Vol. 113, February Printed in
THE JOURNAL OF UROLOGY
Copyright © 1975 by The Williams & Wilkins Co.
RHABDOMYOSARCOMA OF THE SPERMATIC CORD: REPORT OF 3 CASES WITH REVIEW OF THE LITERATURE D. A. SKEEL, H. R. DRINKER, JR.*
AND
R. WITHERINGTON
From the Department of Surgery, Section of Urology, The Medical College of Georgia, Augusta, Georgia
Tumors of the spermatic cord are rare and many are highly malignanL After a review of the literature in 1969, 15 histological varieties were described by Arlen and associates. 1 Of the 150 cases reported by them 35 (23 per cent) of the malignant tumors of the spermatic cord were rhabdomyosarcomas. To the 50 cases of rhabdomyosarcoma reported we will add 3, making a total of 53 cases reported. 1· 3 CASE REPORTS
Case 1. S. S., a 10-year-old black boy, was seen on 22, 1972 with an asymptomatic 10 by 8 by 6 cm. left scrotal mass (fig. 1). The mass had developed during the 3 months prior to hospitalization. Prior to that time the boy had been in good health with no scrotal or testicular abnormalities. The size of the rapidly growing lesion had caused the child to refrain from his favorite activities, such as swimming, primarily for cosmetic reasons. After a completely normal preoperative evaluation, including a chest x-ray, urinalysis, renal and liver function studies, excretory urogram (IVP), bone survey, liver and spleen scan and urinary chorionic gonadotropins, left radical orchiectomy was done through an inguinal incision. The incision had to be extended into the upper scrotum to allow removal of the mass. The tumor was irregular, not well encapsulated and varied in consistency between firm and solid to cystic and necrotic (fig. 2). Normal testicular tissue adjacent to the neoplasm indicated that the spermatic cord was the site of origin (fig. The histologic diagnosis of rhabdomyosarcoma was established at the Armed Forces Institute of Pathology (fig. 4). A lymphangiogram appeared to be normal so bilateral retroperitoneal lymph node dissection was done, which yielded 36 nodes. All nodes were free of tumor. Four months postoperatively a soft, fluctuant swelling, the size of the original Accepted for publication July 5, 1974. Read at annual meeting of Western Section, American Urological Association, San Francisco, California, March 31-April 5, 1974. '''Current address: 2440 Samaritan Drive, San Jose, California 95124. 1 Arlen, M., Grabstald, H. and Whitmore, W. F., Jr.: Malignant tumors of the spermatic cord. Cancer, 23: 525, 1969. 2 Malek, R S., Utz, D. C. and Farrnw, G. M.: Malignant tumors of the spermatic cord. Cancer, 29: 1108, 1972. 3 Ghazali, S.: Embryonic rhabdomyosarcoma of the urogenital tract. Brit. J. Surg., 60: 124, 1973.
mass, developed in the left hemiscrotum. Subsequently, a course of cobalt 60 irradiation directed at the mass and at the inguinal areas bilaterally. No suspicious lymph nodes were ble prior to or after the course of treatment. mass completely resolved and the quently underwent left hemiscrotectomy ilio-inguinal lymphadenectomy. All excised tissue appeared grossly and microscopically free of disand ease. The patient has been followed remains free of obvious disease 1 ½ years after onset and 8 months since the most recent opera·· tion. Case 2. R. 104-537, a 16-year-old white was admitted to the hospital in 1963 because painless swelling in the left scrotal month in duration. A smooth 3 4 cm. mass was found separate from the testis but adherent to spermatic cord. Preoperative evaluation was negative, including urinary chorionic and chest x-ray. The testicle and the cord were removed through an inguinal approach. cal diagnosis was rhabdomyosarcoma of the ma tic cord (fig. 5). The patient underwent no further treatment and is ostensibly free of disease 10 years postoperatively. Case 3. B. C., 184-788, a 16-year-old white was admitted to the hospital on March 17, 18'73 because of an asymptomatic left scrotal mass which had been present for 3 months. Left radi.cal orchiectomy was performed through an approach with ligation of the cord at the internal inguinal ring (fig. 6). The histologic rhabdomyosarcoma of the cord Evaluation of the patient with IVP, gram, renal function studies, liver function and liver and spleen scan revealed enlarged nodes at the left renal hilus and diffuse aly. Exploratory laparotomy revealed a normal appearing, although somewhat enlarged, liver and palpable lymph nodes throughout the and iliac chains bilaterally. The nodes did seem to be enlarged but those in the L2 to region were hard on palpation. A liver negative. Radiation therapy, consisting of 2,500R administered to the para -aortic nodes, linear accelerator. The patient was seen at another hospital, where vincristine, cytoxan and actinomycin D were administered. retroperitoneal lymphadenectomy was nc,rrnnr.Prl and 25 nodes were removed, 2 of which contained metastatic rhabdomyosarcoma. After u~HH~ ,.,,,
279
280
SKEEL, DRINKER AND WITHERINGTON
FIG. 1. Case 1. Scrotal mass that developed in 3 months
FIG. 2. Case 1. Surgical specimen of left testes and cord varied in consistency from solid to soft and necrotic.
from the hospital the patient resumed chemotherapy as an outpatient and continued radiotherapy to a total dose of 5,000R. He did well for 6 months after completing therapy but then severe headaches developed, which led to the diagnosis of metastatic brain disease. Despite radiation therapy to the metastatic site, he died of the disease following a steady downhill course.
Eleven patients with rhabdomyosarcoma of the spermatic cord underwent retroperitoneal lymphadenectomy as part of the treatment (table 1). 1 • 2 • 4 Of the 6 patients described by Arlen and associates 3 have survived 2 to 4 years postoperatively, 1 Malek and associates reported on 2 patients, both of whom survived 2 ½ years postoperatively. 2 Gray
and Biorn reported on 1 patient who is surviving 15 years postoperatively. 4 Our series includes 2 patients, 1 of whom has survived 1 ½ years postoperatively. Thus, of the 11 patients who underwent retroperitoneal lymphadenectomy 7 have survived for 1 ½ to 15 years postoperatively and 4 have died of their disease. Results of therapy in cases reported since 1958 that did not have para-aortic lymphadenectomy and apparently did fit the criteria as candidates for that procedure include 5 patients: 3 are 10-year survivors and 2 patients died of their disease (table 2), Gray and Biorn reported on 1 patient who underwent orchiectomy with high ligation of the cord followed by irradiation therapy and died of the disease. 5 Ghazali reported on 3 patients with 2, 10-year survivors. These patients underwent ingui-
•Gray, C. P. and Biorn, C. L.: Myosarcoma of the spermatic cord. J. Urol., 84: 562, 1960,
' Gray, C. P. and Biorn, C. L.: Rhabdomyosarcoma of the spermatic cord. J. Urol., 74: 402, 1955.
RESULTS
RHABDOMYOSARCOMA OF SPERMATIC CORD
FIG. 3. Case 1. Histologic delineation of normal testicular tissue and neoplastic spermatic cord tumor
Fm. 4. Case L Histologic section of rhabdomyosarcoma of spermatic cord
with 3 Our seactinomycin ries includes 1 patient who underwent inguinal and high ligation of the cord and is surviving 10 years ~nercm~ DISCUSSION
Rokitans first called attention to rhabdomyosarcoma of the cord in 1849. 1 Traditionally rhabdomyosarcoma of the spermatic cord has been encountered in children or young adults
nign tumors of the cord tend to arise in the canal, malignant tumors arise in the cord au. 1accu above the testis. 6 The nr,a~c,nt
THE JOURNAL OF UROLOGY
Copyright © 1975 by The Williams & Wilkins Co.
RHABDOMYOSARCOMA OF THE SPERMATIC CORD: REPORT OF 3 CASES WITH REVIEW OF THE LITERATURE D. A. SKEEL, H. R. DRINKER, JR.*
AND
R. WITHERINGTON
From the Department of Surgery, Section of Urology, The Medical College of Georgia, Augusta, Georgia
Tumors of the spermatic cord are rare and many are highly malignanL After a review of the literature in 1969, 15 histological varieties were described by Arlen and associates. 1 Of the 150 cases reported by them 35 (23 per cent) of the malignant tumors of the spermatic cord were rhabdomyosarcomas. To the 50 cases of rhabdomyosarcoma reported we will add 3, making a total of 53 cases reported. 1· 3 CASE REPORTS
Case 1. S. S., a 10-year-old black boy, was seen on 22, 1972 with an asymptomatic 10 by 8 by 6 cm. left scrotal mass (fig. 1). The mass had developed during the 3 months prior to hospitalization. Prior to that time the boy had been in good health with no scrotal or testicular abnormalities. The size of the rapidly growing lesion had caused the child to refrain from his favorite activities, such as swimming, primarily for cosmetic reasons. After a completely normal preoperative evaluation, including a chest x-ray, urinalysis, renal and liver function studies, excretory urogram (IVP), bone survey, liver and spleen scan and urinary chorionic gonadotropins, left radical orchiectomy was done through an inguinal incision. The incision had to be extended into the upper scrotum to allow removal of the mass. The tumor was irregular, not well encapsulated and varied in consistency between firm and solid to cystic and necrotic (fig. 2). Normal testicular tissue adjacent to the neoplasm indicated that the spermatic cord was the site of origin (fig. The histologic diagnosis of rhabdomyosarcoma was established at the Armed Forces Institute of Pathology (fig. 4). A lymphangiogram appeared to be normal so bilateral retroperitoneal lymph node dissection was done, which yielded 36 nodes. All nodes were free of tumor. Four months postoperatively a soft, fluctuant swelling, the size of the original Accepted for publication July 5, 1974. Read at annual meeting of Western Section, American Urological Association, San Francisco, California, March 31-April 5, 1974. '''Current address: 2440 Samaritan Drive, San Jose, California 95124. 1 Arlen, M., Grabstald, H. and Whitmore, W. F., Jr.: Malignant tumors of the spermatic cord. Cancer, 23: 525, 1969. 2 Malek, R S., Utz, D. C. and Farrnw, G. M.: Malignant tumors of the spermatic cord. Cancer, 29: 1108, 1972. 3 Ghazali, S.: Embryonic rhabdomyosarcoma of the urogenital tract. Brit. J. Surg., 60: 124, 1973.
mass, developed in the left hemiscrotum. Subsequently, a course of cobalt 60 irradiation directed at the mass and at the inguinal areas bilaterally. No suspicious lymph nodes were ble prior to or after the course of treatment. mass completely resolved and the quently underwent left hemiscrotectomy ilio-inguinal lymphadenectomy. All excised tissue appeared grossly and microscopically free of disand ease. The patient has been followed remains free of obvious disease 1 ½ years after onset and 8 months since the most recent opera·· tion. Case 2. R. 104-537, a 16-year-old white was admitted to the hospital in 1963 because painless swelling in the left scrotal month in duration. A smooth 3 4 cm. mass was found separate from the testis but adherent to spermatic cord. Preoperative evaluation was negative, including urinary chorionic and chest x-ray. The testicle and the cord were removed through an inguinal approach. cal diagnosis was rhabdomyosarcoma of the ma tic cord (fig. 5). The patient underwent no further treatment and is ostensibly free of disease 10 years postoperatively. Case 3. B. C., 184-788, a 16-year-old white was admitted to the hospital on March 17, 18'73 because of an asymptomatic left scrotal mass which had been present for 3 months. Left radi.cal orchiectomy was performed through an approach with ligation of the cord at the internal inguinal ring (fig. 6). The histologic rhabdomyosarcoma of the cord Evaluation of the patient with IVP, gram, renal function studies, liver function and liver and spleen scan revealed enlarged nodes at the left renal hilus and diffuse aly. Exploratory laparotomy revealed a normal appearing, although somewhat enlarged, liver and palpable lymph nodes throughout the and iliac chains bilaterally. The nodes did seem to be enlarged but those in the L2 to region were hard on palpation. A liver negative. Radiation therapy, consisting of 2,500R administered to the para -aortic nodes, linear accelerator. The patient was seen at another hospital, where vincristine, cytoxan and actinomycin D were administered. retroperitoneal lymphadenectomy was nc,rrnnr.Prl and 25 nodes were removed, 2 of which contained metastatic rhabdomyosarcoma. After u~HH~ ,.,,,
279
280
SKEEL, DRINKER AND WITHERINGTON
FIG. 1. Case 1. Scrotal mass that developed in 3 months
FIG. 2. Case 1. Surgical specimen of left testes and cord varied in consistency from solid to soft and necrotic.
from the hospital the patient resumed chemotherapy as an outpatient and continued radiotherapy to a total dose of 5,000R. He did well for 6 months after completing therapy but then severe headaches developed, which led to the diagnosis of metastatic brain disease. Despite radiation therapy to the metastatic site, he died of the disease following a steady downhill course.
Eleven patients with rhabdomyosarcoma of the spermatic cord underwent retroperitoneal lymphadenectomy as part of the treatment (table 1). 1 • 2 • 4 Of the 6 patients described by Arlen and associates 3 have survived 2 to 4 years postoperatively, 1 Malek and associates reported on 2 patients, both of whom survived 2 ½ years postoperatively. 2 Gray
and Biorn reported on 1 patient who is surviving 15 years postoperatively. 4 Our series includes 2 patients, 1 of whom has survived 1 ½ years postoperatively. Thus, of the 11 patients who underwent retroperitoneal lymphadenectomy 7 have survived for 1 ½ to 15 years postoperatively and 4 have died of their disease. Results of therapy in cases reported since 1958 that did not have para-aortic lymphadenectomy and apparently did fit the criteria as candidates for that procedure include 5 patients: 3 are 10-year survivors and 2 patients died of their disease (table 2), Gray and Biorn reported on 1 patient who underwent orchiectomy with high ligation of the cord followed by irradiation therapy and died of the disease. 5 Ghazali reported on 3 patients with 2, 10-year survivors. These patients underwent ingui-
•Gray, C. P. and Biorn, C. L.: Myosarcoma of the spermatic cord. J. Urol., 84: 562, 1960,
' Gray, C. P. and Biorn, C. L.: Rhabdomyosarcoma of the spermatic cord. J. Urol., 74: 402, 1955.
RESULTS
RHABDOMYOSARCOMA OF SPERMATIC CORD
FIG. 3. Case 1. Histologic delineation of normal testicular tissue and neoplastic spermatic cord tumor
Fm. 4. Case L Histologic section of rhabdomyosarcoma of spermatic cord
with 3 Our seactinomycin ries includes 1 patient who underwent inguinal and high ligation of the cord and is surviving 10 years ~nercm~ DISCUSSION
Rokitans first called attention to rhabdomyosarcoma of the cord in 1849. 1 Traditionally rhabdomyosarcoma of the spermatic cord has been encountered in children or young adults
nign tumors of the cord tend to arise in the canal, malignant tumors arise in the cord au. 1accu above the testis. 6 The nr,a~c,nt
