- Email: [email protected]
Sarcoma of the Prostate: A Report of Four Cases and a Review of Current Therapy
THE JOmtNAL OF UROLOGY
Vol. 89, No. 1 January 1963 Copyright © 1963 by The Williams & Wilkins Co. Printed in U.S.A.
SARCOMA OF THE PROSTATE: A REPORT OF FOUR CASES AND A REVIEW OF CURRENT THERAPY JERALD SIEGEL From the Division of Urology, Kingsbridge Veterans Administration Hospital, Bronx, N. Y.
The possibility that sarcomas existed in prostatic tissue was first suggested by Stafford of London in 1839 when he reported a case in a 5-year-old boy. Since that time there have been well over 250 cases reported in American and English literature. This paper will add 2 cases of leiomyosarcoma to the world's literature and bring the total of recorded cases to 28. An additional 2 cases of rhabdomyosarcoma will also be presented.
4. N eurogenic fibrosarcoma 5. Fibromyosarcoma2 PATHOLOGY
Rhabdomyosarcoma shows prominent cross and longitudinal striations with phosphotungstic and hematoxylin stains. The cells are large and some contain nuclei. In leiomyosarcoma one does not find striae, but interlacing masses of elongated cells. The nuclei are hyperchromatic and there is abundant, slightly acidophilic cytoplasm. Spindle cell and round cell sarcoma reveals a multitude of spindle or fusiform cells arranged in whorls or bundles, or round cells in solid masses. Myxomatous degeneration may occur. The lymphosarcomatous cells resemble large lymphoblasts which are uniform in size but do exhibit much anaplasia. The anaplastic and pluriplastic sarcomas have a tendency towards pleomorphism, demonstrating several types of cells and also giant cell formation. 2
EMBRYOLOGY
Sarcomas arise exclusively from stromal tissue consisting of muscular and fibrous elements from the mesodermal tissue of the genital cord (which contains the terminal portions of the wolffian and mullerian ducts), and from the circular muscle of the urogenital sinus. It is interesting to note that the muscular structure of the uterus is also derived from the genital cord and may be the originating point of leiomyomas and leiomyosarcomas in the adult.1 The intraprostatic sources are: 1) fibrous tissue, 2) smooth and striated muscle, and 3) interstitial lymphoid foci.
INCIDENCE
Sarcomas tend to be a disease of youth. The youngest reported case is in a 4-month-old child although the oldest case did occur in an 88-year-old man. In 80 per cent of the cases onset occurs before the appearance of benign prostatic hypertrophy. Melicow2 reported 4 cases ranging in age from 16 to 44. Costa 3 reported 25 cases of sarcoma of the prostate in children from O to 9 years of age. In his total of 84 cases, 66 were in male patients under the age of 50. In 1941, Prince and Vest4 reviewed the literature and presented 15 cases of leiomyosarcoma of the prostate, 33 per cent occurring before the age of 20 and a total of 60 per cent occurring
CLASSIFICATION
A. Isoplastic sarcoma 1. Fibrosarcoma (spindle cell and round cell sarcoma) 2. JVIyosarcoma (leiomyosarcoma, smooth muscle and rhabdomyosarcoma, striated muscle) 3. Lymphosarcoma (reticulum cell sarcoma) B. Metaplastic sarcoma C. Anaplastic and pluriplastic sarcoma 1. Angiosarcoma 2. Myxosarcoma 3. Choxondrosarcoma
2 Melicow,M.M., Pelton, T. H. and Fish, G. W.: Sarcoma of the prostate; review of the literature; table of classification; report of four cases. J. Urol., 49: 675-707, 1943. 3 Costa, A.: Sul sarcoma della prostato. Arch. Italian Chir., 21: 417-435, 1928. 4 Prince, C. L. and Vest, S. A.: Leiomyosarcoma of the prostate; report of a case and critical review. J. Urol., 46: 1129-1143, 1941.
Accepted for publication June 26, 1962. Read at the annual meeting, New York Section, American Urological Association, Inc., New York, N. Y., April 25, 1962. 1 Longley, J.: Sarcoma of the prostate and bladder. J. Urol., 73: 417-423, 1955. 78
SARCOMA OF PROSTATE
before the age of 50. In our current series, the ages represented are 24, 48, 56 and 68, respectively. At the Mayo Clinic, between 1940 and 1950, there were 5 sarcomas reported out of a total of 1500 cases of malignant prostatic disease, an incidence rate of 0.3 per cent. At the Squier Urological Clinic, between 1928 and 1942, the incidence of sarcoma was 1 : 1000. At the Veterans Administration Hospital, Bronx, N. Y., between January 1952 and December 1961, there were 4 sarcomas reported out of a total of 734 cases of malignant prostatic disease, an incidence rate of 0.5 per cent. This higher incidence rate may be attributed to the younger age group treated at this institution. Horstmann 5 in 1952 found one round cell sarcoma and 225 prostatic carcinomas in 31,863 autopsies. DIAGNOSIS
In the adult, the presenting symptoms of sarcoma of the prostate are those indicative of urinary tract obstruction. The pediatric patient will usually present in urinary retention, while the adult's initial complaint is usually dysuria. There is sometimes anterior extension of the tumor so as to present an anterior abdominal wall mass. With perineal extension the tumor may simulate a perineal abscess. Hematuria usually results when the bladder becomes infiltrated. On rectal examination prostatic carcinomas are fixed, rigid, hard and nodular. Upon passing the cystoscope carcinomas give the impression of rigidity. Prostatic sarcomas, however, are smooth, tense, and symmetrical, or soft and balloon-like in consistency, not unlike an abscess. It is frequently impossible to distinguish sarcomas from benign adenomas on rectal examination. The bone metastases of carcinoma are osteoblastic, while those of sarcoma are osteoclastic. 6 A study by Smith and Torgenson7 revealed that 40 per cent of the cases presented had distant metastases; 33½ per cent to bone (ribs and 5 Danckers, U. F., Philipsborn, H.F., Baylor, J.E. and Burkhead, H. C.: Sarcomas of the prostate, periprostatic tissue and bladder neck in children; report of a new case. Amer. J. Roentgenol., 84: 555-561, 1960. 6 Stirling, W. C. and Ash, J.E.: Sarcoma of the prostate. J. Urol., 41: 515-533, 1939. 7 Smith, R. R. and Torgenson, W. R.: Sarcoma of the prostate with a digest of the literature. Surg., Gynec. & Obst., 43: 328, 1926.
79
vertebrae); 33½ per cent to lungs; 23 per cent to the liver; 11.7 per cent to pleura; 20.5 per cent to regional lymph nodes; and 17.6 per cent to the spleen. Benign prostatic hypertrophy, prostatic abscess, carcinoma of the prostate, miillerian duct cyst, prostatic cyst, and retrovesical tumors must all be considered in evaluating a case of prostatic sarcoma. CASE REPORTS
Case 1. H. K., BVAH 232-245, a 48-year-old white man, first noticed the onset of symptoms of bladder neck obstruction in November 1957. The following month, after excessive alcoholic intake, the patient had a bout of acute urinary retention necessitating a suprapubic prostatectomy. February 1958, a transurethral prostatectomy was accomplished for increasing distress due to lower urinary tract obstruction. The microscopic diagnosis of leiomyosarcoma was established at this time. Three months later a suprapubic cystostomy was performed for retention. Subsequently the patient was started on radiotherapy, receiving 5700 r through multiple cross fire portals via the 1 million volt modality. Despite radiotherapy, the rectal mass increased in size. Metastases first became evident in February 1959 with the appearance of a nodular density in the third anterior intercostal space. Four months later the prostatic mass had infiltrated the rectum and both lung fields had multiple nodular densities. By May 1960 the patient had experienced a 30-pound weight loss and a draining perianal fistula was present. An excretory urogram revealed a massive, hazy density throughout the entire pelvis. One month later cutaneous ureterostomies were performed for bilateral ureteral obstruction. By the end of October fecal material drained from the suprapubic and perineal areas and also through a sacral decubitus ulcer. The patient died on November 8, 1960. Postmortem examination revealed leiomyosarcoma of the prostate with metastases to lungs and intestines and locally infiltrating the entire pelvis. Case 2. G. S., BVAH-A2240, a 68-year-old white man, had a 2-week history of hematuria and dysuria. March 1956, a transurethral resection of the bladder neck was performed for urethral obstruction. The microscopic diagnosis was anaplastic carcinoma. Two months later bilateral orchiectomy and prostatic biopsy were
80
JERALD SIEGEL
accomplished. The patient was then started on a regimen of diethylstilbesterol. The pathologic picture following this procedure was interpreted as spindle cell sarcoma. In October the patient complained of dysuria, frequency, urgency and
intermittent gross hematuria. His liver was now palpable 3 cm. beneath the right hypochondrium and his prostate gland was small, hard, nodular and fixed. A biopsy at this time revealed anaplastic leiomyosarcoma of the prostate (fig. 1).
Fm. 1. G. S., A-2240, leiomyosarcoma of prostate. A, X147. B, X462
81
SARCOMA OF PROSTATE
January 1957 he received 4000 r through anterior and posterior portals. He did well for the next 2 years on constant catheter drainage. He was re-admitted to the hospital in April 1959 with hematuria. Examination revealed 4 plus prostatic enlargement with extension, fixation, and loss of outline on the left side. A suprapubic cystostomy was accomplished but the patient's course was rapidly downhill and he died September 23, 1959 of uremia. Postmortem examination revealed leiomyosarcoma of the prostate without metastases and bilateral pyelonephritis and hydronephrosis. Case 3. P. M., BVAH 157-675, a 24-year-old white man, was admitted with a 7-month history of intermittent hematuria and bright red rectal bleeding. Rectal examination revealed a large, tender, grapefruit-sized mass, a biopsy of which, that included both rectal and bladder tissue, revealed rhabdomyosarcoma of the prostate (fig. 2). Shortly after admission a laparotomy was performed with the intention of radical extirpation of the tumor. However, a fixed, indurated mass on the right lateral wall of the prostate and bladder, incorporating the great vessels and adherent to the pelvic wall, was found. These findings made it impossible to carry out the intended procedure and bilateral cutaneous ureterostomies were performed. Two weeks later the patient was transferred to the neurosurgical service for palliative laminectomy because of intractable tenesmus. The patient died 6 weeks later. Postmortem examination revealed rhabdomyosarcoma of the prostate with extension to the bladder and to the floor of the pelvis and rectum, and metastasizing to the lungs, liver and both adrenal glands. Case 4. B. S., BVAH 197-734, a 58-year-old white man, underwent suprapubic prostatectomy in December of 1954 for signs and symptoms of lower tract obstruction. The microscopic diagnosis was rhabdomyosarcoma of the prostate. He did well until March 1955 when his symptoms reappeared. He subsequently received 7500 r to the prostate and bladder via the 2 million volt modality. At the conclusion of the radiotherapy he was admitted to the Bronx Veterans Administration Hospital with a 2-week history of incontinence and hernaturia. Physical examination revealed an expanding suprapubic mass, 2 by 2 cm. in size. The rectal lumen was 50 per cent occluded by a large mass. Panendoscopy re-
vealed a normal trigone with a large necrotic mass of prostate and bladder projecting into the lumen of the prostatic urethra. In a month the suprapubic mass doubled in size. Death came rapidly, approximately 1 year following the onset of symptoms. Postmortem findings were rhabdomyosarcoma of the prostate with extension to the bladder and underlying skin. TREATMENT
Opinions are varied as to the best modality of therapy for this disease. Many authors have condemned radical surgery for fear it hastens the patient's death. Radiotherapy has not been impressive but has its place as an adjunct to surgery. Smith8 reported a case in which the patient was alive and well for 2 ½ years after total perineal prostatectomy, vesiculectomy and 2100 r radiotherapy. Melicow states that surgery is usually only beneficial for the relief of obstruc-tion. He does admit that definitive surgery seems to be encouraging in cases of leiomyosarcorna. Radiotherapy remains the procedure of choice in the more radiosensitive lymphosarcoma. 2 Graves and Coleman9 treated a case of myxofibrosarcoma of the prostate with suprapubic enucleation. The patient died 6 ½ years later of oat cell carcinoma of the lungs. Postmortem examination revealed prostatic adenoma, but no residual sarcoma. Scardino and Prince 10 discussed two unusually long case survivals. The first patient with leiomyosarcoma of the prostate was reported by Prince and Vest in 1941, and treated by radical perineal prostatectomy. H,· was living and free of metastases 14 years later. The second case was diagnosed spindle cell sarcoma of the prostate and was likewise treated bv radical perineal prostatectomy. This patient ,;as alive and free of disease 4 years following his surgical procedure. Barone and .Joelsonll treated their patient with leiomyosarcoma by suprapubic prostatectomy and he was free of 8 Smith, G. G.: Three unusual cases of prostatic malignancy; adenocarcinoma with mucus cells: malignant lymphoma; leiomyosarcoma. J. Urol 70: 267, 1953. 9 Graves, R. S. and Coleman, JVI_. W.: Sarcoma of the prostate; an unusual survival. J. Urol 72: 731-734, 1954. _ 1o Scardino, P. L. and Prince, C._L.: Prostatic sarcoma· survival followmg radical permeal prostate~tomy. J. Urol., 72: 729-730,_1954 11 Barone, A. M. and Joelson, J. J_: Le10myosarcoma of the prostate; report of a case. J. Urol., 63: 533-538, 1950.
82
JERALD SIEGEL
Frn. 2. P. M., 157-675, rhabdomyosarcoma of prostate. A, Xl50. B, X450 disease for 28 months. Radiotherapy was not used, either pre- or postoperatively. Counseller12 treated his patient with x-ray and radium and reported a 17.5-year survival. He later modified 12 Counseller, V. S. and Bedard, R. E.: Sarcoma of the prostate gland. J. Urol., 43: 836-843, 1940.
his procedure with partial prostatectomy followed by radium and radiotherapy in the immediate postoperative period. In the past, Deans, Stevens, Barringer and Hess all agreed that radiotherapy was superior to surgery. However, a review of the literature indicates that definitive
SARCOMA OF PROSTATE
---
' - - - '' --~-- - -
therapy for sarcoma should be radical surgery. In our series of 4 cases, the longest survival was 3½ years (case 2) G. S,, who was treated by transurethral resection, orchiectomy and 4000 r. Case 1, H. K,, survived 3 years following suprapubic prostatectomy plus 5700 r. Case 3, P. M., survived 9 months without therapy and case 4, B, S., survived a year following suprapubic prostatectomy and 7500 r. There seem8 to be no uniformity of agreement as to the proper therapy required for patients with sarcoma of the prostate. Naturally each case has to be individualized as to histologic type of tumor, its degree of radiosensitivity, and extent of spread. Radiotherapy, in the postoperative period, does and shall continue to play an important role in sarcoma therapy. However, the definitive treatment nmst continue to be radical surgery, It behooves the modern urologist to maintain a high index of suspicion for this disease entity, since long-term survival seems to depend upon early diagnosis and radical removal of sarcomatous tissue, DISCUSSION
The first proven leiomyosarcoma of the prostate was established by Isambert13 in 1853. In the subsequent 109 years only 28 eases of that entity have been added to the literature, In 1960 Fitzpatrick and Stump 14 reviewed 25 cases of 13 Riba, L. W, and Wheelock, JVI. C.: Leiomyosarcoma of the prostate. J, Urol., 63: 162-164, 1950. 14 Fitzpatrick, T. J, and Stump, G.: Leiomyosarcoma of the prostate; case report and review of the literature, J. UroL, 83: 80-83, 1960,
leiomyosarcoma of the prostate and the reader is referred to this article for an excellent review. In 1960 Danchers and associates 5 reported a case of leiomyosarcoma in a 1-year-old white boy who had experienced dysuria and frequency for 2 weeks. Exploration disclosed a soft tumor attached to the base of the bladder. The child died in the early postoperative period, It is evident that in the young the course of the disease may be so rapid that local symptoms other than dysuria or partial obstruction are seldom encountered. SUMMARY
Sarcoma of the prostate is rare, but cannot be considered uncommon, Therapy of this disease is dependent upon the stage encountered when diagnosis is established, If discovered early, radical surgery with postoperative radiation is indicated. Later in the course of the disease, radiotherapy with surgery, utilized for palliating obstructive disease, is the method of choice. At the Bronx Veterans Administration Hospital there were 734 prostatic malignancies encountered within a 10-year period, four of which were myosarcomas. The author is grateful to Dr. .J. K. Lattimer for his instructive comments during the preparation of this paper and to JVIr. David Lubin and Mr, Glenroy Harahan of the Medical Illustration Division of the Bronx Veterans Administration Hospital for the preparation of the photomicrogra phs.
Vol. 89, No. 1 January 1963 Copyright © 1963 by The Williams & Wilkins Co. Printed in U.S.A.
SARCOMA OF THE PROSTATE: A REPORT OF FOUR CASES AND A REVIEW OF CURRENT THERAPY JERALD SIEGEL From the Division of Urology, Kingsbridge Veterans Administration Hospital, Bronx, N. Y.
The possibility that sarcomas existed in prostatic tissue was first suggested by Stafford of London in 1839 when he reported a case in a 5-year-old boy. Since that time there have been well over 250 cases reported in American and English literature. This paper will add 2 cases of leiomyosarcoma to the world's literature and bring the total of recorded cases to 28. An additional 2 cases of rhabdomyosarcoma will also be presented.
4. N eurogenic fibrosarcoma 5. Fibromyosarcoma2 PATHOLOGY
Rhabdomyosarcoma shows prominent cross and longitudinal striations with phosphotungstic and hematoxylin stains. The cells are large and some contain nuclei. In leiomyosarcoma one does not find striae, but interlacing masses of elongated cells. The nuclei are hyperchromatic and there is abundant, slightly acidophilic cytoplasm. Spindle cell and round cell sarcoma reveals a multitude of spindle or fusiform cells arranged in whorls or bundles, or round cells in solid masses. Myxomatous degeneration may occur. The lymphosarcomatous cells resemble large lymphoblasts which are uniform in size but do exhibit much anaplasia. The anaplastic and pluriplastic sarcomas have a tendency towards pleomorphism, demonstrating several types of cells and also giant cell formation. 2
EMBRYOLOGY
Sarcomas arise exclusively from stromal tissue consisting of muscular and fibrous elements from the mesodermal tissue of the genital cord (which contains the terminal portions of the wolffian and mullerian ducts), and from the circular muscle of the urogenital sinus. It is interesting to note that the muscular structure of the uterus is also derived from the genital cord and may be the originating point of leiomyomas and leiomyosarcomas in the adult.1 The intraprostatic sources are: 1) fibrous tissue, 2) smooth and striated muscle, and 3) interstitial lymphoid foci.
INCIDENCE
Sarcomas tend to be a disease of youth. The youngest reported case is in a 4-month-old child although the oldest case did occur in an 88-year-old man. In 80 per cent of the cases onset occurs before the appearance of benign prostatic hypertrophy. Melicow2 reported 4 cases ranging in age from 16 to 44. Costa 3 reported 25 cases of sarcoma of the prostate in children from O to 9 years of age. In his total of 84 cases, 66 were in male patients under the age of 50. In 1941, Prince and Vest4 reviewed the literature and presented 15 cases of leiomyosarcoma of the prostate, 33 per cent occurring before the age of 20 and a total of 60 per cent occurring
CLASSIFICATION
A. Isoplastic sarcoma 1. Fibrosarcoma (spindle cell and round cell sarcoma) 2. JVIyosarcoma (leiomyosarcoma, smooth muscle and rhabdomyosarcoma, striated muscle) 3. Lymphosarcoma (reticulum cell sarcoma) B. Metaplastic sarcoma C. Anaplastic and pluriplastic sarcoma 1. Angiosarcoma 2. Myxosarcoma 3. Choxondrosarcoma
2 Melicow,M.M., Pelton, T. H. and Fish, G. W.: Sarcoma of the prostate; review of the literature; table of classification; report of four cases. J. Urol., 49: 675-707, 1943. 3 Costa, A.: Sul sarcoma della prostato. Arch. Italian Chir., 21: 417-435, 1928. 4 Prince, C. L. and Vest, S. A.: Leiomyosarcoma of the prostate; report of a case and critical review. J. Urol., 46: 1129-1143, 1941.
Accepted for publication June 26, 1962. Read at the annual meeting, New York Section, American Urological Association, Inc., New York, N. Y., April 25, 1962. 1 Longley, J.: Sarcoma of the prostate and bladder. J. Urol., 73: 417-423, 1955. 78
SARCOMA OF PROSTATE
before the age of 50. In our current series, the ages represented are 24, 48, 56 and 68, respectively. At the Mayo Clinic, between 1940 and 1950, there were 5 sarcomas reported out of a total of 1500 cases of malignant prostatic disease, an incidence rate of 0.3 per cent. At the Squier Urological Clinic, between 1928 and 1942, the incidence of sarcoma was 1 : 1000. At the Veterans Administration Hospital, Bronx, N. Y., between January 1952 and December 1961, there were 4 sarcomas reported out of a total of 734 cases of malignant prostatic disease, an incidence rate of 0.5 per cent. This higher incidence rate may be attributed to the younger age group treated at this institution. Horstmann 5 in 1952 found one round cell sarcoma and 225 prostatic carcinomas in 31,863 autopsies. DIAGNOSIS
In the adult, the presenting symptoms of sarcoma of the prostate are those indicative of urinary tract obstruction. The pediatric patient will usually present in urinary retention, while the adult's initial complaint is usually dysuria. There is sometimes anterior extension of the tumor so as to present an anterior abdominal wall mass. With perineal extension the tumor may simulate a perineal abscess. Hematuria usually results when the bladder becomes infiltrated. On rectal examination prostatic carcinomas are fixed, rigid, hard and nodular. Upon passing the cystoscope carcinomas give the impression of rigidity. Prostatic sarcomas, however, are smooth, tense, and symmetrical, or soft and balloon-like in consistency, not unlike an abscess. It is frequently impossible to distinguish sarcomas from benign adenomas on rectal examination. The bone metastases of carcinoma are osteoblastic, while those of sarcoma are osteoclastic. 6 A study by Smith and Torgenson7 revealed that 40 per cent of the cases presented had distant metastases; 33½ per cent to bone (ribs and 5 Danckers, U. F., Philipsborn, H.F., Baylor, J.E. and Burkhead, H. C.: Sarcomas of the prostate, periprostatic tissue and bladder neck in children; report of a new case. Amer. J. Roentgenol., 84: 555-561, 1960. 6 Stirling, W. C. and Ash, J.E.: Sarcoma of the prostate. J. Urol., 41: 515-533, 1939. 7 Smith, R. R. and Torgenson, W. R.: Sarcoma of the prostate with a digest of the literature. Surg., Gynec. & Obst., 43: 328, 1926.
79
vertebrae); 33½ per cent to lungs; 23 per cent to the liver; 11.7 per cent to pleura; 20.5 per cent to regional lymph nodes; and 17.6 per cent to the spleen. Benign prostatic hypertrophy, prostatic abscess, carcinoma of the prostate, miillerian duct cyst, prostatic cyst, and retrovesical tumors must all be considered in evaluating a case of prostatic sarcoma. CASE REPORTS
Case 1. H. K., BVAH 232-245, a 48-year-old white man, first noticed the onset of symptoms of bladder neck obstruction in November 1957. The following month, after excessive alcoholic intake, the patient had a bout of acute urinary retention necessitating a suprapubic prostatectomy. February 1958, a transurethral prostatectomy was accomplished for increasing distress due to lower urinary tract obstruction. The microscopic diagnosis of leiomyosarcoma was established at this time. Three months later a suprapubic cystostomy was performed for retention. Subsequently the patient was started on radiotherapy, receiving 5700 r through multiple cross fire portals via the 1 million volt modality. Despite radiotherapy, the rectal mass increased in size. Metastases first became evident in February 1959 with the appearance of a nodular density in the third anterior intercostal space. Four months later the prostatic mass had infiltrated the rectum and both lung fields had multiple nodular densities. By May 1960 the patient had experienced a 30-pound weight loss and a draining perianal fistula was present. An excretory urogram revealed a massive, hazy density throughout the entire pelvis. One month later cutaneous ureterostomies were performed for bilateral ureteral obstruction. By the end of October fecal material drained from the suprapubic and perineal areas and also through a sacral decubitus ulcer. The patient died on November 8, 1960. Postmortem examination revealed leiomyosarcoma of the prostate with metastases to lungs and intestines and locally infiltrating the entire pelvis. Case 2. G. S., BVAH-A2240, a 68-year-old white man, had a 2-week history of hematuria and dysuria. March 1956, a transurethral resection of the bladder neck was performed for urethral obstruction. The microscopic diagnosis was anaplastic carcinoma. Two months later bilateral orchiectomy and prostatic biopsy were
80
JERALD SIEGEL
accomplished. The patient was then started on a regimen of diethylstilbesterol. The pathologic picture following this procedure was interpreted as spindle cell sarcoma. In October the patient complained of dysuria, frequency, urgency and
intermittent gross hematuria. His liver was now palpable 3 cm. beneath the right hypochondrium and his prostate gland was small, hard, nodular and fixed. A biopsy at this time revealed anaplastic leiomyosarcoma of the prostate (fig. 1).
Fm. 1. G. S., A-2240, leiomyosarcoma of prostate. A, X147. B, X462
81
SARCOMA OF PROSTATE
January 1957 he received 4000 r through anterior and posterior portals. He did well for the next 2 years on constant catheter drainage. He was re-admitted to the hospital in April 1959 with hematuria. Examination revealed 4 plus prostatic enlargement with extension, fixation, and loss of outline on the left side. A suprapubic cystostomy was accomplished but the patient's course was rapidly downhill and he died September 23, 1959 of uremia. Postmortem examination revealed leiomyosarcoma of the prostate without metastases and bilateral pyelonephritis and hydronephrosis. Case 3. P. M., BVAH 157-675, a 24-year-old white man, was admitted with a 7-month history of intermittent hematuria and bright red rectal bleeding. Rectal examination revealed a large, tender, grapefruit-sized mass, a biopsy of which, that included both rectal and bladder tissue, revealed rhabdomyosarcoma of the prostate (fig. 2). Shortly after admission a laparotomy was performed with the intention of radical extirpation of the tumor. However, a fixed, indurated mass on the right lateral wall of the prostate and bladder, incorporating the great vessels and adherent to the pelvic wall, was found. These findings made it impossible to carry out the intended procedure and bilateral cutaneous ureterostomies were performed. Two weeks later the patient was transferred to the neurosurgical service for palliative laminectomy because of intractable tenesmus. The patient died 6 weeks later. Postmortem examination revealed rhabdomyosarcoma of the prostate with extension to the bladder and to the floor of the pelvis and rectum, and metastasizing to the lungs, liver and both adrenal glands. Case 4. B. S., BVAH 197-734, a 58-year-old white man, underwent suprapubic prostatectomy in December of 1954 for signs and symptoms of lower tract obstruction. The microscopic diagnosis was rhabdomyosarcoma of the prostate. He did well until March 1955 when his symptoms reappeared. He subsequently received 7500 r to the prostate and bladder via the 2 million volt modality. At the conclusion of the radiotherapy he was admitted to the Bronx Veterans Administration Hospital with a 2-week history of incontinence and hernaturia. Physical examination revealed an expanding suprapubic mass, 2 by 2 cm. in size. The rectal lumen was 50 per cent occluded by a large mass. Panendoscopy re-
vealed a normal trigone with a large necrotic mass of prostate and bladder projecting into the lumen of the prostatic urethra. In a month the suprapubic mass doubled in size. Death came rapidly, approximately 1 year following the onset of symptoms. Postmortem findings were rhabdomyosarcoma of the prostate with extension to the bladder and underlying skin. TREATMENT
Opinions are varied as to the best modality of therapy for this disease. Many authors have condemned radical surgery for fear it hastens the patient's death. Radiotherapy has not been impressive but has its place as an adjunct to surgery. Smith8 reported a case in which the patient was alive and well for 2 ½ years after total perineal prostatectomy, vesiculectomy and 2100 r radiotherapy. Melicow states that surgery is usually only beneficial for the relief of obstruc-tion. He does admit that definitive surgery seems to be encouraging in cases of leiomyosarcorna. Radiotherapy remains the procedure of choice in the more radiosensitive lymphosarcoma. 2 Graves and Coleman9 treated a case of myxofibrosarcoma of the prostate with suprapubic enucleation. The patient died 6 ½ years later of oat cell carcinoma of the lungs. Postmortem examination revealed prostatic adenoma, but no residual sarcoma. Scardino and Prince 10 discussed two unusually long case survivals. The first patient with leiomyosarcoma of the prostate was reported by Prince and Vest in 1941, and treated by radical perineal prostatectomy. H,· was living and free of metastases 14 years later. The second case was diagnosed spindle cell sarcoma of the prostate and was likewise treated bv radical perineal prostatectomy. This patient ,;as alive and free of disease 4 years following his surgical procedure. Barone and .Joelsonll treated their patient with leiomyosarcoma by suprapubic prostatectomy and he was free of 8 Smith, G. G.: Three unusual cases of prostatic malignancy; adenocarcinoma with mucus cells: malignant lymphoma; leiomyosarcoma. J. Urol 70: 267, 1953. 9 Graves, R. S. and Coleman, JVI_. W.: Sarcoma of the prostate; an unusual survival. J. Urol 72: 731-734, 1954. _ 1o Scardino, P. L. and Prince, C._L.: Prostatic sarcoma· survival followmg radical permeal prostate~tomy. J. Urol., 72: 729-730,_1954 11 Barone, A. M. and Joelson, J. J_: Le10myosarcoma of the prostate; report of a case. J. Urol., 63: 533-538, 1950.
82
JERALD SIEGEL
Frn. 2. P. M., 157-675, rhabdomyosarcoma of prostate. A, Xl50. B, X450 disease for 28 months. Radiotherapy was not used, either pre- or postoperatively. Counseller12 treated his patient with x-ray and radium and reported a 17.5-year survival. He later modified 12 Counseller, V. S. and Bedard, R. E.: Sarcoma of the prostate gland. J. Urol., 43: 836-843, 1940.
his procedure with partial prostatectomy followed by radium and radiotherapy in the immediate postoperative period. In the past, Deans, Stevens, Barringer and Hess all agreed that radiotherapy was superior to surgery. However, a review of the literature indicates that definitive
SARCOMA OF PROSTATE
---
' - - - '' --~-- - -
therapy for sarcoma should be radical surgery. In our series of 4 cases, the longest survival was 3½ years (case 2) G. S,, who was treated by transurethral resection, orchiectomy and 4000 r. Case 1, H. K,, survived 3 years following suprapubic prostatectomy plus 5700 r. Case 3, P. M., survived 9 months without therapy and case 4, B, S., survived a year following suprapubic prostatectomy and 7500 r. There seem8 to be no uniformity of agreement as to the proper therapy required for patients with sarcoma of the prostate. Naturally each case has to be individualized as to histologic type of tumor, its degree of radiosensitivity, and extent of spread. Radiotherapy, in the postoperative period, does and shall continue to play an important role in sarcoma therapy. However, the definitive treatment nmst continue to be radical surgery, It behooves the modern urologist to maintain a high index of suspicion for this disease entity, since long-term survival seems to depend upon early diagnosis and radical removal of sarcomatous tissue, DISCUSSION
The first proven leiomyosarcoma of the prostate was established by Isambert13 in 1853. In the subsequent 109 years only 28 eases of that entity have been added to the literature, In 1960 Fitzpatrick and Stump 14 reviewed 25 cases of 13 Riba, L. W, and Wheelock, JVI. C.: Leiomyosarcoma of the prostate. J, Urol., 63: 162-164, 1950. 14 Fitzpatrick, T. J, and Stump, G.: Leiomyosarcoma of the prostate; case report and review of the literature, J. UroL, 83: 80-83, 1960,
leiomyosarcoma of the prostate and the reader is referred to this article for an excellent review. In 1960 Danchers and associates 5 reported a case of leiomyosarcoma in a 1-year-old white boy who had experienced dysuria and frequency for 2 weeks. Exploration disclosed a soft tumor attached to the base of the bladder. The child died in the early postoperative period, It is evident that in the young the course of the disease may be so rapid that local symptoms other than dysuria or partial obstruction are seldom encountered. SUMMARY
Sarcoma of the prostate is rare, but cannot be considered uncommon, Therapy of this disease is dependent upon the stage encountered when diagnosis is established, If discovered early, radical surgery with postoperative radiation is indicated. Later in the course of the disease, radiotherapy with surgery, utilized for palliating obstructive disease, is the method of choice. At the Bronx Veterans Administration Hospital there were 734 prostatic malignancies encountered within a 10-year period, four of which were myosarcomas. The author is grateful to Dr. .J. K. Lattimer for his instructive comments during the preparation of this paper and to JVIr. David Lubin and Mr, Glenroy Harahan of the Medical Illustration Division of the Bronx Veterans Administration Hospital for the preparation of the photomicrogra phs.