable mass in the abdomen. On examination, the left testis was found to be in the scrotum; the right was not palpable in the canal or the scrotum. Through a laparotomy incision, a mass W!lS removed and a pathologic diagnosis was made of embryonal carcinoma. Postoperatively, this man was given intensive x-ray therapy, but his urine continued to give a strongly positive Friedman test. The patient died within a year. CASE 4.-A 24-year-old man entered the clinic complaining of low back pain. Abdominal examination revealed a mass extending from the pelvis to the navel. There was also a mass, measuring 8 cm. in diametef", in the right supraclavicular fossa. The right testis was not in the scrotum; the left testis appeared to be normal on palpation. His condition was such that only a biopsy specimen was obtained from the mass in the supraclavicular fossa. The pathologist made a diagnosis of metastatic carcinom'l, teratomatous in origin. The patient died shortly after operation. SUMMARY
The literature is reviewed with regard to the incidence of testicular tumor in ectopic testes in relation to the incidence in testes that are normally present in the scrotum. It is shown that the incidence of malignant disease in the normally situated testes is slightly over 0.5 per cent, while that of the ectopic testes is over 11 per cent. The difference in these statistics is striking and cannot be overlooked. The frequency of bilateral changes. in the ectopic testes with relation to involvement of the scrotal testes was also reviewed. The incidence of involvement of the second ectopic testis was 24.6 per cent, whereas in the scrotal testes after involvement of the first there was an incidence of only 0.7 per cent in the second. Orchidopexy does not preclude the possibility of the development of malignant disease according to Gilbert. A review is presented of 4 cases of malignant disease of the ectopic testes, one of which sho,,:,"ed a very early focus of embryonal carcinoma. CONCLUSIONS
1. The undescended testis is particularly liable to undergo malignant degeneration, possibly from repeated small, unnoticed traumatic disturbances, either by muscle or direct violence or because the important heat regulating mechanism of the scrotum has been lost. 2. We believe that the optimum time for orchidopexy is just before or during adolescence since few testes descend after this time. 3. If it is impossible to replace a unilateral cryptorchid testis in a normal position, it should be removed and no palliative procedure, such as placing it in the abdomen, should be carried out. 4. A patient with an intra-abdominal cryptorchid testis presenting himself for examination should be made aware of the very potential malignancy he carries. 5. The Aschheim-Zondek and Friedman tests are helpful only when positive and nothing takes the place of a highly suspicious attitude toward a painless swelling of the scrotum in an adult male.
MALIGNANT DISEASE IN UNDESCENDED TESTIS
713
REFERENCES 1. Barringer, B. S.: Prognosis in teratoma testis. J. Urol., 52:578-582 (Dec.) 1944. 2. Gilbert, J. B.: Stndies in malignant testis tnmors. Cases and review of 63. J. Urol., 46:740-747 (Oct.) 1941. 3. Hellwig, C. A.: Diagnosis and treatment of tumors of testis. J. Kansas Med. Soc., 46:37-40 (Feb.) 1945. 4. Ormond, J. K. and Prince, P. L.: Malignant tumors of testicle. J. Urol., 4.5: 685-691 (May) 1941.
B.
MURRAY AND EARL E. EWERT
THE presence in the hospital at the same time of two physicians with malignant disease of the undescended testis has prompted us to review our cases of tumors of the undescended testis and to examine our statistics in regard to the number of cases of undescended testicles in the last five years. There is some understandable variation throughout the lit(;rature1 , 3. 4 in the reported incidence of testicular malignancy. The general statistical data show an incidence of 0.5 to 0.6 per cent in relation to malignant disease of all other organs. The Memorial Hospital in New York, however, quoted an incidence of 2.09 per cent with regard to all other malignant tumors in males. In reviewing the literature it is definitely established that the incidence of malignant disease of the testes is markedly accelerated in those that are undescended. In one series of 694 cases of undescended testes, 11 per cent were malignant. Cancer of the testes associated with ectopy in over 11 per cent of the recorded caSeS indicates a correlation 48 times greater than expected by chance association. In patients with cancer of one testis and unilateral cryptorchidism, 97.5 per cent of the tumors are in the ectopic testis. In bilateral cryptorchidism with unilateral testicular cancer, a tumor of the second testis developed in 24 per cent, whereas when both testes were in the scrotum a second testicular tumor developed in but 0.7 per cent of the cases. The frequency of bilateral involvement of the ectopic testis is 32 times that of the scrotal testis. In view of these facts, we Believe that the ectopic testes must be regarded as a potentially malignant tumor. It would seem from the literature of unilateral cryptorchidism that if the testis cannot be re. placed in the scrotum it should be removed. At the Lahey Clinic during the last five years there have been 50 cases of ectopic testicles. During this same period 14 patients were seen with testicular tumor and of these 14, the tumor was in the undescended testis in 4 cases, an incidence of over 28 per cent. Although the scrotal testis is readily accessible to examination, an average period of fifteen months elapsed from the time the patient first noticed the swelling until he presented himself for treatment. Perhaps because of the painless enlargement of the testicle as the most common presenting symptom, this long delay is explainable. These patients are most often seen between the ages of 20 and 50, which is during the years of greatest sexual activity. The diagnosis of malignant disease must be based on an ever709
710
OSCAR B. MURRAY, EARL E. EWERT
present suspicion whenever any type of swelling, usually painless, occurs in this scrotum. The Aschheim-Zondek test is helpful only when positive and does not exclude the presence of malignant disease when negative. It has also been demonstrated that there is no close correlation between the amount of hormone in the urine and the structure of the testicular tumor. One exception, perhaps, is the chorionepithelioma. This condition produces a gonadotropic hormone, often in great amounts. The amount of hormone in the urine after removal of the tumor and x-ray treatment can be used at times as a criterion both as to the efficacy of the therapy and to determine the need for further therapy while the patient is under close observation. We wish to report the 4 patients with tumors of the undescended testis. REPORT OF CASES CASE I.-A 35-year-old physician stated that he had had a right inguinal hernia repaired when he was five years of age at which time the right testis was transfixed to the inguinal canal. It had remained just distal to the external ring
Fig. 254. (Case 1).-The only focus of embryonal carcinoma found is shown (low power). since the time of operation. He was quite definite of the fact that the testicle was in the scrotum prior to operation. He stated that for the past few months there had been some pain and tenderness in the ectopic testis. This was particularly prominent when he stood or when pressure was. applied to the testis.
MALIGNANT DISEASE IN UNDESCENDED TESTIS
711
The right testis could be palpated in the inguinal canal just distal to the external ring. There was no enlargement of the inguinal nodes and no masses palpable in the abdomen. The consistency and the size of the testis seemed to be within normal limits. A right orchiectomy was carried out at this time and the testis was found to be in the mid inguinal canal. It was grossly normal in appearance and size. The pathologic report revealed atrophy of the testicular elements with a small focus of embryonal carcinoma. Serial sections showed no extension beyond the original focus (Figs. 254 and 255).
Fig. 255 (Case 1).-High power magnification of the same field shown in Figure 254.
We believe this case is one of the earliest malignancies of the testes seen. An Aschheim-Zondek test was not done in this case as malignancy was not suspected. CASE 2.-A physician, aged 48, entered the clinic for examination because of a symptomless mass in the left pelvis. There was a history of bilateral cryptorchidism with removal of the right testis at an earlier date. Physical examination was negative except for the palpable mass. Laparotomy was carried out, with removal of the mass; frozen sections at this time revealed carcinoma. The microscopic examination showed embryonal carcinoma with lymphoid stroma and a few atrophic testicular tubules. Grossly, this mass was found to be about 15 cm. in diameter, lying free in the pelvis except for an attachment to the left spermatic cord and an attachment to the lower portion of the ileum by an adhesion.
712
OSCAR B. MURRAY, EARL E. EWERT
CASE 3.-A man, aged 32, was seen at the clinic because of abdominal pain and a palpable mass in the abdomen. On examination, the left testis was found to be in the scrotum; the right was not palpable in the canal or the scrotum. Through a laparotomy incision, a mass W!lS removed and a pathologic diagnosis was made of embryonal carcinoma. Postoperatively, this man was given intensive x-ray therapy, but his urine continued to give a strongly positive Friedman test. The patient died within a year. CASE 4.-A 24-year-old man entered the clinic complaining of low back pain. Abdominal examination revealed a mass extending from the pelvis to the navel. There was also a mass, measuring 8 cm. in diametef", in the right supraclavicular fossa. The right testis was not in the scrotum; the left testis appeared to be normal on palpation. His condition was such that only a biopsy specimen was obtained from the mass in the supraclavicular fossa. The pathologist made a diagnosis of metastatic carcinom'l, teratomatous in origin. The patient died shortly after operation. SUMMARY
The literature is reviewed with regard to the incidence of testicular tumor in ectopic testes in relation to the incidence in testes that are normally present in the scrotum. It is shown that the incidence of malignant disease in the normally situated testes is slightly over 0.5 per cent, while that of the ectopic testes is over 11 per cent. The difference in these statistics is striking and cannot be overlooked. The frequency of bilateral changes. in the ectopic testes with relation to involvement of the scrotal testes was also reviewed. The incidence of involvement of the second ectopic testis was 24.6 per cent, whereas in the scrotal testes after involvement of the first there was an incidence of only 0.7 per cent in the second. Orchidopexy does not preclude the possibility of the development of malignant disease according to Gilbert. A review is presented of 4 cases of malignant disease of the ectopic testes, one of which sho,,:,"ed a very early focus of embryonal carcinoma. CONCLUSIONS
1. The undescended testis is particularly liable to undergo malignant degeneration, possibly from repeated small, unnoticed traumatic disturbances, either by muscle or direct violence or because the important heat regulating mechanism of the scrotum has been lost. 2. We believe that the optimum time for orchidopexy is just before or during adolescence since few testes descend after this time. 3. If it is impossible to replace a unilateral cryptorchid testis in a normal position, it should be removed and no palliative procedure, such as placing it in the abdomen, should be carried out. 4. A patient with an intra-abdominal cryptorchid testis presenting himself for examination should be made aware of the very potential malignancy he carries. 5. The Aschheim-Zondek and Friedman tests are helpful only when positive and nothing takes the place of a highly suspicious attitude toward a painless swelling of the scrotum in an adult male.
MALIGNANT DISEASE IN UNDESCENDED TESTIS
713
REFERENCES 1. Barringer, B. S.: Prognosis in teratoma testis. J. Urol., 52:578-582 (Dec.) 1944. 2. Gilbert, J. B.: Stndies in malignant testis tnmors. Cases and review of 63. J. Urol., 46:740-747 (Oct.) 1941. 3. Hellwig, C. A.: Diagnosis and treatment of tumors of testis. J. Kansas Med. Soc., 46:37-40 (Feb.) 1945. 4. Ormond, J. K. and Prince, P. L.: Malignant tumors of testicle. J. Urol., 4.5: 685-691 (May) 1941.