SPINDLE CELL SARCOMA OF PROSTATE REVIEW OF LITERATURE AND REPORT OF CASE
GEORGE
J.
RUKSTINAT
AND
CHARLES G. WELLER'
Sarcoma of the prostate gland is an uncommon urologic condition which resists therapy and usually runs a rapid downhill course. As a general rule, children succumb most rapidly to this affliction, while adults survive longer, albeit often painfully. According to Bettoni, the following relationship exists between the age of the patient and the duration of his illness. There has been AGE OF PATIENT
DURATION OF ILLNESS
----------~·-----years
months
0-l
3 5
10-20 20-40 Over 40
5½ 13
marked confusion at each attempt to tabulate the exact number of cases of sarcoma of the prostate gland. This has been due to several factors: first, confusing clinical histories; second, inadequate pathological studies; and third and worst of all, a tendency to group all suspected malignancies of the prostate gland as carcinomas where no biopsies were performed, because of the greater probability of the tumor being a cancer. The scepticism voiced by Ray represents the current tendency to demand incontrovertible facts and will bear repetition. "Considerable doubt is cast upon the authenticity of at least some of these cases when it is realized that the site of origin of sarcomatous growths in the prostatic region must often be impossible of determination and many may not be sarcomas at all but diffuse carcinomas." Despite the inadequacies encountered, there are helpful compilations of cases available. Until this illness no longer presents the difficulties it offers medical men at present, it is imperative that every case be reported. In this way, an early symptom may be brought to general notice, a diagnostic procedure emphasized, or a therapeutic experiment 1 From the Norman Bridge Pathological Laboratory, Rush Medical College of the University of Chicago, and the Department of Urology of Rush Medical College of the University of Chicago. 911
912
GEORGE
J.
RUKSTINAT AND CHARLES G. WELLER
recorded. Bettoni in 1923 compiled a series of concise observations on 49 patients. These furnished much of the information in the encyclopedic Henke-Lubarsch Handbuch and have been augmented by Culver. The latter catalogued 75 reports in the literature and added one of his own. Kretschmer shortly thereafter reported two. He doubted that all of the cases were being reported. There are interesting observations in most of the reports which have appeared from time to time but their salient features have been summarized in the comprehensive review of Lowsley and Kimball. These authors listed 140 tumors and incorporated several original observations. In the interval to January 1938, we found 19 additional reports plus ours, bringing the present total to 160. It is interesting to note that 83 instances are reported prior to 1919, while 77, approximately 50 per cent, are recorded in the last 20 years. The latter period coincides with the rapid advancement of urology as a surgical specialty. Notwithstanding the increased attention to urologic conditions, less than 4 sarcomas of the prostate gland per year have been reported. Briefly tabulated, the cases appearing since the report of Lowsley and Kimball are as shown in table 1. Symptoms arise clinically only when the prostatic growth, by its size, causes difficulty in passing urine or stool. The obstruction to the passage of urine presents itself in about two-thirds of the patients and is due to several factors acting singly or in combination. Marked distortion of the prostatic part of the urethra occurs when one lobe is the seat of the new growth. At times, it is difficult or impossible to pass a cystoscope, and after a difficult passage the instrument assumes an unusual position. The frequent invasion of the bladder wall by tumor masses obstructs normal circulation. The resulting edematous nodules partially fill the bladder cavity and at times occlude the internal urethral orifice. These masses may give rise to temporary obstruction or incontinence as in the patient of Kimball. The man was incontinent at night, could pass urine only while standing, and had a rectal prolapse from the strain of trying to void. The elements which normally constitute the prostate gland seem too limited to account for all the types of sarcomas encountered. According to Ewing, "Sarcoma rarely occurs in the prostate and many of the reported cases are of uncertain nature." Certainly, a review of a subject in which only 160 reports are made, would give that impression, when one recalls the 10 or more types of sarcomas that have been listed as
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SPINDLE CELL SARCOMA OF PROSTATE
occurring in the prostate gland. The very complexity of the problem makes it intriguing. The only universally recognized types of prostatic sarcoma are rhabdomyosarcomas. There has been a steady increase in the reports of this type of tumor, with beautiful demonstrations of cells by improved staining techniques. Katzmann established its origin in the roof of the prostatic part of the urethra. Bands and bundles resembling striated TABLE
1.-Cases of sarcoma of prostate reported between 1934 and 1938
AUTHOR
AGE AT ONSET
INTERVAL FROM ONSET OF
TYPE
SYMPTOMS
LOCAL INVASION
METASTASIS
+ +
+ + + + -
TO DEATH ~~-
Mason. ... Cole and Martin . Dial. Gilbert. .... Gilbert.. Ehrhardt. ........
41 yr. 56 yr. 59 yr. 18 yr. 16 yr. 67 yr.
9 mo. 11 mo. 13 mo. 3 mo. 1½ mo. 3 mo.
Bolton .. Townsend. .. . . .......... Foucar. Jelm .. Reagan. . . . . . . . . . . Rosenberg. ... ..... Ray. . . . . . . . . . . . . . . . ' . . . Tennenbaum. . . . . . . . . . . Kimball. ....... ..... Lahayville and Diss. Trabucco and Sandro. Smith, Louis D. . . . ....
25 yr. 27 yr. 26 yr. 45 yr. 6mo. 46 yr. 8 mo . 18 yr. 31 mo. 36 yr. 27 yr. 44 yr.
41 da. 10 mo. 44 da. 18 mo. 4mo. 7½ mo. 1 mo. 9 mo . 10 wk.* 3 mo. 7 mo. 9 mo.
6 da. 2 yr.
8 da. 2½ mo.
Wachs .......... .... Rukstinat and Weller ..
Lymphocytoma Lymphosarcoma Lymphosarcoma Spindle-cell Myxosarcoma Carcinoma simplex and spindle cell sarcoma Fibrosarcoma Fibrosarcoma Rhabdomyosarcoma Myosarcoma Fibrosarcoma Myxosarcoma Spindle cell Spindle cell Spindle cell Rhabdomyosarcoma Small round cell Small round cell; lymphosarcoma Rhabdomyosarcoma Spindle cell
? ? ? ?
?
No postmortem
+ + + + + + + +
-?
+ + +
-
+ + + + + -
+ -
* Alive.
muscle cells occurred in a fi.brillar ground substance and were multinucleated. At times, they were condensed into knobs as are seen commonly in regenerating muscle cells. Katzmann believes the tumor arose at the transition of smooth to striated muscle in the sphincter. Lahayville and Diss are of the opinion that among leiomyosarcomas, mixed cell sarcomas, fi.bromyosarcomas, and giant cell sarcomas are a certain number of degraded rhabdomyosarcomas. These authors admit that dysembryoplasia may account for the origin of some of these tumors.
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GEORGE
J.
RUKSTINAT AND CHARLES G. WELLER
They are, however, careful to point out that there are striated muscle fibers in the prostatic capsule from which such tumors may sprout. True lymphosarcomas of the prostate are still disputed. Lately, some explanations for their occurrence have appeared. Wegelin has a theory of evolution of lymphoid structure which may explain the presence of lymph follicles in the prostate and eventually malignant lymphatic tumors. He maintains that in certain organs such as the skin, thyroid gland, kidney, and prostate gland, lymph follicles, although normally absent, may evolve as a result of inflammation. The follicles may develop into lymphomas and be provided with central arterioles not unlike those in the spleen. Malignant change from any stage of follicle development is possible. In his patient, Wegelin believes degeneration in a prostatic myoma had led to lymphocytic infiltration as an inflammatory response but developed to a stage in which mature lymph follicles comprised the bulk of the tumor. This is a step between true inflammation and sarcoma. Trauma, suspected of a causal association with sarcoma in other organs and tissues, infrequently has been suspected of such a role in the prostate gland. The rapid development of the growth makes its relationship seem only incidental in the two patients who were injured shortly before developing symptoms later proved due to prostatic sarcoma. The first report suggesting such a relationship is that of Schoppler. His patient was a young soldier who fell, struck his perineum on a hard object, and became unconscious. Pain in the bladder region followed. He died in 2 months of a small round cell sarcoma of the prostate gland. The second case presents a series of doubtful features. A man, 64 years old, in trying to climb on a wagon, slipped on the protruding pole and struck his perineum. He received no treatment for the pain in his abdomen which lasted several days. Three months after the accident he experienced difficulty in urinating. He catheterized himself for three months, then noticed bleeding from the urethra. He died 9 months after his injury. Gruber and Maier reported the prostatic growth as a spindle cell sarcoma. It had wide extensions to the bladder and urethra. A rarity among prostatic sarcomas is a combination with carcinoma: the so-called collision tumor. This is an outstanding example of specialization in tumors. The report of Ehrhardt is unique. The patient was 67 years old and died of uremia. His prostate was the size of an apple and had a polyp the size of a hen's egg hanging into the bladder and
SPINDLE CELL SARCOMA OF PROSTATE
915
closing the internal urethral orifice. The polyp was a spindle cell sarcoma in which were nests of a carcinoma simplex. The latter growth occupied the right lateral lobe. The rapid growth of prostatic sarcoma is demonstrated clearly in several patients treated surgically. Ray reported tumor invasions of perineal incisions in 9 days, commenting on their rapid growth. The tumor was of the spindle cell variety and had metastasized to the bladder, perineum, and lungs. That such tumors may exist at birth was demonstrated recently by Wachs. His patient, a baby 6 days old, had never voided urine. As catheterization was impossible, the bladder was punctured suprapubically. At the postmortem examination 2 days later, the tumor in the prostate was 5 cm. high, 4.5 across, and connected with masses which filled the small pelvis. The tumor was composed of embryonic muscle cells which at times had ill-defined cross-striations. Mixed with the muscle cell bundles were polymorphous and polychromatous spindle cells. From these characteristics, a diagnosis was made of congenital spindle cell sarcoma-sarcomatous-rhabdomyoma. Case report. A boy, 2 years old, had difficulty in voiding urine shortly after he had recovered from an upper respiratory tract infection. When attempting to void, he squeezed his penis and strained until he dribbled a little urine and his bowels moved. Alternate acid and alkaline therapy resulted in a little amelioration of symptoms which returned within 2 weeks. Two months after the onset of symptoms, rectal examination by Dr. R. K. Collins disclosed a firm smooth mass originating in the prostatic region and extending to the left of the midline to one inch below the umbilicus. A cystogram revealed a large filling defect in the base of the bladder involving its lower onethird. There were only a few pus and blood cells in the urine and albumen was recorded three plus. A biopsy of the bladder mass was diagnosed as myxosarcoma. The child rapidly became worse, developed uremia, and died 2½ months after the onset of symptoms. At necropsy, the urinary bladder extended 9 cm. above the symphysis pubis and connected with the umbilicus by an infected urachal cyst 3 cm. long and 1 cm. in diameter. The cyst was separated from the bladder only by bladder mucosa. The bladder was 12 cm. in diameter and the distal half of its back wall for 8 cm. square was covered with glistening yellow-white gelatinous masses up to 4 cm. in diameter (fig. 1). The largest mass was on a pedicle and was sufficiently close to the urethra to form a ball valve. When these nodules were cut across, they quickly lost about one-third of their bulk by extrusion of edema fluid. The prostate gland was slightly less edematous, pale yellow, and 4.2 cm. across. The ureters were twice the normal size as
916
GEORGE J. RUKSTINAT AND CHARLES G. WELLER
were the kidneys. Both were involved in a typical advanced ascending inflammation. Metastases were absent from all the organs of the trunk. Histologically, the prostate gland was remarkably altered. Its tubules, encountered at wide intervals, were surrounded by sparse infiltrations of lymphocytes which occurred in concentric rings in edematous tumor tissue (fig. 2). At least two-thirds of the interstitial tissue was edematous and con-
FIG. 1. Gross specimen illustrating urachal cyst, tumor of prostate with extension to bladder, and large kidneys resulting from ascending urinary infection. A pedunculated mass in bladder acted as a ball valve and prevented passage of urine.
tained only occasional polymorphonuclear leukocytes. The tumor cells were typically spindle-shaped, grew in brushlike masses, and penetrated muscle and connective tissue in broad bands. In the bladder, the epithelium covering the tumor masses was intact and lay on an edematous connective tissue layer which separated it from the invading tumor (fig. 3). In the crevices between several tumor masses was a fibrino-purulent exudate. This was encountered in the kidneys and ureters and also in the urachal cyst.
SPINDLE CELL SARCOMA OF PROSTATE
917
FIG. 2. Showing intact prostatic tubules about which there are clusters of spindle cells. A mass of similar cells is in lower right portion of picture, while intervening zone is edematous. FrG. 3. Papillary mass in bladder showing intact epithelium and heavily sarcomatous invasion of submucosa.
918
GEORGE
J.
RUKSTINAT AND CHARLES G. WELLER SUMMARY
A case of spindle cell sarcoma of the prostate gland is reported. The typically rapid clinical course and pathologic features of the growth are emphasized. A review of the recent literature is included. Attention is directed to the common syndromes produced by prostatic sarcomas.
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