PRIMARY CARCINOMA OF THE URETER 1 HERi\!IAN L. KRETSCHMER Chicago, Illinois
Of the malignant tumors occurring in the ureter, carcinomata are more common than sarcomata. Carcinoma occurring in the ureter may be primary or secondary. Secondary carcinoma may originate in the kidney pelvis by direct extension or the ureter may be involved by a carcinoma of the bladder. The ureter may also be involved secondary to a carcinoma of the uterus or the ovary. Aschner, in a recent article, was able to collect 47 cases of primary tumors of the ureter, including both benign and malignant cases. It would thus appear that primary epithelial tumors in the ureter are rare. This paper will be limited to a consideration of primary carcinoma of the ureter. From the available literature I have been able to collect 34 cases to which I wish to add a case under recent observation, thus bringing the total number of cases of primary carcinoma of the ureter available for discussion up to 35 cases. The case under recent observation is the following. G. M., aged seventy-four. Referred by Dr. Charles Collester. Previous illnesses. Twenty years ago patient was operated upon for carcinoma of the lip. Complete cure. Present complaint. Patient was well until five weeks before he came under observation when he began to pass dark red urine. Since the onset of the trouble the urine was never entirely free of blood. The amount of the bleeding varied so that at times the urine was either very dark red or light red. Blood and urine were well mixed: occasionally clots were passed. N octuria has been present for several years. During the last two months he has lost about 10 pounds in weight. Examination. General physical examination was negative. Scar from the previous operation for carcinoma of the lip was normal. No 1 Read l\lay 17, 1923 at Cleveland, Ohio, before the American Association of Genito-Urinary Surgeons at the thirty-fifth annual meeting.
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local recurrence. Kidneys, ureters and bladder negative. Rectal examination showed a slight enlargement of both lateral lobes of the prostate . .Cystoscopic examination. A definite trabeculation of the bladder was seen and at the vesical neck a large median bar was found. The ureteral orifices were normal. No signs of tumor in the bladder or in or around the ureteral orifices. Ureters were catheterized without difficulty or obstruction. Pyelogram. On the right side the catheter took a most unusual course, i.e., upward and outward in an S-shaped curve, the tip overlying the midpart of the crest of the ilium. At the level of the second lumbar a dense rounded shadow was seen, the size of a half dollar. There was marked lipping of the lumbar spine and thickening of the sacro-iliac joint. No stone shadows were seen. Cell count and cultures were as follows : LEUCOCYTES PER CUB IC MILLIMETER
Bladder .............. .. .. ... . .. . . . . . .. . . Right kidney . .. ... ........... . .. ..... . . . Left kidney . . ... . . . .. .. ...... . . . ...... . .
310
190 50
CULTU R ES
Sterile Sterile Sterile
TUBERCLE
BACILLI
Negative Negative Negative
Operation, S eptember 3, 1922. The patient left the city and returned to his home where he was operated upon by Dr. Phemister, to whom I am indebted for his kindness in turning over the specimen to me for study. The usual oblique lumbar incision was made over the right side and a lumbar nephroureterectomy was performed. The patient made an uneventful recovery. SEX
According to the cases reported the incidence of sex seemed to play little, if any, role. Males were more frequently affected than females, but the difference was so slight as to be negligible. The number of males, including the case here reported, amount to 19; females, 16. AGE
Here, as in other forms of malignant disease of the urinary tract, it may be stated that this occurred with greater frequency in advanced years, although there were exceptions to this statement.
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PRIMARY CARCINOMA OF URETER
The exceptions were the cases of Albarran, thirty-six years: Finsterer, thirty-five years: Zironi, thirty-six years; Aschner, thirtyeight years. There were 2 patients, aged eighty: 1 reported by Richter, the other by Toupet and Guebiat. Curiously enough both these patients were females. The following table shows the incidence of age. number of cases
30 to 39 40 to 49 50 to 59 60 to 69 70 to 79 80 to 89 Age not
years. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . years. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . years. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . years. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . years ....................................................... years. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . stated ......................................................
4 7 9 9 2 2 2
PRESENCE OF CALCULI
Although it has repeatedly been stated that the presence of stone may be an etiological factor in causing carcinoma, and in order to support this statement attention has been called to the frequency with which stones and carcinoma of the gall bladder are found at the same time, it would seem that in the urinary tract, stones and carcinoma do not occur simultaneously as often as in the biliary tract. Evidently stone is of no moment as an etiological factor in causing carcinoma of the ureter since only in 5 cases was it present. Its occurrence in the ureter has been mentioned by Davy, Metcalf and Safford, Paschkis and Zironi. In Judd and Struthers' case, the patient stated that he passed a stone. In the St. Thomas Hospital case, stone was present in the opposite kidney, and in Aschner 's case it was found in the kidney on the corresponding side. PATHOLOGY
From a review of these 35 cases it appears that the papillary form of carcinoma occurs most frequently in the ureter, since 18 of the 35 cases were of this type. Next in point of frequency were the medullary carcinomata, 5 cases, and the squamous-celled carcinomata, 5 cases. In 1 case the tumor was described simply as an epithelioma. No further Type of tumor.
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histological description was noted. Epithelial carcinoma was the term used to describe 2 cases. In 2 cases the tumor was described as transitional-celled carcinoma. In 1 case the term carcinoma solidum simplex was used and in another case, simply carcmoma. Since squamous cells are not normally present in the renal pelvis, the presence of squamous-celled carcinoma brings up for discussion the interesting question of metaplasia, the occurrence of which has been previously discussed. 2 number of case
Papillary carcinoma. . .... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 Squamous-celled carcinoma... .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Medullary carcinoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Epithelial carcinoma. ... . .. . . . . . . . . ... . . . . . . . . ...... ... .. ..... . . . ..
2
Transitional-celled carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Epithelioma........ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Carcinoma solidum simplex. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Carcinoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
2 1 1 1
One of the most constant concomitant pathological findings is hydronephrosis. This is but a natural development as it is the direct result of the tumor producing a stricture or obstruction within the ureter, with a resulting hydronephrosis above the obstruction. Hydronephrosis has been recorded as present in 26 of the 35 cases. In 9 cases neither the presence nor absence of hydronephrosis was noted. Tt is possible that the real incidence of hydronephrosis is larger than appears from a review of the literature since some of the authors may have neglected to report its presence. In the case reported in this paper a small hydronephrosis was found above the tumor. This was demonstrated by the pyelogram before operation. Metastases
It would appear that metastases occur in these cases as frequently as in any other form of carcinoma. In 3 cases bone metastases were stated to be present: lumbar vertebra (Adler), 2 H. L. Kretschmer, Surg. Gynecol. and Obstst., Oct ober, 1920 ; Archives of Surg., September, 1922, v.
PRIMARY CARCINOMA OF URETER
ilium (Hektoen), spine (Schmitt). has been reported:
The following distribution
Liver and lymph glands .......................................... Davy Right lung and bladder ....................................... Gerstein Liver, lungs and lymph glands ................................. Rundle Liver, left kidney and spine ................................... Schmitt Liver, lung and lymph glands (2). . . . . . . . ..... Voelcker and Vorpahl Right kidney .................................................... Israel Nerves ........................................................... Kidd
It is interesting to note that in 5 of the 8 cases there ·were metastases in the liver, which appears to be more frequently the seat of metastases than any other organ. Location in the ureter A study of these 35 cases shows that carcinoma occurs more frequently in the lower portion of the ureter since in 19 cases it was found at varying parts in the lower third. In 2 cases it was stated that the tumor was situated in the n1iddle of the ureter and in the remaining 8 cases the upper ureter was invol-rnd. The entire ureter, or practically the entire ureter, was involved in 6 cases. SYMPTOMS
Hematuria heads the list of symptoms because it is the most constant symptom, having been present in 24 of the 35 cases. In some of the remaining cases no mention is niade of bloody urine. This probably does not represent accurately the exact status regarding the occurrence of hemturia, in view of the fact that some of the cases, being autopsy cases, received only brief mention. In several cases definite statements ,vere niade that blood in the urine was absent (Adler, Von Capellen, Hektoen, Toupet and Gueniot, Wising and Blix. Pain was a most important and constant symptom, being present in 26 of the 35 cases. There was nothing characteristic about this pain from: which a diagnosis of carcinoma of the ureter could be made or surmised, nor was it always referred to the same place. In 21 cases pain was the first symptom noted, and was most frequently · referred to the back on the corresponding side on which the tumor was found. The most frequent terms used by the authors in
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describing the location of the pain were, lumbar region, region of the kidney, in the back and in the abdomen. In 5 cases the pain was referred to the hip (Hektoen, Gerstein, Spiess, St. Thomas Hospital and Kidd). In some of the cases the pain, no doubt, was due to the hydronephrosis. DIAGNOSIS
The diagnosis from the history and physical examination is almost impossible, especially when the tumor is small, but when it reaches a large size so that it can be felt by abdominal palpation, the diagnosis can be made or surmised. In women it is possible to palpate the tumor through the vagina and in men carcinoma of the lower ureter may be felt through the rectum . . In order to make an accurate diagnosis, it will be necessary to resort to the use of special diagnostic aids, such as cystoscopy, ureteral catheterization and pyelography. In cases in which the tumor protrudes from the ureteral orifice, the diagnosis can be made by the cystoscope or a diagnosis may be ventured when there is profuse hemorrhage from the ureter associated with obstruction, as demonstrated with the ureteral catheter, assuming, of course, that stone and stricture have been excluded. But we must not forget that both stone and carcinoma may -0ccur at the same time. Persistent bleeding from the ureter after nephrectomy is highly suggestive of ureteral neoplasm. In a recent letter from Dr. Collester he informed me that the patient, whose case is here reported, has again passed blood in the urine. This probably means that the patient has recurrence of tumor formation in the stump of the ureter. In view of the fact that hydronephrosis is so frequently found, pyelograms may give some additional information. The present literature contains nothing in the way of pyelograph1c data since most of the cases were published before pyelograms became part of our routine examination. A pre-operative diagnosis of carcinoma or tumor of the ureter was made by Albarran, Chevasse and Mook, Gerstein, Judd and Struthers, Rathbun, Richter and Zironi. The following is a list of some of the pre-operative diagnoses made: sarcoma of
PRIMARY CARCINOMA OF URETER
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the ilium (Butler); pyonephrosis (Von Capellan); bladder papilloma (Finisterer); ureter calculus (Metcalf and Safford); carcinoma of kidney (Rundle): rheumatism and dementia praecox (Spiess); papilloma of the kidney with secondary involvement of ureter (Kidd). A diagnosis of tumor of the kidney with hydronephrosis was made in the case reported here. This was based upon the age of the patient, slight loss of weight and a persistent painless unilateral renal hematuria, and the pyelogram. REFERENCES ADLER, L.: Monatsber. f. ur.ol., 1905, x, 129. ALBARRAN, J.: Ann. d. med. gen. urin., 1900, xviii, 918. AscHNER, P. W.: Surg., Gyn., and Obst., December, 1922, 749. BUTLER, F. A.: Clifton Med. Bul., 1914, ii, 48. CREVASSE, M., AND MocK, J.: Bul. et. mem. Soc., Chir. Par., 1912, xxxviii, 522. CHIARI, 0. M.: Ztschr. f. urol., 1914, viii, 672. DAVY, R.: Brit. Med. Jour., 1S84, ii, 757. FINISTERER, H.: Wien. Klin. Wchschr., 1915, xxvii, 718. GERSTEIN, K.: Inaug. Dissert., Kiel, 1902. HEKTOEN, L.: Trans. Chicago Path. Soc., 1895, 97, ii, 132. ISRAEL, J .. : Berl. klin. Woch., 1910, xlvii, 2381. JONA, G.: Centrlbl. f. allg. Path. u. path. Anat., 1894, v, 659. JUDD, E. S., AND STRUTHERS, J. E.: Jour. Ural., 1921, vi, 115. KrnD, FRANK: Personal communication. KNACK,---: Deutsch. med. Vi'chschr., 1918, xliv, 982. KRETSCHMER, H. L.: Surg., Gyn. and Obst., October, 1920. Arch. Surg., September, 1922, v. LOWENSTEIN, E.: Inaug. Dissert., Freiberg, 1911. METCALF, W. F., AND SAFFORD, H. E.: Amer. Jour. Med. Sci., 1905, cxxix, 50. MINICK, C. K.: Pest., ed. chir., Pressc., 1902, xxxviii, 941. PASCHKIS, R.: Wien. klin. Wchschr., 1910, xxiii, 361. PASCHKIS, R., AND PLESCHNER, H. G.: Med. Klinik., 1920, xvi, 2, 1254. RATHBUN, N. P.: Internat. Jour. Surg. RICHTER, J.: Ztschr. f. urol., 1909, iii, 416. RUNDLE, H.: Trans. Path., Soc., Lond., 1896, xlvii, 128. SCHMITT, E. E.: Trans. Chicago Path. Soc., 1915, 18, x, 127. Srrns, P.: Centrlbl. f. allg. path. Anat., 1915, xxvi, 561. SUTER, F.: Zeitschrift f. urol. Chir., x, 522. St. Thomas Hospital: St. Thomas Hospital Reports, London, 1904, xxxii, 96. TouPET AND GuENIOT: Bul. Soc. Anat. Par., 1898, lxxiii, 677. VoRPAHL, K.: Inaug. Dissert., Greifwald, 1905. VoELCKER, A. F.: Tr. Path. Soc., Lond., 1895, xlvi, 133. VoN CAPELLEN: Beitr. z. klin. Chir., 1916, xcix, 138. WISHING, P. J., AND Bux, C.: Rev. d. sc. med., 1878, xviii, 457. ZrnoNI: Amer. d. mal. org. genito-urin., 1909, i, 81.