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Two Rare Cases of Bladder Tumor (Urachus Tumor)
TWO RARE CASES OF BLADDER TUMOR (URACHUS TUMOR) T . DE WAARD
A 54-year-old woman for many years had been harassed by constantly recurring bladder trouble. Then she complained of frequent micturition accompanied by pains, and of pains in the lower part of the abdomen. The urine was turbid, containing a large amount of fibers, but emitted no bad smell. Under a simple form of treatment, comprising the use of medicaments and the administration of medicaments into the bladder, rest in bed, etc., the complaints lessened, but at irregular periods they were apt to reappear. Besides the complaints stated, she continually complained of pains in the upper right part of the abdomen. The patient had repeatedly been examined, and the diagnosis was, after cystoscopic examination, determined to be right pyelitis, and, after a gall-bladder examination, a cholecystitis calculosa. Surgical treatment for the latter was rejected by the patient. After that, she applied to me for treatment. Examination of the abdomen showed the region of the gall-bladder and urinary bladder to be very sensitive. There was no muscular tension of any importance. Abnormal resistances could not be felt. The right kidney was a little sensitive when percussing it, the left kidney was not. Nothing abnormal was revealed by the vaginal examination nor the rectal examination. The urine was slightly cloudy, containing many fibers, some 1 cm. in length, and showed an acid reaction. The sediment consisted nearly exclusively of leucocytes and epithelial cells. A trace of albumin was present. Cystoscopy revealed the existence of a diffuse cystitis without particular characteristics. In the median line, in the upper part of the bladder, a slight, clearly outlined swelling could be noticed, in the center of which a tuft of fibers, such as were floating in the urine, could be made out, protruding into the cavity of the bladder. A ureteral sound could be introduced about 2 cm. through these fibers into an apparently existing duct or cavity. The urine from both kidneys was completely normal. It was clear that the source of these fibers in the urine had been discovered. I thought that I had to deal with an unclosed remainder of the urachus in which an inflammation had developed which by its occasional secretions, gave rise to an acute or subacute cystitis. Three treatments of this "duct" with a solution of 2 per cent nitras argenti, introduced through a ureteral sound, had no success at all, but the treatment was very painful. It was then decided to remove the remainder of the urachus by cutting it out. The bladder was filled and the peritoneum laid free by an incision from 554
TWO RARE CASES OF BLADDER TUMOR
555
symphysis to the navel. The peritoneum was then lifted upward as far as possible, and when the bladder could be felt, the hand touched upon a tumor in the bladder wall, of the size of a good-sized mandarin, to which the peritoneum had grown firmly, just in the region where in a normal bladder the peritoneum is also firmly attached to the bladder and has, in a normal state, in many cases to be detached with the aid of the knife. The peritoneal covering of the tumor could not be removed, so an incision was made around it and the abdominal cavity opened. In the abdominal cavity it could be seen that to the tumor was attached a thick string of omentum stretching tightly in the direction of the gall-bladder. The gall-bladder itself appeared quite normal on examination by hand. The string mentioned was cut through, the peritoneum at the rear of the bladder further laid bare, so that the opening in the peritoneum could be closed and the further operation carried out in the retroperitoneal space.
At a distance of about 3 cm. from the exterior edge of the tumor which was sharply outlined in the bladder wall (fig. 1), the whole tumor was removed and the opening in the bladder sutured in two layers. Further treatment was started with an indwelling catheter. At the end of 3 weeks, the opening was closed; at the end of 2 months, the urine was normal and the patient free from her abdominal and bladder troubles. At this moment, about a year after the operation, the patient enjoys perfect health. Four months later, a 37-year-old man was sent to me as suffering from hematuria. Never before had there been any complaints, except for the casual appearance of some pain in the lower region of the abdomen. Somatic examination of the patient revealed no signs of any deviation from the normal state. The urine showed, on microscopic examination, traces of red blood cells, but no albumen. Cystoscopically, there appeared, in the highest part of the bladder, just in the median line, a growth resembling a papilloma which I considered to be the source of the bleeding. After coagulation, the bleeding ceased for a time. After 2 months, however, there occurred profuse bleeding again. Cystoscopic
556
T. DEWAARD
examination, performed during the bleeding, revealed a lozenge-shaped granulating defect in the place mentioned above, measuring about 1 by 1 cm., which bled profusely over its whole surface. There appeared something like a canal of about 1 cm. depth to be present there. After coagulation of this defect in the mucosa, the bleeding ceased, but invariably returned after 10 days. I could not arrive at any other diagnosis except simple "bleeding ulcer," and owing to the persistency of the bleeding I resolved to cut out also this defect. The operation was carried out in the same way as in the case of the former patient. Also here, a tumor of the size of a mandarin could be seen and felt which, again, could not be separated from the peritoneum. The further treatment with an indwelling catheter did not proceed so smoothly. Some time after the patient had been going about again with his wound closed, his temperature rose high owing to the infiltration of urine into the wound, followed by a bladder fistula. After 2 months, however, the patient was able to leave without complaints, the urine having become clear after 5 months.
There is a great difference between the complaints of the 2 patients, although during the operation there was next to no difference at all between the 2 cases. There was much analogy in the outward appearance of the 2 growths. They are topographically situated in the bladder in the same place, i.e. just in the median line of the bladder, at the highest point of it. Both are the approximate size of a mandarin and clearly outlined. They did not protrude into the bladder, because the larger part was outside the bladder (fig. 1). On the outside both were covered with peritoneum which could not be removed. Both were, within, nearly covered with mucosa which cystoscopically looked altogether normal except for a small, lozenge-shaped, granulated defect in the second case. In the first case there was a duct of about 2 cm. depth; in the second there was a small suggestion of a similar duct. A necrotic surface was absent in either case. In both cases the median umbilical ligament disappeared in the peritoneum of the tumor. In neither case did the cystoscopic picture suggest a tumor. After dissection, a difference becomes at once visible. The first tumor is full of colloid nodules which did not exist in the second case. Besides, in one tumor there are in many places calcareous accumulations. Necrotic spots were not noticeable. As far as microscopic diagnosis is concerned, there was, at the beginning, no agreement regarding the first tumor. In the end, however, the diagnosis was colloid carcinoma (adenocarcinoma gelatinosum) (:fig. 2). The second tumor was an unmistakable adenocarcinoma with a
TWO RARE CASES OF BLADDER TUMOR
557
microscopic trace of mucus secretion. Adenocarcinoma in the bladder is the rarest sort of tumor (2 per cent of all bladder tumors). According to Buckel, there are 3 sources of origin, viz.: (1) the para-urethral glands at the neck of the bladder; (2) the remnants of the urachus; (3) glandular formations after cystitis glandularis. It is clear that in 1 and 2 the carcinoma must occupy a typical position. By reason of the typical position of the tumor in both cases, i.e. just in the median line of the bladder dome; the existence of more or less distinctly noticeable ducts opening into the bladder in the place where remainders of the urachus must be looked for; the mucosa, which was
FIG. 2
FIG. 3
found to be quite intact in one case and nearly intact in the other, without any suggestion of the presence of a tumor; the non-projection of both tumors into the inner side, but the projection towards the outward side of the bladder; the close covering of both tumors with the peritoneum, just like the bladder, in the place where the urachus commonly opens into the bladder (the median umbilical ligament disappeared here into the peritoneum)-is always connected with the peritoneum which cannot practically be separated from it; and also by reason of the rare diagnosis of "adenocarcinoma"-we are surely confronted here with tumors whose source is to be sought in the remainders of the urachus and, therefore, in these cases, we are here dealing with urachus carcinoma. 1 1 It must be mentioned that the pathologist could not find any rests of the urachus epithelium in the tumor so a direct proof of the origin of the tumors is absent. In cases such as these, as the pathologist assured me, the proof must come from the surgeon.
558
T. DEWAARD
In the literature, these tumors are stated to be extremely rare. Beer says: "Unusual glandular (colloid) tumors may rarely develop near the urachus, perhaps due to embryonic misplaced tissue of the omphalomesenteric duct." Very likely Beer, either consciously or unconsciously, referred to tumors of the urachus. Begg collected, in 1931, 44 cases of tumors originating in the remains of the urachus. Pathological examination showed the distribution into: 3 fibro-adenomata, 4 adenomata, 19 colloid carcinomata, 8 mixed tumors, 4 myxomata and fibromata, and 6 sarcomata. The carcinoma gelatinosum described above apparently occurs most frequently of all. The simple adenocarcinoma is the rarest of all, only three of them being known in the literature (Begg). The case here described may thus be added as number four. Clinically, these carcinomata have quite a different appearance from carcinoma of the bladder. The true carcinoma of the bladder is characterized by the appearance of bladder complaints at· an early stagecomplaints that may be variable, but never disappear totally, as in the first, or are never totally absent, as in the second instance. From a therapeutic point of view, they also occupy a separate place; they give the impression of being easily operable. Therefore, only one treatment should be applied, i.e.-without exception-surgical treatment. At this moment both patients are altogether free from complaints and their urine is normal. M athenesserlaan 294, Rotterdam, Holland.
A 54-year-old woman for many years had been harassed by constantly recurring bladder trouble. Then she complained of frequent micturition accompanied by pains, and of pains in the lower part of the abdomen. The urine was turbid, containing a large amount of fibers, but emitted no bad smell. Under a simple form of treatment, comprising the use of medicaments and the administration of medicaments into the bladder, rest in bed, etc., the complaints lessened, but at irregular periods they were apt to reappear. Besides the complaints stated, she continually complained of pains in the upper right part of the abdomen. The patient had repeatedly been examined, and the diagnosis was, after cystoscopic examination, determined to be right pyelitis, and, after a gall-bladder examination, a cholecystitis calculosa. Surgical treatment for the latter was rejected by the patient. After that, she applied to me for treatment. Examination of the abdomen showed the region of the gall-bladder and urinary bladder to be very sensitive. There was no muscular tension of any importance. Abnormal resistances could not be felt. The right kidney was a little sensitive when percussing it, the left kidney was not. Nothing abnormal was revealed by the vaginal examination nor the rectal examination. The urine was slightly cloudy, containing many fibers, some 1 cm. in length, and showed an acid reaction. The sediment consisted nearly exclusively of leucocytes and epithelial cells. A trace of albumin was present. Cystoscopy revealed the existence of a diffuse cystitis without particular characteristics. In the median line, in the upper part of the bladder, a slight, clearly outlined swelling could be noticed, in the center of which a tuft of fibers, such as were floating in the urine, could be made out, protruding into the cavity of the bladder. A ureteral sound could be introduced about 2 cm. through these fibers into an apparently existing duct or cavity. The urine from both kidneys was completely normal. It was clear that the source of these fibers in the urine had been discovered. I thought that I had to deal with an unclosed remainder of the urachus in which an inflammation had developed which by its occasional secretions, gave rise to an acute or subacute cystitis. Three treatments of this "duct" with a solution of 2 per cent nitras argenti, introduced through a ureteral sound, had no success at all, but the treatment was very painful. It was then decided to remove the remainder of the urachus by cutting it out. The bladder was filled and the peritoneum laid free by an incision from 554
TWO RARE CASES OF BLADDER TUMOR
555
symphysis to the navel. The peritoneum was then lifted upward as far as possible, and when the bladder could be felt, the hand touched upon a tumor in the bladder wall, of the size of a good-sized mandarin, to which the peritoneum had grown firmly, just in the region where in a normal bladder the peritoneum is also firmly attached to the bladder and has, in a normal state, in many cases to be detached with the aid of the knife. The peritoneal covering of the tumor could not be removed, so an incision was made around it and the abdominal cavity opened. In the abdominal cavity it could be seen that to the tumor was attached a thick string of omentum stretching tightly in the direction of the gall-bladder. The gall-bladder itself appeared quite normal on examination by hand. The string mentioned was cut through, the peritoneum at the rear of the bladder further laid bare, so that the opening in the peritoneum could be closed and the further operation carried out in the retroperitoneal space.
At a distance of about 3 cm. from the exterior edge of the tumor which was sharply outlined in the bladder wall (fig. 1), the whole tumor was removed and the opening in the bladder sutured in two layers. Further treatment was started with an indwelling catheter. At the end of 3 weeks, the opening was closed; at the end of 2 months, the urine was normal and the patient free from her abdominal and bladder troubles. At this moment, about a year after the operation, the patient enjoys perfect health. Four months later, a 37-year-old man was sent to me as suffering from hematuria. Never before had there been any complaints, except for the casual appearance of some pain in the lower region of the abdomen. Somatic examination of the patient revealed no signs of any deviation from the normal state. The urine showed, on microscopic examination, traces of red blood cells, but no albumen. Cystoscopically, there appeared, in the highest part of the bladder, just in the median line, a growth resembling a papilloma which I considered to be the source of the bleeding. After coagulation, the bleeding ceased for a time. After 2 months, however, there occurred profuse bleeding again. Cystoscopic
556
T. DEWAARD
examination, performed during the bleeding, revealed a lozenge-shaped granulating defect in the place mentioned above, measuring about 1 by 1 cm., which bled profusely over its whole surface. There appeared something like a canal of about 1 cm. depth to be present there. After coagulation of this defect in the mucosa, the bleeding ceased, but invariably returned after 10 days. I could not arrive at any other diagnosis except simple "bleeding ulcer," and owing to the persistency of the bleeding I resolved to cut out also this defect. The operation was carried out in the same way as in the case of the former patient. Also here, a tumor of the size of a mandarin could be seen and felt which, again, could not be separated from the peritoneum. The further treatment with an indwelling catheter did not proceed so smoothly. Some time after the patient had been going about again with his wound closed, his temperature rose high owing to the infiltration of urine into the wound, followed by a bladder fistula. After 2 months, however, the patient was able to leave without complaints, the urine having become clear after 5 months.
There is a great difference between the complaints of the 2 patients, although during the operation there was next to no difference at all between the 2 cases. There was much analogy in the outward appearance of the 2 growths. They are topographically situated in the bladder in the same place, i.e. just in the median line of the bladder, at the highest point of it. Both are the approximate size of a mandarin and clearly outlined. They did not protrude into the bladder, because the larger part was outside the bladder (fig. 1). On the outside both were covered with peritoneum which could not be removed. Both were, within, nearly covered with mucosa which cystoscopically looked altogether normal except for a small, lozenge-shaped, granulated defect in the second case. In the first case there was a duct of about 2 cm. depth; in the second there was a small suggestion of a similar duct. A necrotic surface was absent in either case. In both cases the median umbilical ligament disappeared in the peritoneum of the tumor. In neither case did the cystoscopic picture suggest a tumor. After dissection, a difference becomes at once visible. The first tumor is full of colloid nodules which did not exist in the second case. Besides, in one tumor there are in many places calcareous accumulations. Necrotic spots were not noticeable. As far as microscopic diagnosis is concerned, there was, at the beginning, no agreement regarding the first tumor. In the end, however, the diagnosis was colloid carcinoma (adenocarcinoma gelatinosum) (:fig. 2). The second tumor was an unmistakable adenocarcinoma with a
TWO RARE CASES OF BLADDER TUMOR
557
microscopic trace of mucus secretion. Adenocarcinoma in the bladder is the rarest sort of tumor (2 per cent of all bladder tumors). According to Buckel, there are 3 sources of origin, viz.: (1) the para-urethral glands at the neck of the bladder; (2) the remnants of the urachus; (3) glandular formations after cystitis glandularis. It is clear that in 1 and 2 the carcinoma must occupy a typical position. By reason of the typical position of the tumor in both cases, i.e. just in the median line of the bladder dome; the existence of more or less distinctly noticeable ducts opening into the bladder in the place where remainders of the urachus must be looked for; the mucosa, which was
FIG. 2
FIG. 3
found to be quite intact in one case and nearly intact in the other, without any suggestion of the presence of a tumor; the non-projection of both tumors into the inner side, but the projection towards the outward side of the bladder; the close covering of both tumors with the peritoneum, just like the bladder, in the place where the urachus commonly opens into the bladder (the median umbilical ligament disappeared here into the peritoneum)-is always connected with the peritoneum which cannot practically be separated from it; and also by reason of the rare diagnosis of "adenocarcinoma"-we are surely confronted here with tumors whose source is to be sought in the remainders of the urachus and, therefore, in these cases, we are here dealing with urachus carcinoma. 1 1 It must be mentioned that the pathologist could not find any rests of the urachus epithelium in the tumor so a direct proof of the origin of the tumors is absent. In cases such as these, as the pathologist assured me, the proof must come from the surgeon.
558
T. DEWAARD
In the literature, these tumors are stated to be extremely rare. Beer says: "Unusual glandular (colloid) tumors may rarely develop near the urachus, perhaps due to embryonic misplaced tissue of the omphalomesenteric duct." Very likely Beer, either consciously or unconsciously, referred to tumors of the urachus. Begg collected, in 1931, 44 cases of tumors originating in the remains of the urachus. Pathological examination showed the distribution into: 3 fibro-adenomata, 4 adenomata, 19 colloid carcinomata, 8 mixed tumors, 4 myxomata and fibromata, and 6 sarcomata. The carcinoma gelatinosum described above apparently occurs most frequently of all. The simple adenocarcinoma is the rarest of all, only three of them being known in the literature (Begg). The case here described may thus be added as number four. Clinically, these carcinomata have quite a different appearance from carcinoma of the bladder. The true carcinoma of the bladder is characterized by the appearance of bladder complaints at· an early stagecomplaints that may be variable, but never disappear totally, as in the first, or are never totally absent, as in the second instance. From a therapeutic point of view, they also occupy a separate place; they give the impression of being easily operable. Therefore, only one treatment should be applied, i.e.-without exception-surgical treatment. At this moment both patients are altogether free from complaints and their urine is normal. M athenesserlaan 294, Rotterdam, Holland.