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Primary Carcinoma of the Ureter: With Report of a Case
PRIMARY CARCINOMA OF THE URETER WITH REPORT OF A CASE
w.
H. SNYDER, JR.,
AND
B.
s.
WOOD
From the Urological Department, Massachusetts General Hospital, Boston, Massachusetts
Our purpose in this paper is to add a new case of primary carcinoma of the ureter to the literature, to analyze briefly the cases previously reported and to comment on the chief features of the case we present. In view of the excellent recent contributions of Rousselot and Lamon (60), D'Aunoy and Zoeller (13), McCown (40) and Renner (57), we do not believe resummarizing past records would be of value at the present time. CASE REPORT
History. A forty-nine-year-old, white, male, woodworker entered the Emergency Ward of the Massachusetts General Hospital on December 8, 1930, complaining of pain in the right loin of five months duration. Five months previous to admission, while sitting at work, the patient was seized with sharp pain in his right loin radiating to the anterior aspect of the right leg and to the right testicle. Prior to the present illness he was well but had noticed a gradual loss of strength. On the morning of the acute onset he complained of burning on urination and noticed that the urine was reddish brown in color. Daily thereafter he had a dull ache in his right loin with some tenderness in the right costovertebral angle. Several times he had attacks of true renal colic. The most recent one, two weeks before admission, radiated to the right testicle (November, 1930). The acute attacks lasted on the average of about four hours. With the exception of the first attack hematuria, dysuria, urgency and frequency had not been present but the patient had had nocturia during the three weeks before admission. There had been a loss in weight of 12 pounds in the two weeks before admission. He had had no cardio-respiratory or neuromuscular disturbances. Gastrointestinal symptoms were those of marked constipation, definitely 577
578
W. H. SNYDER, JR., AND B. S. WOOD
increased during the three weeks previous to admission. At times 3 tablespoons of magnesium sulphate had no effect. The patient had never noticed tarry, mucoid, bloody or clay-colored stools but had recently had considerable tenesmus. Physical examination. The patient was a rather small, underdeveloped and poorly nourished male, lying in bed complaining of right loin pain. There was internal strabismus of the left eye. The tongue was furred. There were no enlarged glands. The heart was enlarged, rather rapid and with occasional extra-systoles. The lungs were essentially negative. In the abdomen there was an irregular, firm mass in the right lower quadrant about 2 by 3 cm. which was tender on pressure and definitely adherent to the surrounding structures. Rectal examination revealed a slightly enlarged, smooth, soft prostate. No masses were felt. There was slight scoliosis of the spine. Laboratory and x-ray examination. Blood showed red blood cells 4,610,000, hemoglobin 65 per cent, white blood cells 9000, non protein nitrogen 45 mgm. per 100 cc. Urine examination revealed many red blood cells with few epithelial cells. Temperature, pulse and respiration were normal. Cystoscopy showed an essentially negative bladder. Ureteral orifices were normal in size, shape and position. An efflux of clear urine was seen coming from the left orifice but none appeared on the right. A No. 6 catheter was passed to the left kidney pelvis meeting no obstruction, and a normal flow of clear urine was obtained. A left pyelogram was negative. A No. 6 opaque catheter was obstructed on the right side 2 cm. above the ureteral orifice, and repeated attempts to pass the obstruction were unsuccessful. Following this a rather free flow of blood was seen coming from the right ureteral opening. No sodium iodide solution could be forced past the obstruction. A ureterogram of the lower part of the ureter gave a dilated, moth-eaten appearance resembling the picture that is often seen in tuberculosis of the ureter. An intravenous pyelogram with uroselectan was performed the day after admission and showed a normal left kidney pelvis, calyces and left ureter but no dye was visualized on the right side. The lower pole of the right kidney was visible and appeared normal. The findings were those of impaired function of the right kidney. A second cystoscopy performed two days after admission showed the same results as the first. Examination of the sediment from the left ureter was negative. Indigo carmine injected intravenously appeared on the left side in three minutes but nothing appeared on the right. The barium enema report was:
PRIMARY CARCINOMA OF URETER
579
"The colon filled without delay. No defects observed. Cecum is high in position but sharp in contour and regular. Examination essentially negative." X-rays of the chest were essentially negative. A flat abdominal plate showed no evidence of metastases and the pelvis was negative.
FIG.
1.
PYELOGRAM DEMONSTRATING COMPLETE OBSTRUCTION OF LOWER THIRD OF RIGH'r URETER
r Operation. On December 12, 1930, a partial ureterectomy and exploration were performed by Dr. J. D. Barney and Dr. Ross Mintz. Preliminary diagnosis was ectopic right kidney. Postoperative diagnosis was tumor of ureter with question of malignancy. An extraperitoneal incision made on the right, parallel to Poupart's
580
W. H. SNYDER, JR., AND B. S. WOOD
ligament, revealed a cecum which was adherent to the pelvic wall. After dissecting this off, a hard mass, deep red in color, the size of an English walnut, was exposed. From the lower end of this mass a large dilated ureter was definitely isolated, clamped and divided and the distal end tied. The mass described was dissected free from its surroundings leaving a considerable amount of adherent tissue overlying the common iliac arteries. A thick-walled structure resembling the ureter was clamped and tied as it came from the upper pole of the tumor. As this structure was cut through there was a discharge of cloudy urine. This proximal portion was dissected up to a fairly normal feeling lower pole of the right kidney. It was impossible to consider doing a nephrectomy as the patient was not in good condition and the incision was too low for this procedure. Accordingly the ureter was ligated and dropped back into the wound. Course. Four days after operation the patient died of what appeared to be an acute peritonitis. He coughed up some mucopurulent material on the second day. On the third day his respirations rose to 40 per minute and he became slightly cyanotic. Pulmonary embolism was considered but felt unlikely. The abdomen was tense and distended. The respirations were short and labored. His pulse was rapid and thready and the patient became comatose shortly before death. Surgical specimen. Pathological report by Dr. John I. Bradley of the specimen obtained at operation was as follows: "The specimen consists of a pyramidal-shaped mass of rather firm, yellowish-white, smooth tissue measuring 4.2 by 2.6 by 1.3 cm. At each end, there is a small portion of patent ureter protruding. Section shows a firm, yellowish, white, ovoid mass, which definitely infiltrates the ureteral wall and practically occludes the ureter for a distance of about 4 cm. The entire mass is within the ureter. The mucosal surface in the involved portion is irregular and eroded. "Microscopic examination shows an epidermoid carcinoma, composed of medullary masses of rather poorly differentiated but uniform cells. Mitotic figures are infrequent. The tumor is regarded as having a medium rate of growth and a rather low degree of malignancy (epidermoid carcinoma)." Autopsy report. A resume of the positive findings in the post mortem examination is as follows: 1. Gross report. The right kidney was slightly smaller than usual. The peritoneal fat was firmly adherent and the capsule stripped away with moderate difficulty. The kidney was fluctuant. On section the
--------------- --- - - - - - - - - - ~ --- - - - - - ~ - - - - - - -
PRIMARY CARCINOMA OF URETER
581
pelvis and calyces were moderately dilated and filled with thin, yellowish, turbid fluid. The cortex and medulla were thin. Only about 6 cm. of the upper end of the right ureter was present. Its lower portion was occluded and tied by catgut. The portion of the ureter above the
FIG.
2.
PHOTOGRAPH OF CARCINOMA OF RIGHT URETER REMOVED AT OPERATION
tie was dilated. In the region of the promonotory of the sacrum there were a few moderately enlarged, firm glands. These extended up to the level of the first lumbar vertebra. The largest one measured 2 cm. in diameter. Its cut surface was yellow, finely granular, opaque, and
582
W. H. SNYDER, JR., AND B. S. WOOD
showed a number of focal areas of hemorrhagic necrosis. No other metastases were found in the liver, kidney, spleen, pancreas or other organs. The lung showed bronchial pneumonia and lung abscesses. 2. Microscopic report. In the retroperitoneal glands "the normal architecture is destroyed by diffuse infiltration of large, poorly differentiated tumor cells, epithelial in character and showing definite areolar arrangement. There are many mitoses. There is moderate fibrosis of the gland. The capsule and surrounding fat is invaded." 3. Anatomic diagnosis: (Carcinoma of the ureter); metastasis to retroperitoneal lymph-nodes; hydronephrotic atrophy of the kidney; bronchopneumonia; lung abscess; emphysema. ANALYSIS OF PREVIOUSLY REPORTED CASES
We have been able to find 69 cases previously described in the literature which we feel are acceptable. These include all but 8 of the 48 cases presented by Rousselot and Lamon and in addition 17 other cases reported in the literature, plus our own case, making a total of 70 accepted cases. We have reviewed all the articles in our bibliography except a few cases abstracted by Rousselot and Lamon which have sufficient data to meet the requirements of the other chosen cases. We have excluded certain previously credited cases because of one or more of the following reasons: 1. Microscopic report not obtainable such as in the cases of Suter (68), one case, Zobel (79), Rayer (56), Poll (52) and Kneise (35), one case. 2. Reasonable doubt as to malignancy of the tumor, such as in the cases of Walker (76), Poll (52) and Kraft (36). 3. Renal or metastatic origins of the tumor not satisfactorily excluded as in the cases of Rayer (56), Albaran (2), Rathbun (55), Kneise (35), one case, Toupet and Gueniot (69). In these cases either the kidney was not examined or there was evidence of extraureteral origin. 4. Full r!:i_port of the cases not obtainable as in case reported by Zobel
(79). In 32 of the 70 accepted cases the possibility of a metastatic origin of the ureteral carcinoma has been certainly excluded by post-mortem examinations. While the remaining 38 cases lack the final proof of autopsy, all the evidence obtained indicates that the growth was primary in the ureter. Successful results from nephroureterectomy have been claimed in 30 of the 50 operative cases but satisfactory follow-up is rarely reported. Five cases are reported well after observation of a year or
PRIMARY CARCINOMA OF URETER
583
more; namely those of Chiari (10), Crance and Knickerbocker (11), Kneise (34), Player (51) and Suter (68). COMMENT
This is the first case on record in the files of the Massachusetts General Hospital. The cardinal symptoms of pain and hematuria were present but there was nothing in the history to distinguish the condition from other more common urinary disturbances. Catheterization and pyelograms revealed an obstructing lesion low in the right ureter which, coupled with the physical findings of a mass in the right lower quadrant, suggested an ectopic kidney. This was the preoperative diagnosis. In retrospect, the rather free bleeding on ureteral catheterization should probably have been given more consideration. As Glas pointed out, free bleeding is suggestive of tumor in contrast to the peristaltic type of bleeding more frequently seen with stone or other types of obstruction. In view of the condition found at operation and autopsy it seems probable that a successful result might have been expected had the patient been seen earlier in the disease. Excision of the growth could not be followed by nephrectomy, which is the indicated treatment, because of the poor condition of the patient. Death in this case was due primarily to the obstruction of the ureter which in turn resulted in destruction of the kidney with accompanying infection ending in generalized loss of resistance and a final overwhelming infection. The fact that ureteral tumors, like bladder tumors, as a rule metastasize late, makes early operation with resection of the growth a worth while objective. The difficulty lies in making the diagnosis. SUMMARY
A case of carcinoma of the ureter has been reported in some detail. A review of the literature discloses 69 acceptable cases. Reasons for exclusion of previously reported cases have been stated and results of surgical treatment have been given. We feel justified in making this report as a means of bringing before the reader again the existence of this rather rare condition so that it may be more frequently considered in a differential diagnosis.
584
W. H. SNYDER, JR., AND B. S. WOOD
REFERENCES (1) (2) (3) (4) (5) (6) (7) (8) (9) (IO)
(11) (12) (13) (14) (15) (16) (17) (18) (19) (20) (21) (22) (23) (24) (25) (26) (27) (28) (29) (30) (31) (32) (33) (34) (35) (36) (37) (38) (39)
tADLER, L.: Monatsb. f. Urol., 1905, x, 129. :j:ALBARRAN, J.: Bull. et Mem. de la Soc. de Chir., Paris, 1906, xxxii, 669. *AscHNER, P. W.: Surg., Gynecol. and 0bstet., 1922, xxxv, 749. tAscoLI, R.: Arch. Ital. di Urol., 1930, vi, 611. *BLATT, P. : Wien. Klin. Wchnschr., 1926, xxxix, 1130. tBuTLER, F. A.: Clifton Med. Bull., 1930, ii, 48. *CHAUVIN, E. : Jour. d'Urol., 1930, xxix, 421. CHAUVIN, E., AND CERATI: Arch. d. Mal. d. Reins, 1931, v, 631. *CHEVAssu, M., AND MocK, J.: Bull. et Mem. Soc. de Chir., Paris, 1912, xxxviii, 522. *CHIART, 0. M . : Ztschr. f. Urol., 1914, viii, 672. *CRANCE, A. M., AND KNICKERBOCKER, H .: Jour. Amer. Med. Assoc., 1924, lxxxii, 1930. tCRoss, J. B.: Jour. Med. Assoc., Georgia, 1931, xx, 135. *D'AUNOY, R., AND ZOELLER, A.: Arch. Path., 1930, ix, 17. *DAVIS, E ., AND SAcHs, A.: Jour. Amer. Med. Assoc., 1931, xcvi, 2096. tDAVY, R.: Brit. Med. Jour., 1884, ii, 757. *DAY, R. V., FAIRCHILD, F . D., AND MARTIN, H. W. : Surg., Gynecol. and 0bstet., 1925, xl, 486. *DzIALOSZYNSKI, A.: Ztschr. f. Urol., 1929, xxiii, 1004. *EINzIG: Ztschr. f. Urol., 1931, xxv, 649. *FINSTERER, H.: Wien. Klin. Wchnschr., 1915, xxvi, 718. tGERSTEIN, K . : Inaugural Dissertations, Kiel, 1902. tGLAs, R.: Wien. Klin. Wchnschr., 1926, xxxix, 1145. *GOTTLIEB, J.: Ztschr. f. Urol. Chir., 1926, xx, 230. *GRUNEBERG, J.: Ztschr. f. Urol., 1930, xxiv, 260. *HAEBLER: Reported by Dzialoszynski. tHEKTOEN, L.: Jour. Amer. Med. Assoc., 1896, xxvi, 1115. *HUNT, V. C.: Surg. Clin. No. Amer., 1927, vii, 1464. tisRAEL, J.: Berl. Klin. Wchnschr., 1910, xlvii, 2381. tJoNA, G. : Centralbl. f. Allg. Path. u. Path. Anat., 1894, v, 649. *JUDD, E. S., AND STRUTHERS, J . E.: Jour. Urol., 1921, vi, 115. tKLAGEs, F.: Ztschr. f. Urol. Chir., 1929, xxviii, 413. tKLAGEs, F .: Ztschr. f. Urol. Chir., 1929, xxviii, 413. *KLEINSCHMIDT, R.: Deutsche Ztschr. f. Chir., 1925, cxci, 103. tKNACK: Deutsche Med. Wchnschr., 1918, xliv, 982. *KNEISE, 0. : Ztschr. f. Urol., 1930, xxiv, 10. :j:KNEISE, 0.: Ztschr. f . Urol., 1930, xxiv, 10. :j:KRAFT: Ztschr. f. Urol., 1930, xvi, 385. *KRETSCHMER, H . L.: Surg., Gynecol., and 0bstet., 1924, xxxviii, 47. *MACALPINE, J. B.: Proc. Roy. Soc. Med., 1928, xxi, 1889. t MARIANI, G. : Atti. d. r . Acad. d. F isiocrit. in Siena, 1931, v, 341.
* Indicates an accepted case. t Indicates an accepted case with reported autopsy. :j: Indicates a doubtful case.
PRIMARY CARCINOMA OF URETER
585
(40) *McCowN, P. E.: Jour. Amer. Med. Assoc., 1930, xciv, 468. (41) tMEEKER, L. H., AND McCARTHY: Jour. Amer. Med. Assoc., 1923, lxxxi, 104. (42) *METCALF, W. F., AND SAFFORD, H. E.: Amer. Jour. Med. Sci., 1905, cxxix, 50. (43) tMINICH, K.: Pest. Med. Chir. Presse., 1902, xxiv, 941. (44) *MORSE, H. D.: Canad. Med. Assoc. Jour., 1925, xv, 902. (45) *MRAZ, J. Z.: Jour. Urol., 1924, xii, 49. (46) *NILSSEN, A.: Kristiania Kirurg. Forh., 1915, xiii. (47) tOcKERBLAD, N. F., AND HELWIG, F. C.: Jour. Urol., 1930, xxiv, 451. (48) *PAPIN, M.: Jour. d'Urol., 1924, xvii, 325. (49) tPASCHKIS, R.: Wien. Klin. Wchnschr., 1910, xxiii, 361. (50) *PASCHKIS, R., AND PLESCHNER, H.: Med. Klin., 1920, xvi, 1254. (51) *PLAYER, L. F.: Urol. and Cutan. Rev., 1928, xxv, 438. (52) tPoLL, B.: Beitr. z. Clin. Chir., 1899, xxiii, 822. (53) *POSNER, H. L.: Monatschr. f. Geburtsch. u. Gynaek., 1926, lxxv, 86. (54) *QUINBY, W. C.: Jour. Urol., 1920, iv, 439. (55) tRATHBUN: Int. Jour. Surg., 1920, xxxiii, 157. (56) tRAYER, P.: Traite des Maladies des Reins. Paris, 1841, Tome III, p. 699. (57) tRENNER, M. J.: Surg., Gynecol. and Obstet., 1931, Iii, 793. (58) tRICHTER, J.: Ztschr. f. Urol., 1909, iii, 416. (59) *ROHRER, P. A.: Jour. Urol., 1930, xxiv, 639. (60) tRoussELOT, L. M., AND LAMON, J. D.: Surg., Gynecol. and Obstet., 1930, 1, 17. (61) tRuNDLE, H.: Tr. Path. Soc. London, 1896, xlvii, 128. (62) tST. THOMAS HosP. REPORTS: 1904, xxxii, 96. (63) *SCHEELE, K.: Fortschr. a. d. geb. d. Roentgenstrahlen, 1927, xxxvi, 825. (64) tScHMITT, E. C.: Jour. Cancer Research, 1916, i, 461. (65) tSoMMER, R.: Ztschr. f. Urol., 1932, xxvi, 1. (66) tSPEiss, P.: Centralbl. f. Allg. Path. u. Path. Anat., 1915, xxvi, 553. (67) *STEWART, R. L;: Brit. Jour. Surg., 1926, xiii, 667. (68) *SUTER, F.: Zeitschr. f. Urol. Chir., 1922, x, 522. (69) tTouPET AND GuENIOT, P.: Bull. Soc. Anat. de Paris, 1898, xii, 677. (70) *UNIVERSITY COLLEGE HosPITAL: Museum specimen, Brit. Jour. Urol., 1930, ii, 170. (71) *VAN CAPELLEN: Beitr. z. Klin. Chir., 1916, xcix, 138. (72) *VIETHEN, H.: Ztschr. f. Urol. Chir., 1930, xxix, 274. (73) tVoELCHER, A. F.: Tr. Path. Soc. London, 1895, xlvi, 133. (74) tVoLANTE: Arch. Ital. di Urol., 1927, iv, 105. (75) tVoRPAHL, K.: Amer. Jour. Urol., 1905, ii, 509. (76) tWALKER,"J. W. T.: Proc. Roy. Soc. Med., 1921, xiv, 42. (77) tWISING, P. J., AND BLIX, C.: Hygeia, 1878, xl, 468. (78) tZrnoNI, G.: Ann. d. Mal, d'Org. Genito-Urin., 1909, ii, 81. (79) tZoBEL, H.: Centralbl. f. Allg. Path. u. Path. Anat., 1925, xxxvi, 142.
w.
H. SNYDER, JR.,
AND
B.
s.
WOOD
From the Urological Department, Massachusetts General Hospital, Boston, Massachusetts
Our purpose in this paper is to add a new case of primary carcinoma of the ureter to the literature, to analyze briefly the cases previously reported and to comment on the chief features of the case we present. In view of the excellent recent contributions of Rousselot and Lamon (60), D'Aunoy and Zoeller (13), McCown (40) and Renner (57), we do not believe resummarizing past records would be of value at the present time. CASE REPORT
History. A forty-nine-year-old, white, male, woodworker entered the Emergency Ward of the Massachusetts General Hospital on December 8, 1930, complaining of pain in the right loin of five months duration. Five months previous to admission, while sitting at work, the patient was seized with sharp pain in his right loin radiating to the anterior aspect of the right leg and to the right testicle. Prior to the present illness he was well but had noticed a gradual loss of strength. On the morning of the acute onset he complained of burning on urination and noticed that the urine was reddish brown in color. Daily thereafter he had a dull ache in his right loin with some tenderness in the right costovertebral angle. Several times he had attacks of true renal colic. The most recent one, two weeks before admission, radiated to the right testicle (November, 1930). The acute attacks lasted on the average of about four hours. With the exception of the first attack hematuria, dysuria, urgency and frequency had not been present but the patient had had nocturia during the three weeks before admission. There had been a loss in weight of 12 pounds in the two weeks before admission. He had had no cardio-respiratory or neuromuscular disturbances. Gastrointestinal symptoms were those of marked constipation, definitely 577
578
W. H. SNYDER, JR., AND B. S. WOOD
increased during the three weeks previous to admission. At times 3 tablespoons of magnesium sulphate had no effect. The patient had never noticed tarry, mucoid, bloody or clay-colored stools but had recently had considerable tenesmus. Physical examination. The patient was a rather small, underdeveloped and poorly nourished male, lying in bed complaining of right loin pain. There was internal strabismus of the left eye. The tongue was furred. There were no enlarged glands. The heart was enlarged, rather rapid and with occasional extra-systoles. The lungs were essentially negative. In the abdomen there was an irregular, firm mass in the right lower quadrant about 2 by 3 cm. which was tender on pressure and definitely adherent to the surrounding structures. Rectal examination revealed a slightly enlarged, smooth, soft prostate. No masses were felt. There was slight scoliosis of the spine. Laboratory and x-ray examination. Blood showed red blood cells 4,610,000, hemoglobin 65 per cent, white blood cells 9000, non protein nitrogen 45 mgm. per 100 cc. Urine examination revealed many red blood cells with few epithelial cells. Temperature, pulse and respiration were normal. Cystoscopy showed an essentially negative bladder. Ureteral orifices were normal in size, shape and position. An efflux of clear urine was seen coming from the left orifice but none appeared on the right. A No. 6 catheter was passed to the left kidney pelvis meeting no obstruction, and a normal flow of clear urine was obtained. A left pyelogram was negative. A No. 6 opaque catheter was obstructed on the right side 2 cm. above the ureteral orifice, and repeated attempts to pass the obstruction were unsuccessful. Following this a rather free flow of blood was seen coming from the right ureteral opening. No sodium iodide solution could be forced past the obstruction. A ureterogram of the lower part of the ureter gave a dilated, moth-eaten appearance resembling the picture that is often seen in tuberculosis of the ureter. An intravenous pyelogram with uroselectan was performed the day after admission and showed a normal left kidney pelvis, calyces and left ureter but no dye was visualized on the right side. The lower pole of the right kidney was visible and appeared normal. The findings were those of impaired function of the right kidney. A second cystoscopy performed two days after admission showed the same results as the first. Examination of the sediment from the left ureter was negative. Indigo carmine injected intravenously appeared on the left side in three minutes but nothing appeared on the right. The barium enema report was:
PRIMARY CARCINOMA OF URETER
579
"The colon filled without delay. No defects observed. Cecum is high in position but sharp in contour and regular. Examination essentially negative." X-rays of the chest were essentially negative. A flat abdominal plate showed no evidence of metastases and the pelvis was negative.
FIG.
1.
PYELOGRAM DEMONSTRATING COMPLETE OBSTRUCTION OF LOWER THIRD OF RIGH'r URETER
r Operation. On December 12, 1930, a partial ureterectomy and exploration were performed by Dr. J. D. Barney and Dr. Ross Mintz. Preliminary diagnosis was ectopic right kidney. Postoperative diagnosis was tumor of ureter with question of malignancy. An extraperitoneal incision made on the right, parallel to Poupart's
580
W. H. SNYDER, JR., AND B. S. WOOD
ligament, revealed a cecum which was adherent to the pelvic wall. After dissecting this off, a hard mass, deep red in color, the size of an English walnut, was exposed. From the lower end of this mass a large dilated ureter was definitely isolated, clamped and divided and the distal end tied. The mass described was dissected free from its surroundings leaving a considerable amount of adherent tissue overlying the common iliac arteries. A thick-walled structure resembling the ureter was clamped and tied as it came from the upper pole of the tumor. As this structure was cut through there was a discharge of cloudy urine. This proximal portion was dissected up to a fairly normal feeling lower pole of the right kidney. It was impossible to consider doing a nephrectomy as the patient was not in good condition and the incision was too low for this procedure. Accordingly the ureter was ligated and dropped back into the wound. Course. Four days after operation the patient died of what appeared to be an acute peritonitis. He coughed up some mucopurulent material on the second day. On the third day his respirations rose to 40 per minute and he became slightly cyanotic. Pulmonary embolism was considered but felt unlikely. The abdomen was tense and distended. The respirations were short and labored. His pulse was rapid and thready and the patient became comatose shortly before death. Surgical specimen. Pathological report by Dr. John I. Bradley of the specimen obtained at operation was as follows: "The specimen consists of a pyramidal-shaped mass of rather firm, yellowish-white, smooth tissue measuring 4.2 by 2.6 by 1.3 cm. At each end, there is a small portion of patent ureter protruding. Section shows a firm, yellowish, white, ovoid mass, which definitely infiltrates the ureteral wall and practically occludes the ureter for a distance of about 4 cm. The entire mass is within the ureter. The mucosal surface in the involved portion is irregular and eroded. "Microscopic examination shows an epidermoid carcinoma, composed of medullary masses of rather poorly differentiated but uniform cells. Mitotic figures are infrequent. The tumor is regarded as having a medium rate of growth and a rather low degree of malignancy (epidermoid carcinoma)." Autopsy report. A resume of the positive findings in the post mortem examination is as follows: 1. Gross report. The right kidney was slightly smaller than usual. The peritoneal fat was firmly adherent and the capsule stripped away with moderate difficulty. The kidney was fluctuant. On section the
--------------- --- - - - - - - - - - ~ --- - - - - - ~ - - - - - - -
PRIMARY CARCINOMA OF URETER
581
pelvis and calyces were moderately dilated and filled with thin, yellowish, turbid fluid. The cortex and medulla were thin. Only about 6 cm. of the upper end of the right ureter was present. Its lower portion was occluded and tied by catgut. The portion of the ureter above the
FIG.
2.
PHOTOGRAPH OF CARCINOMA OF RIGHT URETER REMOVED AT OPERATION
tie was dilated. In the region of the promonotory of the sacrum there were a few moderately enlarged, firm glands. These extended up to the level of the first lumbar vertebra. The largest one measured 2 cm. in diameter. Its cut surface was yellow, finely granular, opaque, and
582
W. H. SNYDER, JR., AND B. S. WOOD
showed a number of focal areas of hemorrhagic necrosis. No other metastases were found in the liver, kidney, spleen, pancreas or other organs. The lung showed bronchial pneumonia and lung abscesses. 2. Microscopic report. In the retroperitoneal glands "the normal architecture is destroyed by diffuse infiltration of large, poorly differentiated tumor cells, epithelial in character and showing definite areolar arrangement. There are many mitoses. There is moderate fibrosis of the gland. The capsule and surrounding fat is invaded." 3. Anatomic diagnosis: (Carcinoma of the ureter); metastasis to retroperitoneal lymph-nodes; hydronephrotic atrophy of the kidney; bronchopneumonia; lung abscess; emphysema. ANALYSIS OF PREVIOUSLY REPORTED CASES
We have been able to find 69 cases previously described in the literature which we feel are acceptable. These include all but 8 of the 48 cases presented by Rousselot and Lamon and in addition 17 other cases reported in the literature, plus our own case, making a total of 70 accepted cases. We have reviewed all the articles in our bibliography except a few cases abstracted by Rousselot and Lamon which have sufficient data to meet the requirements of the other chosen cases. We have excluded certain previously credited cases because of one or more of the following reasons: 1. Microscopic report not obtainable such as in the cases of Suter (68), one case, Zobel (79), Rayer (56), Poll (52) and Kneise (35), one case. 2. Reasonable doubt as to malignancy of the tumor, such as in the cases of Walker (76), Poll (52) and Kraft (36). 3. Renal or metastatic origins of the tumor not satisfactorily excluded as in the cases of Rayer (56), Albaran (2), Rathbun (55), Kneise (35), one case, Toupet and Gueniot (69). In these cases either the kidney was not examined or there was evidence of extraureteral origin. 4. Full r!:i_port of the cases not obtainable as in case reported by Zobel
(79). In 32 of the 70 accepted cases the possibility of a metastatic origin of the ureteral carcinoma has been certainly excluded by post-mortem examinations. While the remaining 38 cases lack the final proof of autopsy, all the evidence obtained indicates that the growth was primary in the ureter. Successful results from nephroureterectomy have been claimed in 30 of the 50 operative cases but satisfactory follow-up is rarely reported. Five cases are reported well after observation of a year or
PRIMARY CARCINOMA OF URETER
583
more; namely those of Chiari (10), Crance and Knickerbocker (11), Kneise (34), Player (51) and Suter (68). COMMENT
This is the first case on record in the files of the Massachusetts General Hospital. The cardinal symptoms of pain and hematuria were present but there was nothing in the history to distinguish the condition from other more common urinary disturbances. Catheterization and pyelograms revealed an obstructing lesion low in the right ureter which, coupled with the physical findings of a mass in the right lower quadrant, suggested an ectopic kidney. This was the preoperative diagnosis. In retrospect, the rather free bleeding on ureteral catheterization should probably have been given more consideration. As Glas pointed out, free bleeding is suggestive of tumor in contrast to the peristaltic type of bleeding more frequently seen with stone or other types of obstruction. In view of the condition found at operation and autopsy it seems probable that a successful result might have been expected had the patient been seen earlier in the disease. Excision of the growth could not be followed by nephrectomy, which is the indicated treatment, because of the poor condition of the patient. Death in this case was due primarily to the obstruction of the ureter which in turn resulted in destruction of the kidney with accompanying infection ending in generalized loss of resistance and a final overwhelming infection. The fact that ureteral tumors, like bladder tumors, as a rule metastasize late, makes early operation with resection of the growth a worth while objective. The difficulty lies in making the diagnosis. SUMMARY
A case of carcinoma of the ureter has been reported in some detail. A review of the literature discloses 69 acceptable cases. Reasons for exclusion of previously reported cases have been stated and results of surgical treatment have been given. We feel justified in making this report as a means of bringing before the reader again the existence of this rather rare condition so that it may be more frequently considered in a differential diagnosis.
584
W. H. SNYDER, JR., AND B. S. WOOD
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