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Myosarcoma of the Spermatic Cord
THE JOURNAL OF UROLOGY
Vol. 84, No. 4, October 1960 Printed in U.S.A.
MYOSARCOMA OF THE SPERMATIC CORD CARY P. GRAY
AND
CARLL. BIORN
From the Division of Urology, Palo Alto Medical Clinic, Palo Alto, Cal.
Spermatic cord tumors are rare and myosarcomas of the spermatic cord are exceedingly so. These tumors, whether from striated or nonstriated muscle fibers, grow rapidly and are extremely malignant and usually fatal regardless of where they are found. They occur in early life in most cases and over one-half the recorded cases occurred in children under five years of age. Because very few cases have been carefully studied and reported, little is known of the progress of these tumors. The genesis of myosarcomas of the spermatic cord has been thought to be either from the displacement of myotome tissue along the vas deferens and from existing skeletal muscle or from unusual development of a teratoma.1 In the report of our first case,2 we advocated more radical treatment of myosarcomas of the spermatic cord than had hitherto been advised. Our recommendation was based on the autopsy finding in our patient of obvious local lymphatic dissemination of the tumor without evidence of hematogenous spread. This patient had been treated only with orchiectomy and irradiation therapy, which had been advised by two tumor clinics to which the case was submitted for consultation. On the basis of this experience and after dis. cussion with others who had had a great deal of experience with testicular tumors,3 we concluded that if a patient with myosarcoma of the spermatic cord had no evident hematogenous metastatic involvement (bone, liver, or lung), radical orchiectomy with radical retroperitoneal periaortic lymph node dissection and resection of the spermatic cord to the origin of the vessels at the aorta and venae cava should be done. Furthermore, in spite of the young age of these patients, we favored postoperative irradiation. The radical form of treatment recommended after our initial experience2 was first employed in Accepted for publication February 29, 1960. 1 Friedman, N. B.: Personal communication. 2 Gray, C. P. and Biorn, C. L.: Rhabdornyosarcoma of the sperrnatic cord. J. Urol., 74: 402406, 1955. 3 Lewis, L. G.: Personal cornrnunication.
a subsequent case which is the subject of this report. It is now over 18 months since treatment was concluded and the patient is well with no evident metastasis. We hope that this report of apparently good results will encourage others to pursue the same course of action in the treatment of this highly malignant and almost uniformly fatal tumor. The subject of this report is a child, aged 37~ years, who was first seen on May 27, 1958 for examination of an enlarged testicle. The patient's mother stated that the enlargement was of only a few days in duration. He had a right hydrocele with a palpable, irregularly enlarged, painless intrascrotal tumor which did not transilluminate. The excretory urogram, bone survey and chest films were all normal. The blood and urine studies were within normal limits. On June 4, 1958 surgical exploration of the scrotum was done through a right inguinal incision after previously having clamped the spermatic cord with a "rubber shod" clamp. The testicle and cord were removed. Grossly the tissue consisted of a soft pinkish gray tumor mass distinct from the testicle but involving the epididymis and lower cord (fig. 1). Microscopic examination (fig. 2,A) demonstrated tumor cells which varied from rounded or oval form to spindle shape and included clusters of oval cells with abundant pink cytoplasm. The nuclei were vesicular and locally hyperchromatic. Mitotic figures were very numerous. The cells were without definite pattern and surrounded by thinwalled blood vessels. In some of the cells fine fibrils were found; a rare fibril showed cross striations. In one area the tumor cells were found lying between tubules of the epididymis and continuous with its stroma. The patient was presented to the tumor clinic and after a review of the previous case, the radical approach was approved. On June 11, 1958 a second operation was performed. A transthoracico-abdominal incision made between the tenth and eleventh ribs was extended onto the anterior abdominal wall. The diaphragm was opened then the spermatic cord
562
MYOSARCOJ\/IA OF SPERJ\/IATIC
and its blood and lymphatic ves;;els were reseded to their points of origin, following which the pm·iaortic nodes ancl lymph ,·aseular channels were r,",P<'T,l'n tagging :tll thr structures as they were removed. Vismdization was exeellent, and the exploration ,vas therefore cxknded beyond the mid]i1w and carried to a point below the bifurcation of the aorta. The noclc s superior to 0
corm
the right renal pedicle and the adjacent tissue were extensively replaced by niesoderm:d tumor (fig. 2,B). AJl other nodes wern free of tumor. The patient received 20 mg. nitrogen mustard in an attempt to destroy any tumor cells which might have been disseminated into the bloocl. stream. The surgery :1nd ehernothernpy wr:rc tolerated well. He wr1s dismissecl from the ho:c; pita] on the eleventh postoperative in e;;cdlent condition. Radiation tbernpy was hcgun on the fourteenth postoperative cfay. Over a period of 3G he receivedapproxirnatdy3500 rads ton, 9 12 cm" abdominal field. This included the hilum of tlw right kidney and extended to the left of the midline and superiorly to the symphysis pubis. He was treatc,d at 70 cm. distance, 250 K.V., 1.."J mm
Fro. 1. Cross specinwn. Solid tumor involves spernrntic eon! a11cl r,pidiclyrnis but distinct from testicular p:uenchym:1.
Cu. h.v.l. starting with a dose of 50 rads and increasing the dose increment to a maximum ol 200 rads daily. This patient ,n1s last seen on December fl.. 1959, which was 18 months following surgery His blood count and sedimentation rate wen, normal. ChPst films and bone snrveys did not demonstrate metastasis. He :,ppearecl to be in c,xcdlrnt heitlth and was maintaining n, normal symmetrical growth pr1ttenL
]'re+. 2. Photomicrogrn]lhS. /I, note ov:d to spindle shaped tumor cells with vesicular,
nuclei and numerous mitotic hgnres. Cross-striations are evident in some areas (arrow). dermal tumor cells in renal J,iln.r lymph nodes :we! extending into adjacent adipose tissue,
564
CARY P. GRAY AND C.\RL L. BIOR~ DISCUSSION
The results of the study and findings of these cases appear to indicate that contrary to past and present opinions 4 , 5, there are a certain number of patients with myosarcomas of the genital organs, who, because of lymphatic dissemination of tumor cells, should have radical surgical treatment and irradiation therapy, as herein described. It is apparently incorrect to assume that all of these tumors spread by blood vascular channels. Obviously, if preoperative studies reveal evidence of hematogenous spread, then orchiectomy with high ligation of the cord 4 Kohler, F, P, and Murphy, J. J.: Rhabdomyosarcoma of the male genital tract. J. Urol.
82: 500-503, 1959. ' ~ Satter, E. J., Heidner, F. C. and Wear, J. F.:
Pnmary sarcoma of the spermatic cord and epididymis. J. Urol., 82: 148-154, 1959.
uncl deep irradiation therapy would be the treatment of choice. It is our opinion that the use of nitrogen mustt1rd t1t the termination of surgery nmy well be 1111 important ,1djunct to surgical therapy. CONCLUSION
J\Iyosarcomas of the spermatic cord may metastasize by lymphatic as well as vascular channels. ·without evidenee of vascular spread, the procedure of choice is dissection of the retroperitoneal lymphatic vessels ,1nd lymph nodes and the removal of the entire spermatic cord to its point of origin. A thoracico-abdominal t1pproach is preferable in children. Irradiation to tolerance is an important adjunct to resection. It m,1y well be that anti-cancer chemotherapy will provide an additional advantage in the attempt to eradicate this disease.
Vol. 84, No. 4, October 1960 Printed in U.S.A.
MYOSARCOMA OF THE SPERMATIC CORD CARY P. GRAY
AND
CARLL. BIORN
From the Division of Urology, Palo Alto Medical Clinic, Palo Alto, Cal.
Spermatic cord tumors are rare and myosarcomas of the spermatic cord are exceedingly so. These tumors, whether from striated or nonstriated muscle fibers, grow rapidly and are extremely malignant and usually fatal regardless of where they are found. They occur in early life in most cases and over one-half the recorded cases occurred in children under five years of age. Because very few cases have been carefully studied and reported, little is known of the progress of these tumors. The genesis of myosarcomas of the spermatic cord has been thought to be either from the displacement of myotome tissue along the vas deferens and from existing skeletal muscle or from unusual development of a teratoma.1 In the report of our first case,2 we advocated more radical treatment of myosarcomas of the spermatic cord than had hitherto been advised. Our recommendation was based on the autopsy finding in our patient of obvious local lymphatic dissemination of the tumor without evidence of hematogenous spread. This patient had been treated only with orchiectomy and irradiation therapy, which had been advised by two tumor clinics to which the case was submitted for consultation. On the basis of this experience and after dis. cussion with others who had had a great deal of experience with testicular tumors,3 we concluded that if a patient with myosarcoma of the spermatic cord had no evident hematogenous metastatic involvement (bone, liver, or lung), radical orchiectomy with radical retroperitoneal periaortic lymph node dissection and resection of the spermatic cord to the origin of the vessels at the aorta and venae cava should be done. Furthermore, in spite of the young age of these patients, we favored postoperative irradiation. The radical form of treatment recommended after our initial experience2 was first employed in Accepted for publication February 29, 1960. 1 Friedman, N. B.: Personal communication. 2 Gray, C. P. and Biorn, C. L.: Rhabdornyosarcoma of the sperrnatic cord. J. Urol., 74: 402406, 1955. 3 Lewis, L. G.: Personal cornrnunication.
a subsequent case which is the subject of this report. It is now over 18 months since treatment was concluded and the patient is well with no evident metastasis. We hope that this report of apparently good results will encourage others to pursue the same course of action in the treatment of this highly malignant and almost uniformly fatal tumor. The subject of this report is a child, aged 37~ years, who was first seen on May 27, 1958 for examination of an enlarged testicle. The patient's mother stated that the enlargement was of only a few days in duration. He had a right hydrocele with a palpable, irregularly enlarged, painless intrascrotal tumor which did not transilluminate. The excretory urogram, bone survey and chest films were all normal. The blood and urine studies were within normal limits. On June 4, 1958 surgical exploration of the scrotum was done through a right inguinal incision after previously having clamped the spermatic cord with a "rubber shod" clamp. The testicle and cord were removed. Grossly the tissue consisted of a soft pinkish gray tumor mass distinct from the testicle but involving the epididymis and lower cord (fig. 1). Microscopic examination (fig. 2,A) demonstrated tumor cells which varied from rounded or oval form to spindle shape and included clusters of oval cells with abundant pink cytoplasm. The nuclei were vesicular and locally hyperchromatic. Mitotic figures were very numerous. The cells were without definite pattern and surrounded by thinwalled blood vessels. In some of the cells fine fibrils were found; a rare fibril showed cross striations. In one area the tumor cells were found lying between tubules of the epididymis and continuous with its stroma. The patient was presented to the tumor clinic and after a review of the previous case, the radical approach was approved. On June 11, 1958 a second operation was performed. A transthoracico-abdominal incision made between the tenth and eleventh ribs was extended onto the anterior abdominal wall. The diaphragm was opened then the spermatic cord
562
MYOSARCOJ\/IA OF SPERJ\/IATIC
and its blood and lymphatic ves;;els were reseded to their points of origin, following which the pm·iaortic nodes ancl lymph ,·aseular channels were r,",P<'T,l'n tagging :tll thr structures as they were removed. Vismdization was exeellent, and the exploration ,vas therefore cxknded beyond the mid]i1w and carried to a point below the bifurcation of the aorta. The noclc s superior to 0
corm
the right renal pedicle and the adjacent tissue were extensively replaced by niesoderm:d tumor (fig. 2,B). AJl other nodes wern free of tumor. The patient received 20 mg. nitrogen mustard in an attempt to destroy any tumor cells which might have been disseminated into the bloocl. stream. The surgery :1nd ehernothernpy wr:rc tolerated well. He wr1s dismissecl from the ho:c; pita] on the eleventh postoperative in e;;cdlent condition. Radiation tbernpy was hcgun on the fourteenth postoperative cfay. Over a period of 3G he receivedapproxirnatdy3500 rads ton, 9 12 cm" abdominal field. This included the hilum of tlw right kidney and extended to the left of the midline and superiorly to the symphysis pubis. He was treatc,d at 70 cm. distance, 250 K.V., 1.."J mm
Fro. 1. Cross specinwn. Solid tumor involves spernrntic eon! a11cl r,pidiclyrnis but distinct from testicular p:uenchym:1.
Cu. h.v.l. starting with a dose of 50 rads and increasing the dose increment to a maximum ol 200 rads daily. This patient ,n1s last seen on December fl.. 1959, which was 18 months following surgery His blood count and sedimentation rate wen, normal. ChPst films and bone snrveys did not demonstrate metastasis. He :,ppearecl to be in c,xcdlrnt heitlth and was maintaining n, normal symmetrical growth pr1ttenL
]'re+. 2. Photomicrogrn]lhS. /I, note ov:d to spindle shaped tumor cells with vesicular,
nuclei and numerous mitotic hgnres. Cross-striations are evident in some areas (arrow). dermal tumor cells in renal J,iln.r lymph nodes :we! extending into adjacent adipose tissue,
564
CARY P. GRAY AND C.\RL L. BIOR~ DISCUSSION
The results of the study and findings of these cases appear to indicate that contrary to past and present opinions 4 , 5, there are a certain number of patients with myosarcomas of the genital organs, who, because of lymphatic dissemination of tumor cells, should have radical surgical treatment and irradiation therapy, as herein described. It is apparently incorrect to assume that all of these tumors spread by blood vascular channels. Obviously, if preoperative studies reveal evidence of hematogenous spread, then orchiectomy with high ligation of the cord 4 Kohler, F, P, and Murphy, J. J.: Rhabdomyosarcoma of the male genital tract. J. Urol.
82: 500-503, 1959. ' ~ Satter, E. J., Heidner, F. C. and Wear, J. F.:
Pnmary sarcoma of the spermatic cord and epididymis. J. Urol., 82: 148-154, 1959.
uncl deep irradiation therapy would be the treatment of choice. It is our opinion that the use of nitrogen mustt1rd t1t the termination of surgery nmy well be 1111 important ,1djunct to surgical therapy. CONCLUSION
J\Iyosarcomas of the spermatic cord may metastasize by lymphatic as well as vascular channels. ·without evidenee of vascular spread, the procedure of choice is dissection of the retroperitoneal lymphatic vessels ,1nd lymph nodes and the removal of the entire spermatic cord to its point of origin. A thoracico-abdominal t1pproach is preferable in children. Irradiation to tolerance is an important adjunct to resection. It m,1y well be that anti-cancer chemotherapy will provide an additional advantage in the attempt to eradicate this disease.