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Leiomyosarcoma of the Scrotum in a man who had received Scrotal Irradiation as a Child
0022-534 7/88/1391-0136$02.00/0 THE JOURNAL OF UROLOGY
Vol. 139, January
Copyright© 1988 by The Williams & Wilkins Co.
Printed in U.S.A.
LEIOMYOSARCOMA OF THE SCROTUM IN A MAN WHO HAD RECEIVED SCROTAL IRRADIATION AS A CHILD DANIEL P. DALTON, ALAN M. RUSHOVICH, THOMAS A. VICTOR
AND
RICHARD LARSON
Departments of Urology, Pathology and General Surgery, Northwestern University Medical School, Chicago and Evanston Hospital, Evanston, Illinois
ABSTRACT
We report a rare case of a radiation-induced leiomyosarcoma of the scrotal wall. (J. Ural., 139: 136-138, 1988)
Leiomyosarcomas of the scrotal wall are rare with an extensive review of the literature revealing 8 previously reported cases. 1- 7 Cooney, although not given credit previously, reported the first case in 1931. 1 In addition, Siegal and Gaffey described a 35-year-old man with recurrent leiomyomas of the scrotum treated with local excisions, irradiation and radium in whom a leiomyosarcoma eventually developed. 8 We report an additional case of leiomyosarcoma of the scrotum in a man who had received scrotal irradiation as a child.
DISCUSSION
This case represents the tenth reported leiomyosarcoma arising from the scrotal wall (that is not arising from the testicular tunics, epididymis or spermatic cord) and the second reported case with local irradiation as a probable etiological factor. Our review of the literature on this subject is presented in the table. When confronted with such uncommon neoplasms, the surgeon must base his treatment plan on basic anatomical princi-
CASE REPORT
M. B., a 34-year-old white man, was seen for excision of lipomas of the abdominal wall and right thigh. He also complained of some scrotal itching, and a small, benign-appearing nodule was found on the right hemiscrotum. Medical history was significant for a course of radiation therapy to treat a scrotal hemangioma in 1951 (fig. 1). A total dose of 840 rad was delivered in 2 treatments within 3½ months. Physical examination was significant for lipomas and a freely mobile, 1 cm. nodule in the right hemiscrotum. The nodule was distinct from the deeper scrotal contents and there was no associated erythema or ulceration. No inguinal adenopathy was appreciated. Laboratory evaluation, including a complete blood count, urinalysis, electrolytes, liver function tests and a chest x-ray, was normal. The patient underwent local excision of the lipomas and the scrotal wall nodule on March 14, 1985. Pathological examination confirmed the soft tissue lesions to be lipomas whereas the scrotal nodule was a cutaneous leiomyosarcoma. Wide local excision was performed 1 week later with no residual tumor noted histologically. The patient was well 18 months postoperatively with no evidence of local recurrence.
FIG. 1. Appearance of scrotal hemangioma before radiation therapy (1951).
PATHOLOGICAL FINDINGS
The scrotal biopsy specimen was an ellipse of skin containing a tan/white dermal nodule measuring 1 x 0.5 x 0.5 cm. Microscopically, a cellular, spindle-celled tumor, consistent with a leiomyosarcoma, was seen in the deep dermis (fig. 2). The tumor extended into the subcutaneous tissue. Repeat excision of the biopsy site showed no residual tumor but it did reveal proliferation of smooth muscle nests in the mid dermis. Focally the smooth muscle in an arteriole showed significant nuclear atypia. Electron micrographs on formalin-fixed tissue revealed cytoplasmic intermediate filaments (fig. 3). In addition, the specimen was stained using an avitin-biotin immunoperoxidase technique with anti-desmin. The positive stain further supports the diagnosis of leiomyosarcoma. Accepted for publication June 2, 1987.
FIG. 2. Typical spindle cell appearance with mitotic figures. Reduced from XlOO.
136
137
LEIOMYOSARCOMA OF SCROTUM
ples and knowledge of the behavior of histologically similar neoplasms arising elsewhere in the body. Leiomyosarcomas of the scrotal wall most likely arise from the tunica dartos scroti, although such a neoplasm could occur in any smooth musclecontaining structure (such as arrectores pilorum muscles). Unlike the para-aortic and retroperitoneal lymphatic drainage of the testicle and spermatic cord, that of the scrotal wall primarily is to the inguinal, internal and external iliac nodes. Of the 3 previously reported patients with adequate followup information inguinal nodal metastases developed in 2 (1 at 8 years and 1 at 1 year postoperatively). 2• 4 •8 As others have mentioned, an aggressive approach with prophylactic inguinal node dissection may be beneficial in selected cases. 3 • 4 • 6 Leiomyosarcomas of the scrotal wall may be classified as a lesion of the superficial soft tissue. Fields and Helwig described differing biological behavior of such neoplasms based on the involvement of the subcutaneous tissues. 9 Of the 65 cases of cutaneous leiomyosarcomas described 40 per cent exhibited local recurrence after wide local excision. A higher rate of
FIG. 3. Electron micrograph reveals cytoplasmic intermediate filaments. Inset shows magnified view of filaments. Reduced from X24,000.
recurrence (4 7 per cent) was noted in those cutaneous lesions with extension into the subcutis. However, none developed lymphatic or hematogenous metastases. In marked contrast was the observed 50 per cent recurrence and 33 per cent incidence of hematogenous metastases in the cases with lesions arising from the subcutaneous tissues. The biologically benign behavior of cutaneous leiomyosarcomas compared to the malignant potential of subcutaneous lesions makes this distinction of primary importance. Our case falls into the more favorable category of cutaneous leiomyosarcomas. Of note in the aforementioned study is the exclusion of lesions arising in the "genital skin", a decision for which no explanation is supplied. To evaluate the potential role of adjuvant therapy one must research the literature concerning soft tissue sarcomas. In a study of 100 patients with primary soft tissue sarcomas of the extremities at the M. D. Anderson Hospital wide local excision with adjuvant radiation therapy (5,000 to 7,000 rad) was evaluated. 10 The 80 per cent local recurrence rate noted in historical controls was reduced to 13 per cent. Except for childhood rhabdomyosarcomas adjuvant chemotherapy has met with limited success. 11 Thus, adjuvant radiotherapy may be indicated in patients with leiomyosarcomas arising in the subcutaneous tissues of the scrotum. It is well known that a variety of neoplasms may develop as a result of therapeutic radiation. In 1948 Cahan and associates outlined the necessary criteria to categorize a malignancy as radiation induced: the neoplasm in question must arise within the field of prior irradiation, a relatively long latent period should be present and the neoplasm should be histologically proved. 12 Our case meets these requirements and most likely represents a radiation-induced leiomyosarcoma. In a review of leiomyomas arising from the tunica dartos scroti Siegal and Gaffey described a patient seen initially in 1914 with a leiomyoma treated with repeated excisions and radiation treatments (see table). 8 The eventual development of a leiomyosarcoma (photomicrographs not published) in this patient represents to our knowledge the first case of radiation-induced leiomyosarcoma of the scrotum. Leiomyosarcoma of the scrotal wall remains a rare lesion. Wide local excision and routine followup appear to be adequate
Reported cases of leiomyosarcoma of the scrotum Reference
Pt. Age
Duration of Symptoms
Treatment
Cooney' Immergut and associates 2
62 38
7½ mos. 4 yrs.
Local excision Local excision
Sikorowa and Proniewicz 3
43
3 yrs.
64
2 yrs.
Ray and associates 4
67
"Many" yrs.
Ashraf and associates' Johnson and associates6
60 89
6 mos. 3 mos.
Tada and associates 7 Siegal and Gaffey'*
58 35
6 mos. Unknown
Present case*
35
1 yr.
Rt. hemiscrotectomy, excision rt. testis and adnexa Lt. hemiscrotectomy, excision It. testis Local excision, scrotectomy, excision of testes and adnexae 2 mos. later owing to local recurrence Lt. hemiscrotectomy Excisional biopsy, wide local excision Wide local excision Excision leiomyoma and local irradiation; excision recurrent leiomyoma and local irradiation 3 yrs. later; radium treatment of local recurrence 2 yrs. later; lt. hemiscrotectomy, orchiectomy, radium treatment for multiple recurrent leiomyoma 1 yr. later; radium treatment for biopsy-proved recurrent leiomyoma 1 yr. later; biopsy-proved leiomyosarcoma, wide local excision and local irradiation Local excision
* Cases with local irradiation as a possible etiological factor.
Clinical Course Alive 1 yr. postop. Died 2½ yrs. postop. with metastatic disease No followup No fol!owup Died 8 yrs. 9 mos. postop. with metastatic disease
Alive 1 yr. postop. Died 1 mo. postop. of unrelated disease Alive 1 mo. postop. Died 15 yrs. after last procedure with metastatic disease
Alive 18 mos. postop. with no evidence of recurrence
~-,-
138
DALTON AND ASSOCIATES
treatment for cutaneous leiomyosarcomas, whereas more aggressive approaches, including prophylactic inguinal node dissections and perhaps radiation therapy, may be warranted for those arising in the subcutaneous tissue. Furthermore, our case emphasizes the importance of a history of local irradiation when evaluating scrotal lesions. REFERENCES 1. Cooney, J. D.: Leiomyosarcoma of scrotum. Urol. Cutan. Rev., 35: 487, 1931. 2. Immergut, S., Epstein, S., LeVY, G. and Cottier, Z. R.: Leiomyosarcoma of scrotum: case report. J. Urol., 93: 479, 1965. 3. Sikorowa, L. and Proniewicz, K.: Leiomyosarcoma scroti. Nowotwory, 19: 117, 1969. 4. Ray, B., Huvos, A.G. and Whitmore, W. F., Jr.: Unusual malignant tumors of the scrotum: review of 5 cases. J. Urol., 108: 760, 1972.
5. Ashraf, S. M., Tyagi, S. P. and Ansari, B. A.: Leiomyosarcoma of the scrotum. South Afr. Med. J., 42: 97, 1978. 6. Johnson, S., Rundell, M. and Platt, W.: Leiomyosarcoma of the scrotum: a case report with electron microscopy. Cancer, 41: 1830, 1978. 7. Tada, Y., Hashinaka, Y., Kadowaki, T., Takasugi, Y., Shin, T. and Koto, K.: Leiomyosarcoma of the scrotum: a case report. Hinjokika Hiyo, 26: 889, 1980. 8. Siegal, G. P. and Gaffey, T. A.: Solitary leiomyomas arising from the tunica dartos scroti. J. Urol., 116: 69, 1976. 9. Fields, J.P. and Helwig, E. B.: Leiomyosarcoma of the skin and subcutaneous tissue. Cancer, 4 7: 156, 1981. 10. Carabell, S. C. and Goodman, R. L.: Radiation therapy for soft tissue sarcoma. Sem. Oncol., 8: 201, 1981. 11. Sutow, W. W. and Maurer, H. M.: Chemotherapy of sarcomas-a perspective. Sem. Oncol., 8: 207, 1981. 12. Cahan, W. G., Woodard, H. Q., Higinbotham, N. L., Stewart, F. W. and Coley, B. L.: Sarcoma arising in irradiated bone. Report of eleven cases. Cancer, 1: 3, 1948.
Vol. 139, January
Copyright© 1988 by The Williams & Wilkins Co.
Printed in U.S.A.
LEIOMYOSARCOMA OF THE SCROTUM IN A MAN WHO HAD RECEIVED SCROTAL IRRADIATION AS A CHILD DANIEL P. DALTON, ALAN M. RUSHOVICH, THOMAS A. VICTOR
AND
RICHARD LARSON
Departments of Urology, Pathology and General Surgery, Northwestern University Medical School, Chicago and Evanston Hospital, Evanston, Illinois
ABSTRACT
We report a rare case of a radiation-induced leiomyosarcoma of the scrotal wall. (J. Ural., 139: 136-138, 1988)
Leiomyosarcomas of the scrotal wall are rare with an extensive review of the literature revealing 8 previously reported cases. 1- 7 Cooney, although not given credit previously, reported the first case in 1931. 1 In addition, Siegal and Gaffey described a 35-year-old man with recurrent leiomyomas of the scrotum treated with local excisions, irradiation and radium in whom a leiomyosarcoma eventually developed. 8 We report an additional case of leiomyosarcoma of the scrotum in a man who had received scrotal irradiation as a child.
DISCUSSION
This case represents the tenth reported leiomyosarcoma arising from the scrotal wall (that is not arising from the testicular tunics, epididymis or spermatic cord) and the second reported case with local irradiation as a probable etiological factor. Our review of the literature on this subject is presented in the table. When confronted with such uncommon neoplasms, the surgeon must base his treatment plan on basic anatomical princi-
CASE REPORT
M. B., a 34-year-old white man, was seen for excision of lipomas of the abdominal wall and right thigh. He also complained of some scrotal itching, and a small, benign-appearing nodule was found on the right hemiscrotum. Medical history was significant for a course of radiation therapy to treat a scrotal hemangioma in 1951 (fig. 1). A total dose of 840 rad was delivered in 2 treatments within 3½ months. Physical examination was significant for lipomas and a freely mobile, 1 cm. nodule in the right hemiscrotum. The nodule was distinct from the deeper scrotal contents and there was no associated erythema or ulceration. No inguinal adenopathy was appreciated. Laboratory evaluation, including a complete blood count, urinalysis, electrolytes, liver function tests and a chest x-ray, was normal. The patient underwent local excision of the lipomas and the scrotal wall nodule on March 14, 1985. Pathological examination confirmed the soft tissue lesions to be lipomas whereas the scrotal nodule was a cutaneous leiomyosarcoma. Wide local excision was performed 1 week later with no residual tumor noted histologically. The patient was well 18 months postoperatively with no evidence of local recurrence.
FIG. 1. Appearance of scrotal hemangioma before radiation therapy (1951).
PATHOLOGICAL FINDINGS
The scrotal biopsy specimen was an ellipse of skin containing a tan/white dermal nodule measuring 1 x 0.5 x 0.5 cm. Microscopically, a cellular, spindle-celled tumor, consistent with a leiomyosarcoma, was seen in the deep dermis (fig. 2). The tumor extended into the subcutaneous tissue. Repeat excision of the biopsy site showed no residual tumor but it did reveal proliferation of smooth muscle nests in the mid dermis. Focally the smooth muscle in an arteriole showed significant nuclear atypia. Electron micrographs on formalin-fixed tissue revealed cytoplasmic intermediate filaments (fig. 3). In addition, the specimen was stained using an avitin-biotin immunoperoxidase technique with anti-desmin. The positive stain further supports the diagnosis of leiomyosarcoma. Accepted for publication June 2, 1987.
FIG. 2. Typical spindle cell appearance with mitotic figures. Reduced from XlOO.
136
137
LEIOMYOSARCOMA OF SCROTUM
ples and knowledge of the behavior of histologically similar neoplasms arising elsewhere in the body. Leiomyosarcomas of the scrotal wall most likely arise from the tunica dartos scroti, although such a neoplasm could occur in any smooth musclecontaining structure (such as arrectores pilorum muscles). Unlike the para-aortic and retroperitoneal lymphatic drainage of the testicle and spermatic cord, that of the scrotal wall primarily is to the inguinal, internal and external iliac nodes. Of the 3 previously reported patients with adequate followup information inguinal nodal metastases developed in 2 (1 at 8 years and 1 at 1 year postoperatively). 2• 4 •8 As others have mentioned, an aggressive approach with prophylactic inguinal node dissection may be beneficial in selected cases. 3 • 4 • 6 Leiomyosarcomas of the scrotal wall may be classified as a lesion of the superficial soft tissue. Fields and Helwig described differing biological behavior of such neoplasms based on the involvement of the subcutaneous tissues. 9 Of the 65 cases of cutaneous leiomyosarcomas described 40 per cent exhibited local recurrence after wide local excision. A higher rate of
FIG. 3. Electron micrograph reveals cytoplasmic intermediate filaments. Inset shows magnified view of filaments. Reduced from X24,000.
recurrence (4 7 per cent) was noted in those cutaneous lesions with extension into the subcutis. However, none developed lymphatic or hematogenous metastases. In marked contrast was the observed 50 per cent recurrence and 33 per cent incidence of hematogenous metastases in the cases with lesions arising from the subcutaneous tissues. The biologically benign behavior of cutaneous leiomyosarcomas compared to the malignant potential of subcutaneous lesions makes this distinction of primary importance. Our case falls into the more favorable category of cutaneous leiomyosarcomas. Of note in the aforementioned study is the exclusion of lesions arising in the "genital skin", a decision for which no explanation is supplied. To evaluate the potential role of adjuvant therapy one must research the literature concerning soft tissue sarcomas. In a study of 100 patients with primary soft tissue sarcomas of the extremities at the M. D. Anderson Hospital wide local excision with adjuvant radiation therapy (5,000 to 7,000 rad) was evaluated. 10 The 80 per cent local recurrence rate noted in historical controls was reduced to 13 per cent. Except for childhood rhabdomyosarcomas adjuvant chemotherapy has met with limited success. 11 Thus, adjuvant radiotherapy may be indicated in patients with leiomyosarcomas arising in the subcutaneous tissues of the scrotum. It is well known that a variety of neoplasms may develop as a result of therapeutic radiation. In 1948 Cahan and associates outlined the necessary criteria to categorize a malignancy as radiation induced: the neoplasm in question must arise within the field of prior irradiation, a relatively long latent period should be present and the neoplasm should be histologically proved. 12 Our case meets these requirements and most likely represents a radiation-induced leiomyosarcoma. In a review of leiomyomas arising from the tunica dartos scroti Siegal and Gaffey described a patient seen initially in 1914 with a leiomyoma treated with repeated excisions and radiation treatments (see table). 8 The eventual development of a leiomyosarcoma (photomicrographs not published) in this patient represents to our knowledge the first case of radiation-induced leiomyosarcoma of the scrotum. Leiomyosarcoma of the scrotal wall remains a rare lesion. Wide local excision and routine followup appear to be adequate
Reported cases of leiomyosarcoma of the scrotum Reference
Pt. Age
Duration of Symptoms
Treatment
Cooney' Immergut and associates 2
62 38
7½ mos. 4 yrs.
Local excision Local excision
Sikorowa and Proniewicz 3
43
3 yrs.
64
2 yrs.
Ray and associates 4
67
"Many" yrs.
Ashraf and associates' Johnson and associates6
60 89
6 mos. 3 mos.
Tada and associates 7 Siegal and Gaffey'*
58 35
6 mos. Unknown
Present case*
35
1 yr.
Rt. hemiscrotectomy, excision rt. testis and adnexa Lt. hemiscrotectomy, excision It. testis Local excision, scrotectomy, excision of testes and adnexae 2 mos. later owing to local recurrence Lt. hemiscrotectomy Excisional biopsy, wide local excision Wide local excision Excision leiomyoma and local irradiation; excision recurrent leiomyoma and local irradiation 3 yrs. later; radium treatment of local recurrence 2 yrs. later; lt. hemiscrotectomy, orchiectomy, radium treatment for multiple recurrent leiomyoma 1 yr. later; radium treatment for biopsy-proved recurrent leiomyoma 1 yr. later; biopsy-proved leiomyosarcoma, wide local excision and local irradiation Local excision
* Cases with local irradiation as a possible etiological factor.
Clinical Course Alive 1 yr. postop. Died 2½ yrs. postop. with metastatic disease No followup No fol!owup Died 8 yrs. 9 mos. postop. with metastatic disease
Alive 1 yr. postop. Died 1 mo. postop. of unrelated disease Alive 1 mo. postop. Died 15 yrs. after last procedure with metastatic disease
Alive 18 mos. postop. with no evidence of recurrence
~-,-
138
DALTON AND ASSOCIATES
treatment for cutaneous leiomyosarcomas, whereas more aggressive approaches, including prophylactic inguinal node dissections and perhaps radiation therapy, may be warranted for those arising in the subcutaneous tissue. Furthermore, our case emphasizes the importance of a history of local irradiation when evaluating scrotal lesions. REFERENCES 1. Cooney, J. D.: Leiomyosarcoma of scrotum. Urol. Cutan. Rev., 35: 487, 1931. 2. Immergut, S., Epstein, S., LeVY, G. and Cottier, Z. R.: Leiomyosarcoma of scrotum: case report. J. Urol., 93: 479, 1965. 3. Sikorowa, L. and Proniewicz, K.: Leiomyosarcoma scroti. Nowotwory, 19: 117, 1969. 4. Ray, B., Huvos, A.G. and Whitmore, W. F., Jr.: Unusual malignant tumors of the scrotum: review of 5 cases. J. Urol., 108: 760, 1972.
5. Ashraf, S. M., Tyagi, S. P. and Ansari, B. A.: Leiomyosarcoma of the scrotum. South Afr. Med. J., 42: 97, 1978. 6. Johnson, S., Rundell, M. and Platt, W.: Leiomyosarcoma of the scrotum: a case report with electron microscopy. Cancer, 41: 1830, 1978. 7. Tada, Y., Hashinaka, Y., Kadowaki, T., Takasugi, Y., Shin, T. and Koto, K.: Leiomyosarcoma of the scrotum: a case report. Hinjokika Hiyo, 26: 889, 1980. 8. Siegal, G. P. and Gaffey, T. A.: Solitary leiomyomas arising from the tunica dartos scroti. J. Urol., 116: 69, 1976. 9. Fields, J.P. and Helwig, E. B.: Leiomyosarcoma of the skin and subcutaneous tissue. Cancer, 4 7: 156, 1981. 10. Carabell, S. C. and Goodman, R. L.: Radiation therapy for soft tissue sarcoma. Sem. Oncol., 8: 201, 1981. 11. Sutow, W. W. and Maurer, H. M.: Chemotherapy of sarcomas-a perspective. Sem. Oncol., 8: 207, 1981. 12. Cahan, W. G., Woodard, H. Q., Higinbotham, N. L., Stewart, F. W. and Coley, B. L.: Sarcoma arising in irradiated bone. Report of eleven cases. Cancer, 1: 3, 1948.