- Email: [email protected]
Tumors of the Renal Pelvis1
TlJl\11:0RS OF THE RENAL PELVIS 1 A REVIEW OF THE LITERATURE AND REPORT OF A CASE DAVID WO MACKENZIE AND MAX RATNER From the Department of Urology, Royal Victoria Hospital Montreal, Canada
New growths of renal pelvis as compared with those of the kidney parenchyma are relatively infrequent. It is estimated that on an average only 5 to 7 per cent of all renal tumors occur primarily in the kidney pelvis. Thus Albarran in 1901 found 47 tumors of the kidney pelvis in a series of 625 renal growths. In a collection of 585 cases Albarran and Imbert in 1903 were able to find only 42 cases that arose primarily in the renal pelvis. Recently Hunt reported on 318 cases; only 13 were growths of the kidney pelvis. From these reports it is obvious that only a small percentage of kidney tumors have their origin in the pelvis. Of course there are reports showing higher proportions but these are scarce. Thomson-Walker for example found 12 cases of tumor of the renal pelvis in a personal series of 66 renal growths. Since 1917 there have been 96 cases of tumor of the kidney in our Service at the Royal Victoria Hospital. Of these only one was a true growth of the renal pelvis. Up till the present time there are in the literature about 218 cases of growth of the kidney pelvis. In 1926 Meltzer reported 181 cases, and in 1927 Hunt added 7 more, making a total of 188. Since 1927 about 30 additional cases have been found in the literature. PATHOLOGY
The types of tumor that arise in the renal pelvis are similar to those that occur in the bladder. Practically all of them are 1 Paper read at the meeting of the American Association of Genito-urinary Surgeons, Niagara Falls, Canada, May 26, 1932.
405
406
DAVID W. MACKENZIE AND MAX RATNER
derived from mesodermal tissue. There is also a small number of growths that are of the squamous cell type. Whether this is due to metaplasia or to a developmental abnormality is not definite. Ewing classifies the growths of the renal pelvis as follows : (I) papilloma; (2) papillary epithelioma; (3) squamous cell carcinoma; and (4) alveolar carcinoma. Thomas and Regnier follow Ewing's classification rather closely but add in addition the so called connective tissue tumors. The latter is a very small group and includes the adenoma, fibroma and sarcoma. Papilloma
About 40 to 50 per cent of all tumors of the renal pelvis belo,ng to this class. They are usually multiple and appear as villous or wart-like growths similar to those found in the bladder. When placed in water they become somewhat branched and elongated, and resemble very closely seaweed. Occasionally the growth has an extensive distribution and may involve the ureter, the bladder and even the ureter on the opposite side. Often there is one large tumor and several smaller ones surrounding it. Frequently these growth are associated with salty incrustations and even with definite calculi. These papillomata are all very vascular and bleed easily. This accounts for the marked haematuria that is usually the outstanding symptom in the disease. Microscopically the picture is typical. The structure of the papilloma is simple and uniform. It consists of a series of branching clusters of stroma covered by many layers of transitional epithelium. The stroma usually is made up of fine branching blood vessels which are in close association with some connective tissue and smooth muscle fibres. The tumor cells are cubical, cylindrical or elongated, and there is present round cell infiltration. The base of the tumor is free from any invasion of the tumor cells. These growths are usually quite benign but have the tendency, like the simple papillomata of the bladder, to become malignant. They also have the quality of producing transplants along the entire course of the ureter and even down to the bladder.
TUMORS OF THE RENAL PELVIS
407
Papillary epithelioma
This group comprises about 20 to 30 per cent of the growths of the renal pelvis. These tumors are also wart-like character but appear more compact involve a greater area than the simple papillomata. The villous processes are usually not as long or branching, and very often show areas of ulceration. Here too the growth may involve the entire ureter and even the bladder. In the early stages of the growth there is definite evidence of involvement of the submucosa and later the renal parenchyma is encroached upon. In advanced cases there is usually distension of the renal pelvis and the presence of multiple cysts in the cortex of the kidney. These tumors are also extremely vascular and bleed very easily. Microscopically the tumor cells are found to have invaded the base of the tumor and the submucosa. In some growths one finds areas of benign and malignant cells side by side suggesting a transformation of a benign to a malignant tumor. Alveolar carcinoma
This form of tumor is probably a far advanced papillomatous growth which has lost its papillomatous character. It now assumes an alveolar or scirrhous type of growth. These tumors are large) infiltrating very malignant and form metastases very readily. Squamous cell carcinoma
This is a small group but a fair number of cases have been reported in the literature. Kretschmer found 2 cases in a series of forty-three non-papillary growths of the renal pelvis. In addition he found 11 cases of pavement epithelioma, one epidermoid carcinoma and four epitheliomata, all showing a tendency to form epithelium resembling the skin. Scholl and Foulds in 1924 have reported 5 cases. Recently Bowen and Bennett have also reported such a case. All the authors emphasize the importance of previous infection as a causative agent. Leukoplakia with calculi also seems to play a part as
408
DAVID W. MACKENZIE AND MAX RATNER
several of the cases described have been associated with these conditions. Apparently the epithelium of the renal pelvis is capable of epidertnidization. These tumors are usually quite large and the squamous qualities are quite pronounced. There is often pronounced hornification with the presence of many epithelial pearls. These growths are definitely less vascular than the papillomata. They grow rapidly and involve the renal parenchyma and the surrounding tissues. CONNECTIVE TISSUE TUMORS
Histoid growths of the pelvis are extremely rare. They include the fibroma, adenoma and the sarcoma. Plant in 1929 reports a diffuse adenoma of the renal pelvis. Boross also in 1929 describes a case with a fibroma of the renal pelvis. METASTASES
The simple papilloma is benign but has the inherent quality of spreading. This often results in very extensive growths involving the kidney parenchyma, the ureter, the bladder, and even the ureter on the opposite side. The papillary epithelioma is a malignant growth and very of ten takes the form of a:p. infiltrating carcinoma. Metastases are found in the adrenals, peritoneum, liver, lungs, bones and lymph nodes'. The squamous cell and the alveolar carcinoma are both highly malignant. Metastases usually occur early and may involve any organ of the body. ETIOLOGY
Age periods. The majority of the cases on record have occurred between the ages of forty and sixty years. The youngest patient reported was two years of age (Kananka); the oldest patient eighty-two years of age. Sex. Males seem to be affected more often than females. In a large series of cases that Thomas and Regnier reported 62 per cent of them occurred in males. Infection. Chronic infection of the renal pelvis seems to play
TUMORS OF THE RENAL PELVIS
409
a prominent part in these tumors. Many of the cases reported have had a history of renal infection, calculi, or of both. As a result several of the authors emphasize the dangers of neglecting infections and stones in the kidney. Scholl and Foulds have reported 5 cases of squamous cell carcinoma of the renal pelvis; 4 of which had calculi and infection. Kretschmer in 1917 reviewed 43 cases of non-papillary growths of the renal pelvis, of these 11 were associated with calculi. Bowen and Bennett have also reported a similar case just recently (1930). CONGENITAL ABNORMALITIES
There does not seem to be any special predisposition to new growths in the congenitally abnormal pelves. There are however a few cases on record. Thomas and Regnier mention a case in a horseshoe kidney. Roberts in 1928 describes a case in a double ureter and pelvis. The tumor was present in the lower pelvis. SYMPTOMATOLOGY
H aematuria, The most outstanding symptom tumors of the renal pelvis is haematuria. In a large series of cases studiedJ Thomas and Regnier found that 67 per cent of them had blood in the urine at one time or another in the course the disease. Moreover in half the number of the cases the first symptom noticed was bloody urine. In their own personal series (5 cases) haematuria was the outstanding symptom. Meltzer states that haematuria is present in 70 per cent of the papillary growths and in 50 per cent of the non-papillary growths. An interesting fact is that several cases have intermittent attacks of haematuria with long intervals of freedom. There are a few cases on record that have been free from haematuria for periods from ten to twenty years. Pain. This symptoms is not a constant one. At times a patient will complain of a dull ache in one or other loin. Occasionally there will be a typical renal or ureter.al colic. This is undoubtedly due to the passage of a small transplant or blood clot down the ureter. Rather severe pain may also result when :an
410
DAVID W. MACKENZIE AND MAX RATNER
obstruction occurs at the uretero-pelvic angle causing a hydronephrosis or a haematonephrosis. Finally it must be remembered that there may be no pain whatsoever. Tumor. In a large number of cases no mass can be palpated. This is particularly true when the growth is small or when it is spreading towards the upper pole of the kidney. The most common cause of a palpable tumor is probably an associated hydronephrosis or haematonephrosis due to some obstruction. When the latter is relieved as a result of the passage of a small piece of growth or blood clot the tumor mass very often disappears. Passage of tissue. Manypatientswithgrowthsof therenalpelvis particularly papillomata will volunteer the information that they are always passing small pieces of tissue covered with blood clot. These fragments of tumor resemble very closely those that are passed in cases of bladder tumor, and very often such a diagnosis is made. Loss of weight and strength. These are late symptoms and occur when metastases are present. Urinary symptoms. Frequency, urgency and dysuria occur only when the bladder becomes irritated by blood clots or pieces of tumor. DIAGNOSIS
There are no symptoms or signs that are pathognomonic of a tumor of the renal pelvis. In order to arrive at a possible diagnosis all available data are to be studied. The history, physical findings, urinary studies and finally cystoscopy with pyelography are all essential in the diagnosis. Cystoscopy and pyelography are undoubtedly our greatest aids. When the growth has not extended into the bladder the latter viscus appears perfectly normal. Very often, particularly during an attack of haematuria blood may be seen escaping from one or other ureteral orifice. This sign will immediately draw your attention to the diseased side. If the ureter is not obstructed by the growth, the catheter will pass easily to the renal pelvis and bloody urine will be collected. At times however the growth involves the ureter and as a result the catheter may be obstructed.
TUMORS OF THE RENAL PELVIS
411
Retrograde pyelography is to be performed in all cases. If a tumor of the renal pelvis is present there is a definite filling defect but that is not sufficient for a diagnosis. It must remembered that a tumor the renal parenchyma or a blood in the renal pelvis may give you identical pictures, In the differential diagnosis, renal tuberculosis, acute pyelonephritis, renal calculi and tumors of the renal parenchyma are to be excluded. PROGNOSIS
Although the simple papilloma of the renal pelvis is a benign growth yet clinically it is potentially malignant. It has the property of forming transplants in the ureter, bladder and even in the opposite ureter. It is for this reason that nephrectomy and complete ureterectomy with excision of the ureteral orifice is advocated. There are cases on record that were first subjected to a nephrectorny and later had a complete ureterectomy on account of recurrence. Thomson-Walker reports 2 such cases in 1929. The malignant papilloma has a better prognosis than the other malignant growths of the renal pelvis. Metastases occur late and are usually not widespread. The most malignant tumor of the renal pelvis is the squamous cell carcinoma. It metastasizes early and very extensively. The results are very poor even with early treatment. Kretschmer reports on 8 cases. Five died at operation and 3 very soon after. Scholl and Foulds operated on 5 cases: 1 was dead eight days after operation; 3 were dead during the first four months and only 1 was alive and apparently well six months after operation. The alveolar carcinoma is also a very malignant growth. It metastasizes early and may involve any organ or tissue in the body. TREATMENT
On account of tendency of tumors of the renal pelvis to extend down the ureter and even to the bladder, the treatment of choice today is nephrectomy and complete ureterectomy. In the
412
DAVID W. MACKENZIE AND MAX RATNER
past nephrectomy with or without partial ureterectomy was performed. This procedure was found to be too conservative, for several of the patients returned later with involvement of the ureter and even the bladder. Hunt and others are even more radical. They advise nephrectomy, complete uretectomy and segmental resection of that part of the bladder that includes the intramural portion of the ureter. Several authors advise the use of deep x-ray therapy or radium after removal of the growth. CONCLUSIONS
1. New growths of the renal pelvis are still of sufficient rarity
to be of extreme interest to the urologist. 2. The disease is very often wrongly diagnosed. 3. The diagnosis may be made from the history, symptoms, urinary findings, and finally, from cystoscopy and pyelography. 4. The treatment consists of nephrectomy and complete ureterectomy. 5. The prognosis even in simple papilloma is to be guarded. CASE REPORT
The following is the record of the patient with such a growth: J. A. T., male, aged seventy-one years, was admitted to the Urological Service at the Royal Victoria Hospital on December 30, 1931. Complaints. (1) Painless haematuria; (2) frequency of urination. History of present illness. Patient was perfectly well until October 15, 1929, when following a long motor trip he began to pass bright red blood in the urine. There were no other urinary symptoms such as dysuria or frequency of urination. The bleeding lasted off and on for about one month and then disappeared. There was no further haematuria until October, 1931. The blood was always well mixed with the urine, and there was in addition some frequency of urination: day 5- 6; night 1- 2. There were no other urinary symptoms except the passage of small pieces of tissue. There was never any pain in either loin. The patient's private physician examined one of the pieces of tissue and found cells which resembled very closely those that are found in papillary carcinoma of the bladder.
TUMORS OF THE RENAL PELVIS
413
Personal history. Usual childhood diseases. Typhoid fever in young adult life. No other serious diseases. Family history. Mother died of pulmonary tuberculosis. Physical examination. Patient was a well developed and nourished white male. Head: Normal. N eek: Bilaterally enlarged submaxillary glands. Nose and throat: Negative. Respiratory system: Negative. Circulatory system: There was a moderate degree of arteriosclerosis, and a hypertension ranging between 178 to 200 systolic, and 108 to 110 diastolic. The heart was moderately enlarged, and the aortic second sound was markedly accentuated. Electrocardiograph: Shows a left sided preponderance. Regular rate. Normal conduction time. Gastro-intestinal system: The liver was enlarged, the edge was felt two finger breaths below the costal margin. No other organs except the lower pole of the right kidney palpated. Nervous system: Negative. Skin and locomotor system: Negative. Genito-urinary system: Kidneys-no costo-lumbar tenderness on either side. The lower pole of the right kidney was palpated. No further organs or masses palpated on either side. Ureters: No tenderness or masses along the course of either ureter. Bladder: No suprapubic distension or tenderness. Penis: Normal. Urethra: No discharge. A soft ruber catheter no. 14F. passed easily to bladder. Residual urine 4 drams. Scrotum and contents: Normal except for slight varicosity of pampiniform plexus on both sides. Rectal examination: Prominent external haemorrhoids; sphincter tone good. Prostate is moderately enlarged, glandular, smooth, not fixed, not tender. Seminal vesicles not palpated. Laboratory findings. Urine: Smoky reddish, acid, 1018, albumin trace, sugar none. Red blood cells + +. White blood cells a few. Urates. Epithelial cells. One cubic centimeter phthalein intramuscularly: first hour, 35 per cent; second hour, 25 per cent; total, 60 per cent. Blood Wassermann negative. Cystoscopy. No. 24F. instrument easily introduced. There is slight intravesical enlargement of prostate. Bladder mucosa is normal. No THE JOURNAL OF UROLOGY, VOL. XXVIII, NO.
4
414
DAVID W. MAcKENZIE AND MAX RATNER
tumor or foreign body seen. Both ureteral orifices normal in contour and position. Cloudy reddish urine was seen coming from the right
FIG.
1.
PHOTOGRAPH OF THE PYELOGRAM IN THE CASE DISCUSSED
Note the absence of the upper calyces and the marked filling defect in the pelvis
ureteral orifice. Normal urine was seen coming from the left ureteral orifice. Both ureters were easily catheterized to the renal pelves and
TUMORS OF THE RENAL PELVIS
415
specimens obtained. Right: macroscopic, bloody; volume, 8 cc.; white blood cells urea, 0.0035; microscopic, red blood cells + occasionaL Left: Macroscopic, clear watery; volume, 20 cc.; urea, 0.0035; microscopic, red blood cells rare, epithelia.l cells. X-ray with catheters in position shows catheters in normai position. The right kidney shadow is large and indefinite. The left kidney 8hadow is outlined. No shadows suspicious of stone seen. Pyelogram, right, with 8 cc. 12 per cent sodium iodide, shows a definite filling defect of upper portion of pelvis (fig. 1\
+ ++,
li'IG. 2. PHOTOGRAPH OF THE SPECIMEN Note the papillary growth involving the upper prut of the pelvis and extending towards and into the cortex.
Pyelogram, left, with 4 cc. 12 per cent sodium iodide, shows a relatively normal renal pelvis. Diagnosis. Malignant growth of right kidney. In view of the history of passing pieces of tissue, the possibility of a papilloma of the renal pelvis was considered. Treatment. Nephrectomy and ureterectomy, right, on January 2, ]932.
416
DAVID W. MACKENZIE AND MAX RATNER
Pathological report. Right kidney: a kidney slightly larger than normal, split open showing a papillary growth which involves the upper part of the pelvis. This growth appears well demarcated but has compressed the medulla and cortex in this area with the formation of a filbert sized cyst. The remaining portion of the kidney shows considerable peripelvic fat, and definite markings and coarse irregular lobation under the capsule (fig. 2).
Fm. 3.
PHOTOMICROGRAPH
(Low
POWER) SHOWING A DEFlNI'l'E PAPILLOMATOUS GROWTH
Note evidence of degeneration of some of the papillary outgrowths
Microscopic examination. Sections show a papillomatous growth of stratified transitional cells similar to that met with in the bladder and apparently taking origin in the pelvis of the kidney. It has invaded the parenchyma which is compressed and while fairly well demarcated shows evidence of malignant infiltration. Section taken through small pin-
TUMORS OF THE RENAL PELVIS
417
head greyish nodule in cortex shows it to consist of growth similar to that described above. There is also considerable metastatic involvement in the cortex in this area (figs. 3 and 4). Anatomical Summary. Transitional celled papilloma of the pelvis which has become cancerous.
FIG. 4. SIMILAR TO FIGURE 3 (HIGH POWER) There is definite involvement of the parenchyma with stratified transitional cells.
Results. Patient had an uneventful convalescence and was discharged as cured on February I, 1932. May 4, 1932. Reported well.
305 Medical Arts Building, Montreal, Canada
418
DAVID W. MAcKENZIE AND MAX RATNER
REFERENCES ALBARRAN, J.: Neoplasmes primitifs du bassinet et de l'uretere. Ann. d. Mal. d. Org. Genito Urin., Paris, 1900, xviii, 701. ALBARRAN, J., AND IMBERT, L.: Les Tumeurs du Rein. Paris, 1903, Masson et Cie, p. 769. BoWEN, J. A., AND BENNETT, G. A.: Squamous cell carcinoma of the renal pelvis. Jour. Urol., 1930, xxiv, 495. BoRoss, E.: Fibroma of the renal pelvis. Orvosi hetil, October, 1929, lxxiii, 1055. EwrnG, JAMES: Neoplastic Diseases. Third edition, 1928. HUNT, V. C.: Papillary epithelioma of renal pelvis. Jour. Urol., September, 1927, xviii, 225. KANANKA, V.: Malignant papilloma of renal pelvis. Medicina Kaunas, February, 1928, ix, 120. KRETSCHMER, H. L.: Primary non-papillary carcinoma of the renal pelvis. Jour. Urol., 1917, i, 405. MELTZER, M.: Papillary carcinoma of the renal pelvis. Jour. Urol., 1926, xvi, 335. PLANT, A.: Diffuse adenoma of renal pelvis. Ztschr. f. Urol. Chir., 1929, xxvi, 562. RoBERTS, 0. W.: Malignant papilloma of renal pelvis in double ureter and pelvis. Lancet, July, 1928, ii, 67. ScHOLL, A. J., AND FouLDS, G. S.: Squamous-cell tumors of the renal pelvis. Ann. Surg., 1924, lxxx, 594. THOMAS, G. J., AND REGNIER, E. A.: Tumors of the kidney pelvis and ureter. Jour. Urol., 1924, xi, 205. THOMSON-WALKER, Srn J.: Three cases of ureterectomy for papilloma, with comments. Brit. Jour. Urol., June, 1929, i, 141.
New growths of renal pelvis as compared with those of the kidney parenchyma are relatively infrequent. It is estimated that on an average only 5 to 7 per cent of all renal tumors occur primarily in the kidney pelvis. Thus Albarran in 1901 found 47 tumors of the kidney pelvis in a series of 625 renal growths. In a collection of 585 cases Albarran and Imbert in 1903 were able to find only 42 cases that arose primarily in the renal pelvis. Recently Hunt reported on 318 cases; only 13 were growths of the kidney pelvis. From these reports it is obvious that only a small percentage of kidney tumors have their origin in the pelvis. Of course there are reports showing higher proportions but these are scarce. Thomson-Walker for example found 12 cases of tumor of the renal pelvis in a personal series of 66 renal growths. Since 1917 there have been 96 cases of tumor of the kidney in our Service at the Royal Victoria Hospital. Of these only one was a true growth of the renal pelvis. Up till the present time there are in the literature about 218 cases of growth of the kidney pelvis. In 1926 Meltzer reported 181 cases, and in 1927 Hunt added 7 more, making a total of 188. Since 1927 about 30 additional cases have been found in the literature. PATHOLOGY
The types of tumor that arise in the renal pelvis are similar to those that occur in the bladder. Practically all of them are 1 Paper read at the meeting of the American Association of Genito-urinary Surgeons, Niagara Falls, Canada, May 26, 1932.
405
406
DAVID W. MACKENZIE AND MAX RATNER
derived from mesodermal tissue. There is also a small number of growths that are of the squamous cell type. Whether this is due to metaplasia or to a developmental abnormality is not definite. Ewing classifies the growths of the renal pelvis as follows : (I) papilloma; (2) papillary epithelioma; (3) squamous cell carcinoma; and (4) alveolar carcinoma. Thomas and Regnier follow Ewing's classification rather closely but add in addition the so called connective tissue tumors. The latter is a very small group and includes the adenoma, fibroma and sarcoma. Papilloma
About 40 to 50 per cent of all tumors of the renal pelvis belo,ng to this class. They are usually multiple and appear as villous or wart-like growths similar to those found in the bladder. When placed in water they become somewhat branched and elongated, and resemble very closely seaweed. Occasionally the growth has an extensive distribution and may involve the ureter, the bladder and even the ureter on the opposite side. Often there is one large tumor and several smaller ones surrounding it. Frequently these growth are associated with salty incrustations and even with definite calculi. These papillomata are all very vascular and bleed easily. This accounts for the marked haematuria that is usually the outstanding symptom in the disease. Microscopically the picture is typical. The structure of the papilloma is simple and uniform. It consists of a series of branching clusters of stroma covered by many layers of transitional epithelium. The stroma usually is made up of fine branching blood vessels which are in close association with some connective tissue and smooth muscle fibres. The tumor cells are cubical, cylindrical or elongated, and there is present round cell infiltration. The base of the tumor is free from any invasion of the tumor cells. These growths are usually quite benign but have the tendency, like the simple papillomata of the bladder, to become malignant. They also have the quality of producing transplants along the entire course of the ureter and even down to the bladder.
TUMORS OF THE RENAL PELVIS
407
Papillary epithelioma
This group comprises about 20 to 30 per cent of the growths of the renal pelvis. These tumors are also wart-like character but appear more compact involve a greater area than the simple papillomata. The villous processes are usually not as long or branching, and very often show areas of ulceration. Here too the growth may involve the entire ureter and even the bladder. In the early stages of the growth there is definite evidence of involvement of the submucosa and later the renal parenchyma is encroached upon. In advanced cases there is usually distension of the renal pelvis and the presence of multiple cysts in the cortex of the kidney. These tumors are also extremely vascular and bleed very easily. Microscopically the tumor cells are found to have invaded the base of the tumor and the submucosa. In some growths one finds areas of benign and malignant cells side by side suggesting a transformation of a benign to a malignant tumor. Alveolar carcinoma
This form of tumor is probably a far advanced papillomatous growth which has lost its papillomatous character. It now assumes an alveolar or scirrhous type of growth. These tumors are large) infiltrating very malignant and form metastases very readily. Squamous cell carcinoma
This is a small group but a fair number of cases have been reported in the literature. Kretschmer found 2 cases in a series of forty-three non-papillary growths of the renal pelvis. In addition he found 11 cases of pavement epithelioma, one epidermoid carcinoma and four epitheliomata, all showing a tendency to form epithelium resembling the skin. Scholl and Foulds in 1924 have reported 5 cases. Recently Bowen and Bennett have also reported such a case. All the authors emphasize the importance of previous infection as a causative agent. Leukoplakia with calculi also seems to play a part as
408
DAVID W. MACKENZIE AND MAX RATNER
several of the cases described have been associated with these conditions. Apparently the epithelium of the renal pelvis is capable of epidertnidization. These tumors are usually quite large and the squamous qualities are quite pronounced. There is often pronounced hornification with the presence of many epithelial pearls. These growths are definitely less vascular than the papillomata. They grow rapidly and involve the renal parenchyma and the surrounding tissues. CONNECTIVE TISSUE TUMORS
Histoid growths of the pelvis are extremely rare. They include the fibroma, adenoma and the sarcoma. Plant in 1929 reports a diffuse adenoma of the renal pelvis. Boross also in 1929 describes a case with a fibroma of the renal pelvis. METASTASES
The simple papilloma is benign but has the inherent quality of spreading. This often results in very extensive growths involving the kidney parenchyma, the ureter, the bladder, and even the ureter on the opposite side. The papillary epithelioma is a malignant growth and very of ten takes the form of a:p. infiltrating carcinoma. Metastases are found in the adrenals, peritoneum, liver, lungs, bones and lymph nodes'. The squamous cell and the alveolar carcinoma are both highly malignant. Metastases usually occur early and may involve any organ of the body. ETIOLOGY
Age periods. The majority of the cases on record have occurred between the ages of forty and sixty years. The youngest patient reported was two years of age (Kananka); the oldest patient eighty-two years of age. Sex. Males seem to be affected more often than females. In a large series of cases that Thomas and Regnier reported 62 per cent of them occurred in males. Infection. Chronic infection of the renal pelvis seems to play
TUMORS OF THE RENAL PELVIS
409
a prominent part in these tumors. Many of the cases reported have had a history of renal infection, calculi, or of both. As a result several of the authors emphasize the dangers of neglecting infections and stones in the kidney. Scholl and Foulds have reported 5 cases of squamous cell carcinoma of the renal pelvis; 4 of which had calculi and infection. Kretschmer in 1917 reviewed 43 cases of non-papillary growths of the renal pelvis, of these 11 were associated with calculi. Bowen and Bennett have also reported a similar case just recently (1930). CONGENITAL ABNORMALITIES
There does not seem to be any special predisposition to new growths in the congenitally abnormal pelves. There are however a few cases on record. Thomas and Regnier mention a case in a horseshoe kidney. Roberts in 1928 describes a case in a double ureter and pelvis. The tumor was present in the lower pelvis. SYMPTOMATOLOGY
H aematuria, The most outstanding symptom tumors of the renal pelvis is haematuria. In a large series of cases studiedJ Thomas and Regnier found that 67 per cent of them had blood in the urine at one time or another in the course the disease. Moreover in half the number of the cases the first symptom noticed was bloody urine. In their own personal series (5 cases) haematuria was the outstanding symptom. Meltzer states that haematuria is present in 70 per cent of the papillary growths and in 50 per cent of the non-papillary growths. An interesting fact is that several cases have intermittent attacks of haematuria with long intervals of freedom. There are a few cases on record that have been free from haematuria for periods from ten to twenty years. Pain. This symptoms is not a constant one. At times a patient will complain of a dull ache in one or other loin. Occasionally there will be a typical renal or ureter.al colic. This is undoubtedly due to the passage of a small transplant or blood clot down the ureter. Rather severe pain may also result when :an
410
DAVID W. MACKENZIE AND MAX RATNER
obstruction occurs at the uretero-pelvic angle causing a hydronephrosis or a haematonephrosis. Finally it must be remembered that there may be no pain whatsoever. Tumor. In a large number of cases no mass can be palpated. This is particularly true when the growth is small or when it is spreading towards the upper pole of the kidney. The most common cause of a palpable tumor is probably an associated hydronephrosis or haematonephrosis due to some obstruction. When the latter is relieved as a result of the passage of a small piece of growth or blood clot the tumor mass very often disappears. Passage of tissue. Manypatientswithgrowthsof therenalpelvis particularly papillomata will volunteer the information that they are always passing small pieces of tissue covered with blood clot. These fragments of tumor resemble very closely those that are passed in cases of bladder tumor, and very often such a diagnosis is made. Loss of weight and strength. These are late symptoms and occur when metastases are present. Urinary symptoms. Frequency, urgency and dysuria occur only when the bladder becomes irritated by blood clots or pieces of tumor. DIAGNOSIS
There are no symptoms or signs that are pathognomonic of a tumor of the renal pelvis. In order to arrive at a possible diagnosis all available data are to be studied. The history, physical findings, urinary studies and finally cystoscopy with pyelography are all essential in the diagnosis. Cystoscopy and pyelography are undoubtedly our greatest aids. When the growth has not extended into the bladder the latter viscus appears perfectly normal. Very often, particularly during an attack of haematuria blood may be seen escaping from one or other ureteral orifice. This sign will immediately draw your attention to the diseased side. If the ureter is not obstructed by the growth, the catheter will pass easily to the renal pelvis and bloody urine will be collected. At times however the growth involves the ureter and as a result the catheter may be obstructed.
TUMORS OF THE RENAL PELVIS
411
Retrograde pyelography is to be performed in all cases. If a tumor of the renal pelvis is present there is a definite filling defect but that is not sufficient for a diagnosis. It must remembered that a tumor the renal parenchyma or a blood in the renal pelvis may give you identical pictures, In the differential diagnosis, renal tuberculosis, acute pyelonephritis, renal calculi and tumors of the renal parenchyma are to be excluded. PROGNOSIS
Although the simple papilloma of the renal pelvis is a benign growth yet clinically it is potentially malignant. It has the property of forming transplants in the ureter, bladder and even in the opposite ureter. It is for this reason that nephrectomy and complete ureterectomy with excision of the ureteral orifice is advocated. There are cases on record that were first subjected to a nephrectorny and later had a complete ureterectomy on account of recurrence. Thomson-Walker reports 2 such cases in 1929. The malignant papilloma has a better prognosis than the other malignant growths of the renal pelvis. Metastases occur late and are usually not widespread. The most malignant tumor of the renal pelvis is the squamous cell carcinoma. It metastasizes early and very extensively. The results are very poor even with early treatment. Kretschmer reports on 8 cases. Five died at operation and 3 very soon after. Scholl and Foulds operated on 5 cases: 1 was dead eight days after operation; 3 were dead during the first four months and only 1 was alive and apparently well six months after operation. The alveolar carcinoma is also a very malignant growth. It metastasizes early and may involve any organ or tissue in the body. TREATMENT
On account of tendency of tumors of the renal pelvis to extend down the ureter and even to the bladder, the treatment of choice today is nephrectomy and complete ureterectomy. In the
412
DAVID W. MACKENZIE AND MAX RATNER
past nephrectomy with or without partial ureterectomy was performed. This procedure was found to be too conservative, for several of the patients returned later with involvement of the ureter and even the bladder. Hunt and others are even more radical. They advise nephrectomy, complete uretectomy and segmental resection of that part of the bladder that includes the intramural portion of the ureter. Several authors advise the use of deep x-ray therapy or radium after removal of the growth. CONCLUSIONS
1. New growths of the renal pelvis are still of sufficient rarity
to be of extreme interest to the urologist. 2. The disease is very often wrongly diagnosed. 3. The diagnosis may be made from the history, symptoms, urinary findings, and finally, from cystoscopy and pyelography. 4. The treatment consists of nephrectomy and complete ureterectomy. 5. The prognosis even in simple papilloma is to be guarded. CASE REPORT
The following is the record of the patient with such a growth: J. A. T., male, aged seventy-one years, was admitted to the Urological Service at the Royal Victoria Hospital on December 30, 1931. Complaints. (1) Painless haematuria; (2) frequency of urination. History of present illness. Patient was perfectly well until October 15, 1929, when following a long motor trip he began to pass bright red blood in the urine. There were no other urinary symptoms such as dysuria or frequency of urination. The bleeding lasted off and on for about one month and then disappeared. There was no further haematuria until October, 1931. The blood was always well mixed with the urine, and there was in addition some frequency of urination: day 5- 6; night 1- 2. There were no other urinary symptoms except the passage of small pieces of tissue. There was never any pain in either loin. The patient's private physician examined one of the pieces of tissue and found cells which resembled very closely those that are found in papillary carcinoma of the bladder.
TUMORS OF THE RENAL PELVIS
413
Personal history. Usual childhood diseases. Typhoid fever in young adult life. No other serious diseases. Family history. Mother died of pulmonary tuberculosis. Physical examination. Patient was a well developed and nourished white male. Head: Normal. N eek: Bilaterally enlarged submaxillary glands. Nose and throat: Negative. Respiratory system: Negative. Circulatory system: There was a moderate degree of arteriosclerosis, and a hypertension ranging between 178 to 200 systolic, and 108 to 110 diastolic. The heart was moderately enlarged, and the aortic second sound was markedly accentuated. Electrocardiograph: Shows a left sided preponderance. Regular rate. Normal conduction time. Gastro-intestinal system: The liver was enlarged, the edge was felt two finger breaths below the costal margin. No other organs except the lower pole of the right kidney palpated. Nervous system: Negative. Skin and locomotor system: Negative. Genito-urinary system: Kidneys-no costo-lumbar tenderness on either side. The lower pole of the right kidney was palpated. No further organs or masses palpated on either side. Ureters: No tenderness or masses along the course of either ureter. Bladder: No suprapubic distension or tenderness. Penis: Normal. Urethra: No discharge. A soft ruber catheter no. 14F. passed easily to bladder. Residual urine 4 drams. Scrotum and contents: Normal except for slight varicosity of pampiniform plexus on both sides. Rectal examination: Prominent external haemorrhoids; sphincter tone good. Prostate is moderately enlarged, glandular, smooth, not fixed, not tender. Seminal vesicles not palpated. Laboratory findings. Urine: Smoky reddish, acid, 1018, albumin trace, sugar none. Red blood cells + +. White blood cells a few. Urates. Epithelial cells. One cubic centimeter phthalein intramuscularly: first hour, 35 per cent; second hour, 25 per cent; total, 60 per cent. Blood Wassermann negative. Cystoscopy. No. 24F. instrument easily introduced. There is slight intravesical enlargement of prostate. Bladder mucosa is normal. No THE JOURNAL OF UROLOGY, VOL. XXVIII, NO.
4
414
DAVID W. MAcKENZIE AND MAX RATNER
tumor or foreign body seen. Both ureteral orifices normal in contour and position. Cloudy reddish urine was seen coming from the right
FIG.
1.
PHOTOGRAPH OF THE PYELOGRAM IN THE CASE DISCUSSED
Note the absence of the upper calyces and the marked filling defect in the pelvis
ureteral orifice. Normal urine was seen coming from the left ureteral orifice. Both ureters were easily catheterized to the renal pelves and
TUMORS OF THE RENAL PELVIS
415
specimens obtained. Right: macroscopic, bloody; volume, 8 cc.; white blood cells urea, 0.0035; microscopic, red blood cells + occasionaL Left: Macroscopic, clear watery; volume, 20 cc.; urea, 0.0035; microscopic, red blood cells rare, epithelia.l cells. X-ray with catheters in position shows catheters in normai position. The right kidney shadow is large and indefinite. The left kidney 8hadow is outlined. No shadows suspicious of stone seen. Pyelogram, right, with 8 cc. 12 per cent sodium iodide, shows a definite filling defect of upper portion of pelvis (fig. 1\
+ ++,
li'IG. 2. PHOTOGRAPH OF THE SPECIMEN Note the papillary growth involving the upper prut of the pelvis and extending towards and into the cortex.
Pyelogram, left, with 4 cc. 12 per cent sodium iodide, shows a relatively normal renal pelvis. Diagnosis. Malignant growth of right kidney. In view of the history of passing pieces of tissue, the possibility of a papilloma of the renal pelvis was considered. Treatment. Nephrectomy and ureterectomy, right, on January 2, ]932.
416
DAVID W. MACKENZIE AND MAX RATNER
Pathological report. Right kidney: a kidney slightly larger than normal, split open showing a papillary growth which involves the upper part of the pelvis. This growth appears well demarcated but has compressed the medulla and cortex in this area with the formation of a filbert sized cyst. The remaining portion of the kidney shows considerable peripelvic fat, and definite markings and coarse irregular lobation under the capsule (fig. 2).
Fm. 3.
PHOTOMICROGRAPH
(Low
POWER) SHOWING A DEFlNI'l'E PAPILLOMATOUS GROWTH
Note evidence of degeneration of some of the papillary outgrowths
Microscopic examination. Sections show a papillomatous growth of stratified transitional cells similar to that met with in the bladder and apparently taking origin in the pelvis of the kidney. It has invaded the parenchyma which is compressed and while fairly well demarcated shows evidence of malignant infiltration. Section taken through small pin-
TUMORS OF THE RENAL PELVIS
417
head greyish nodule in cortex shows it to consist of growth similar to that described above. There is also considerable metastatic involvement in the cortex in this area (figs. 3 and 4). Anatomical Summary. Transitional celled papilloma of the pelvis which has become cancerous.
FIG. 4. SIMILAR TO FIGURE 3 (HIGH POWER) There is definite involvement of the parenchyma with stratified transitional cells.
Results. Patient had an uneventful convalescence and was discharged as cured on February I, 1932. May 4, 1932. Reported well.
305 Medical Arts Building, Montreal, Canada
418
DAVID W. MAcKENZIE AND MAX RATNER
REFERENCES ALBARRAN, J.: Neoplasmes primitifs du bassinet et de l'uretere. Ann. d. Mal. d. Org. Genito Urin., Paris, 1900, xviii, 701. ALBARRAN, J., AND IMBERT, L.: Les Tumeurs du Rein. Paris, 1903, Masson et Cie, p. 769. BoWEN, J. A., AND BENNETT, G. A.: Squamous cell carcinoma of the renal pelvis. Jour. Urol., 1930, xxiv, 495. BoRoss, E.: Fibroma of the renal pelvis. Orvosi hetil, October, 1929, lxxiii, 1055. EwrnG, JAMES: Neoplastic Diseases. Third edition, 1928. HUNT, V. C.: Papillary epithelioma of renal pelvis. Jour. Urol., September, 1927, xviii, 225. KANANKA, V.: Malignant papilloma of renal pelvis. Medicina Kaunas, February, 1928, ix, 120. KRETSCHMER, H. L.: Primary non-papillary carcinoma of the renal pelvis. Jour. Urol., 1917, i, 405. MELTZER, M.: Papillary carcinoma of the renal pelvis. Jour. Urol., 1926, xvi, 335. PLANT, A.: Diffuse adenoma of renal pelvis. Ztschr. f. Urol. Chir., 1929, xxvi, 562. RoBERTS, 0. W.: Malignant papilloma of renal pelvis in double ureter and pelvis. Lancet, July, 1928, ii, 67. ScHOLL, A. J., AND FouLDS, G. S.: Squamous-cell tumors of the renal pelvis. Ann. Surg., 1924, lxxx, 594. THOMAS, G. J., AND REGNIER, E. A.: Tumors of the kidney pelvis and ureter. Jour. Urol., 1924, xi, 205. THOMSON-WALKER, Srn J.: Three cases of ureterectomy for papilloma, with comments. Brit. Jour. Urol., June, 1929, i, 141.