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Leiomyosarcoma fo the Bladder and Subsequent Urethral Recurrence
002:2-3:147/81112ii4-05Fn.0W2.00/0 Vol. 125.
T=--1E JcuR:SJAL o? UROLO{;·y
Copyright© 1981 by The YVilliarns & V\lilkins Co.
Printed in
LEIOMYOSARCOMA OF THE BLADDER AND SUBSEQUENT URETHRAL RECURRENCE A. MICHAEL ALABASTER, WILLIS P. JORDAN, JR., MARK S. SOLOWAY, RONALD M. SHIPPEL AND JOSEPH M. YOUNG From the Urology Section and Laboratory Seruice, Veterans Administration Medical Center, the Department of Urology and The University of Tennessee Center for the Health Sciences, Memphis, Tennessee
ABSTRACT
A case of leiomyosarcoma of the bladder with subsequent urethral recurrence is presented. Although it is well documented that urethral recurrence, or more likely new occurrence, is not uncommon following transitional cell carcinoma of the bladder, we believe that this is the first reported instance of a sarcoma of the bladder and urethral recurrence. Therefore, management of sarcoma of the bladder might include distal urethrectomy at the time of cystoprostatectomy. The risk of occurrence of a new primary tumor in the urethral remnant following cystoprostatectomy for transitional cell carcinoma is of sufficient magnitude to consider concomitant prophylactic urethrectomy. When confronted with the infrequent sarcoma of the bladder, however, there are little data to provide guide lines on the management of the urethra. Herein we report a case of urethral recurrence following cystoprostatectomy for leiomyosarcoma of the bladder. CASE REPORT
A 54-year-old white man presented with a 3-month history of lower tract obstructive symptoms and painless hematuria. Excretory urography revealed a filling defect in the base of the bladder. Endoscopic examination showed a friable tumor arising from the trigone. A biopsy revealed leiomyosarcoma. The patient underwent radical cystoprostatectomy with ileal conduit diversion and the postoperative course was uneventful. However, 5 months later he had bloody discharge from the external urethral meatus. The patient also noticed tissue passing from the meatus and examination revealed a protruding mass from the external meatus. Biopsy confirmed this to be leiomyosarcoma. The remainder of the urethra was excised in continuity with a distal penectomy. The first specimen was a radical resection of the bladder and prostate. When the bladder was opened the prostatic bed was hemorrhagic and necrotic (fig. 1). Multiple sections through this area disclosed a poorly differentiated mesodermal tumor with a tendency to form fascicles. There were numerous giant tumor cells and an abundance of mitoses (fig. 2). Hemorrhage and necrosis were extensive. stains indicated the tumor to be of smooth muscle origin, presumably from the bladder wall, since review of the sections of the original transurethral biopsies did not show prostatic involvement. No cross-striations were identified in the sections. There was no extension of tumor beyond the bladder-prostate region and no lymph nodes were involved. The second specimen was the remaining segment of urethra and distal portion of the penis (fig. 3). Hemorrhagic tissue protruded from the urethral meatus. A fungating mass was present in the distal urethra, which included the glans. Histologic sections disclosed a mesodermal tumor similar to the original bladder tumor, with even better defined fascicle formation and somewhat fewer mitoses. A Masson trichrome stain was characteristic of smooth muscle origin (fig. 4).
histologic type of bladder tumor after epithelial tumors. Sarcomas account for 0.38 to 0.67 per cent of all bladder tumors. 1 They are more common in men than women, with a ratio of 2:1. Sarcomas are observed more frequently in younger age groups but bladder sarcoma seems to be an exception. In 1 series of 14 patients 58 per cent were >45 years old." In 1959 Powers and associates reviewed the literature and found 324 cases of sarcoma of the bladder.i Because of the many synonyms and related terms it has been suggested that the generic term myosarcoma be used. Virtually, all the myosarcomas have a poor prognosis. In the largest collections
DISCUSSION
Malignant tumors of muscle are the second most frequent Accepted for publication July 18, 1980.
Frc. 1. A, bladder showing hemorrhagic and necrotic trigone area. B, closer view of bladder reveals hemorrhagic and necrotic trigone area.
584
ALABASTER AND ASSOCIATES
Fm. 2. A, low power view of sarcoma with some fascicle formation and spindle cell pattern. B, high power view shows marked pleomorphism
Fm. 3. A, resected urethra and distal penis with mass. B, distal penis showing necrotic tumor in urethra and meatal region.
reported leiomyosarcoma and rhabdomyosarcoma were the most common types. 3 Leiomyosarcoma is synonymous with malignant leiomyosar-
coma, myosarcoma, spindle cell sarcoma and metastasizing leiomyoma. This tumor usually is composed of smooth muscle cells. The most common age at presentation is ~40 years. This tumor may occur anywhere in the bladder but it tends to be a broad-based solid mass located off the trigone in a distensible area of the bladder. 4 The histologic features usually consist of spindle-shaped, elongated tumor cells in parallel bundles. The prognosis, although poor, is better than for rhabdomyosarcoma. It also has been noted that survival of leiomyosarcoma of the bladder is better in female than in male subjects. 5 Rhabdomyosarcoma of the bladder is synonymous with botryoid sarcoma, myxomatous sarcoma and giant cell sarcoma. The adult type of rhabdomyosarcoma, when initially seen, usually is advanced and ulcerated, and may occur anywhere in the bladder. The histologic picture is extremely variable and may even assume a spindle cell type appearance. The prognosis is poor, although survival is better with multimodality treatment. Malignant mesodermal mixed tumors are highly malignant and composed of elements of carcinoma and differentiated sarcoma, usually chondrosarcoma or osteosarcoma. Synonyms include carcinosarcoma, adenosarcoma and miillerian mixed tumors. These tumors usually are found in older individuals. The histologic patterns are similar to the uterine and ovarian tumors and, therefore, they are referred to as miillerian mixed tumors. Local recurrence and extension to surrounding structures are more common than metastasis. Generally, myosarcomas are not radiosensitive and surgical extirpation is the only hope for
LEIOfAYOSARCOUKA OF BLADDER AND SlJBSEQDEioJT 7JRETHRA_L RECURRENCE
.585
FIG. 4. High power view of recurrent leiomyosarcoma
cure. Involvement of the urethra is not uncommon in transitional cell bladder carcinoma. Cordonnier and Spjut observed that approximately 4 per cent of patients with carcinoma of the bladder subsequently had carcinoma of the urethra. 6 Gowing found urethral carcinoma in 18 per cent of the patients who died of bladder carcinoma. 7 Experimental work has revealed that primary tumors of the bladder can spread to the penile urethra by the lymphatic route or by implantation of exfoliated tumor cells. There are different opinions concerning prophylactic urethrectomy in male patients with bladder cancer. Johnson and Guinn believed that safe and acceptable management included frequent urethroscopy and cytologic examination. 8 Others advise total urethrectomy for those tumors that involve the bladder neck or invade the prostatic urethra. 9 We reviewed the literature concerning sarcoma of the bladder and believe that this is the first reported case of recurrence in the distal urethra. Owing to the rarity of this tumor it is difficult to make definitive statements concerning prophylactic urethrectomy. Although urethrectomy usually is considered for transitional cell carcinoma of the bladder urethral recurrence was not considered a likely possibility in our case. However, we now believe that radical surgical excision of a leiomyosarcoma of the
bladder should include a urethrectomy if the patient's condition does not preclude the additional operative time. REFERENCES l. Flint, L. D. and Dick, V. S.: Myosarcoma of the urinary bladder:
2. 3. 4. 5. 6. 7. 8. 9.
preliminary report of a favorable case. Lahey Clinic Bull., 6: 181, 1949. Mackenzie, A. R., Whitmore, W. F., Jr. and Melamed, M. R.: Myosarcomas of the bladder and prostate. Cancer, 22: 833, 1968. Powers, J. H., Hawn, C. V. Z. and Carter, R. D.: Osteogenic sarcoma and transitional cell carcinoma occurring simultaneously in the urinary bladder. J. Urol., 76: 263, 1959. McCrea, L. E. and Post, E. A.: Sarcoma of bladder. Urol. Sur., 5: 307, 1955. Tara, H. H. and Mentus, N. L.: Leiomyosarcoma of urinary bladder. Urology, 2: 460, 1973. Cordonnier, J. J. and Spjut, H. J.: Urethral occurrence of bladder carcinoma following cystectomy. J. Urol., 87: 398, 1962. Gowing, N. F.: Urethral carcinoma associated with cancer of the bladder. Brit. J. Urol., 32: 428, 1960. Johnson, D. E. and Guinn, G. A.: Surgical management of urethral carcinoma occurring after cystectomy. J. Urol., Hl3: 314, 1970. Riches, E.W. and Cullen, T. H.: Carcinoma of the urethra. Brit. J. Urol., 23: 209, 1951.
T=--1E JcuR:SJAL o? UROLO{;·y
Copyright© 1981 by The YVilliarns & V\lilkins Co.
Printed in
LEIOMYOSARCOMA OF THE BLADDER AND SUBSEQUENT URETHRAL RECURRENCE A. MICHAEL ALABASTER, WILLIS P. JORDAN, JR., MARK S. SOLOWAY, RONALD M. SHIPPEL AND JOSEPH M. YOUNG From the Urology Section and Laboratory Seruice, Veterans Administration Medical Center, the Department of Urology and The University of Tennessee Center for the Health Sciences, Memphis, Tennessee
ABSTRACT
A case of leiomyosarcoma of the bladder with subsequent urethral recurrence is presented. Although it is well documented that urethral recurrence, or more likely new occurrence, is not uncommon following transitional cell carcinoma of the bladder, we believe that this is the first reported instance of a sarcoma of the bladder and urethral recurrence. Therefore, management of sarcoma of the bladder might include distal urethrectomy at the time of cystoprostatectomy. The risk of occurrence of a new primary tumor in the urethral remnant following cystoprostatectomy for transitional cell carcinoma is of sufficient magnitude to consider concomitant prophylactic urethrectomy. When confronted with the infrequent sarcoma of the bladder, however, there are little data to provide guide lines on the management of the urethra. Herein we report a case of urethral recurrence following cystoprostatectomy for leiomyosarcoma of the bladder. CASE REPORT
A 54-year-old white man presented with a 3-month history of lower tract obstructive symptoms and painless hematuria. Excretory urography revealed a filling defect in the base of the bladder. Endoscopic examination showed a friable tumor arising from the trigone. A biopsy revealed leiomyosarcoma. The patient underwent radical cystoprostatectomy with ileal conduit diversion and the postoperative course was uneventful. However, 5 months later he had bloody discharge from the external urethral meatus. The patient also noticed tissue passing from the meatus and examination revealed a protruding mass from the external meatus. Biopsy confirmed this to be leiomyosarcoma. The remainder of the urethra was excised in continuity with a distal penectomy. The first specimen was a radical resection of the bladder and prostate. When the bladder was opened the prostatic bed was hemorrhagic and necrotic (fig. 1). Multiple sections through this area disclosed a poorly differentiated mesodermal tumor with a tendency to form fascicles. There were numerous giant tumor cells and an abundance of mitoses (fig. 2). Hemorrhage and necrosis were extensive. stains indicated the tumor to be of smooth muscle origin, presumably from the bladder wall, since review of the sections of the original transurethral biopsies did not show prostatic involvement. No cross-striations were identified in the sections. There was no extension of tumor beyond the bladder-prostate region and no lymph nodes were involved. The second specimen was the remaining segment of urethra and distal portion of the penis (fig. 3). Hemorrhagic tissue protruded from the urethral meatus. A fungating mass was present in the distal urethra, which included the glans. Histologic sections disclosed a mesodermal tumor similar to the original bladder tumor, with even better defined fascicle formation and somewhat fewer mitoses. A Masson trichrome stain was characteristic of smooth muscle origin (fig. 4).
histologic type of bladder tumor after epithelial tumors. Sarcomas account for 0.38 to 0.67 per cent of all bladder tumors. 1 They are more common in men than women, with a ratio of 2:1. Sarcomas are observed more frequently in younger age groups but bladder sarcoma seems to be an exception. In 1 series of 14 patients 58 per cent were >45 years old." In 1959 Powers and associates reviewed the literature and found 324 cases of sarcoma of the bladder.i Because of the many synonyms and related terms it has been suggested that the generic term myosarcoma be used. Virtually, all the myosarcomas have a poor prognosis. In the largest collections
DISCUSSION
Malignant tumors of muscle are the second most frequent Accepted for publication July 18, 1980.
Frc. 1. A, bladder showing hemorrhagic and necrotic trigone area. B, closer view of bladder reveals hemorrhagic and necrotic trigone area.
584
ALABASTER AND ASSOCIATES
Fm. 2. A, low power view of sarcoma with some fascicle formation and spindle cell pattern. B, high power view shows marked pleomorphism
Fm. 3. A, resected urethra and distal penis with mass. B, distal penis showing necrotic tumor in urethra and meatal region.
reported leiomyosarcoma and rhabdomyosarcoma were the most common types. 3 Leiomyosarcoma is synonymous with malignant leiomyosar-
coma, myosarcoma, spindle cell sarcoma and metastasizing leiomyoma. This tumor usually is composed of smooth muscle cells. The most common age at presentation is ~40 years. This tumor may occur anywhere in the bladder but it tends to be a broad-based solid mass located off the trigone in a distensible area of the bladder. 4 The histologic features usually consist of spindle-shaped, elongated tumor cells in parallel bundles. The prognosis, although poor, is better than for rhabdomyosarcoma. It also has been noted that survival of leiomyosarcoma of the bladder is better in female than in male subjects. 5 Rhabdomyosarcoma of the bladder is synonymous with botryoid sarcoma, myxomatous sarcoma and giant cell sarcoma. The adult type of rhabdomyosarcoma, when initially seen, usually is advanced and ulcerated, and may occur anywhere in the bladder. The histologic picture is extremely variable and may even assume a spindle cell type appearance. The prognosis is poor, although survival is better with multimodality treatment. Malignant mesodermal mixed tumors are highly malignant and composed of elements of carcinoma and differentiated sarcoma, usually chondrosarcoma or osteosarcoma. Synonyms include carcinosarcoma, adenosarcoma and miillerian mixed tumors. These tumors usually are found in older individuals. The histologic patterns are similar to the uterine and ovarian tumors and, therefore, they are referred to as miillerian mixed tumors. Local recurrence and extension to surrounding structures are more common than metastasis. Generally, myosarcomas are not radiosensitive and surgical extirpation is the only hope for
LEIOfAYOSARCOUKA OF BLADDER AND SlJBSEQDEioJT 7JRETHRA_L RECURRENCE
.585
FIG. 4. High power view of recurrent leiomyosarcoma
cure. Involvement of the urethra is not uncommon in transitional cell bladder carcinoma. Cordonnier and Spjut observed that approximately 4 per cent of patients with carcinoma of the bladder subsequently had carcinoma of the urethra. 6 Gowing found urethral carcinoma in 18 per cent of the patients who died of bladder carcinoma. 7 Experimental work has revealed that primary tumors of the bladder can spread to the penile urethra by the lymphatic route or by implantation of exfoliated tumor cells. There are different opinions concerning prophylactic urethrectomy in male patients with bladder cancer. Johnson and Guinn believed that safe and acceptable management included frequent urethroscopy and cytologic examination. 8 Others advise total urethrectomy for those tumors that involve the bladder neck or invade the prostatic urethra. 9 We reviewed the literature concerning sarcoma of the bladder and believe that this is the first reported case of recurrence in the distal urethra. Owing to the rarity of this tumor it is difficult to make definitive statements concerning prophylactic urethrectomy. Although urethrectomy usually is considered for transitional cell carcinoma of the bladder urethral recurrence was not considered a likely possibility in our case. However, we now believe that radical surgical excision of a leiomyosarcoma of the
bladder should include a urethrectomy if the patient's condition does not preclude the additional operative time. REFERENCES l. Flint, L. D. and Dick, V. S.: Myosarcoma of the urinary bladder:
2. 3. 4. 5. 6. 7. 8. 9.
preliminary report of a favorable case. Lahey Clinic Bull., 6: 181, 1949. Mackenzie, A. R., Whitmore, W. F., Jr. and Melamed, M. R.: Myosarcomas of the bladder and prostate. Cancer, 22: 833, 1968. Powers, J. H., Hawn, C. V. Z. and Carter, R. D.: Osteogenic sarcoma and transitional cell carcinoma occurring simultaneously in the urinary bladder. J. Urol., 76: 263, 1959. McCrea, L. E. and Post, E. A.: Sarcoma of bladder. Urol. Sur., 5: 307, 1955. Tara, H. H. and Mentus, N. L.: Leiomyosarcoma of urinary bladder. Urology, 2: 460, 1973. Cordonnier, J. J. and Spjut, H. J.: Urethral occurrence of bladder carcinoma following cystectomy. J. Urol., 87: 398, 1962. Gowing, N. F.: Urethral carcinoma associated with cancer of the bladder. Brit. J. Urol., 32: 428, 1960. Johnson, D. E. and Guinn, G. A.: Surgical management of urethral carcinoma occurring after cystectomy. J. Urol., Hl3: 314, 1970. Riches, E.W. and Cullen, T. H.: Carcinoma of the urethra. Brit. J. Urol., 23: 209, 1951.