HYPERNEPHROMAS THAT ARE TOO EARLY TO DIAGNOSE 1 LAWRENCE R. WHARTON From the Department of Gynecology of the Johns Hopkins Hospital and Medical School
How shall we handle those cases in which we strongly suspect malignant disease of the kidney, but in which the clinical evidence is not conclusive? Shall we temporize and await developments, trusting that the condition is not malignant? Shall we irradiate prophylactically? Or shall we explore the suspicious kidney? This problem is largely of our own manufacture, because for years we have been urging people to have periodic health examinations and to report to their family doctors if there is irregular bleeding from any source, the mouth, rectum, vagina or urinary tract. Intelligent patients and intelligent family doctors are heeding this advice, with the result that we are beginning to see an increasing number of patients in whom irregular bleeding has been present for only a very few days or weeks. This greatly increases our responsibility. The various organs of the body differ in their accessibility and in the ease with which they can be studied. Thus, in the mouth, vagina, cervix, uterus, rectum, urethra and bladder, one can either see, feel or examine by biopsy every square millimeter of the surface and eliminate malignancy almost with certainty. Furthermore, many of these determinations are simple office procedures and entail very little inconvenience. The kidney, however, is inaccessible, and hence is not subjected to microscopic pathological study. Hence, by necessity, the diagnosis of a renal tumor is usually deferred until it produces gross pathological disturbances and unmistakable symptoms. If we wait until the gross changes are unmistakable, we shall make no mistakes in diagnosis, perform absolutely no unnecessary operations and cure practically no patients. Our diagnostic accuracy and ultimate mortality will be close to 100 per cent. The reality of this problem is clear. It would be inconceivably unscientific and unjustified to explore a,11 cases of renal hematuria; it is even more unfortunate to ·withhold indicated treatment if the patient has an early tumor. How can we separate these cases? What is the answer to this problem? 1 Read before annual meeting, American Urological Association, White Sulphur Springs, W. Va., May 30, 1939. 713
714
LAWRENCE R. WHARTON
I know no sure solution. We shall make mistakes whichever course we follow, because we are dealing with malignancy in an organ which is hard to examine. In spite of our known limitations, however, it seems to me that there is room for improvement in the diagnosis of early hypernephroma and it is to this problem that I call your attention. My own observations and experience have led me to adopt the following principles in trying to solve this problem. These principles are not infallible, for I shall show you some mistakes. But, in my experience, practically all of the survivals in my own hypernephroma cases, are found in the group that I have treated in accordance with these principles. In the first place, the diagnosis of an early renal tumor must often be based on the summation of all the clinical data, the clinical situation as a whole, rather than on any single finding. Even in advanced cases, one or more of the characteristic features may be missing; in early cases, the diagnostic data are usually even more uncertain, and there may be no single unmistakable finding which clinches the diagnosis. Indeed, in some instances, the very absence of any infallible sign may lead us to overlook other evidence that is highly suggestive and makes the diagnosis quite probable. And this leads me to the second conclusion that I have reached, namely, that when the evidence points to the probable diagnosis of renal tumor, even though it is not conclusive, the proper treatment is immediate exploration of the suspected kidney. This decision implies the exercise of sound judgment and accurate examination, in order that one may avoid unnecessary operations. The third conclusion is that preoperative irradiation is not advisable in those cases in which the diagnosis is uncertain, and in which the operation is exploratory. In these cases, one wishes to see things as they are. The suspected tumor is probably small. If it is liquefied or partly destroyed by irradiation, the tumor may be extremely difficult to recognize. The surrounding tissue changes are also confusing. This dilemma arose in one of my cases, and led to an incorrect diagnosis when the kidney was explored. The fourth point is that a renal exploratory is best conducted by the lumbar route. Although I strongly advise the transperitoneal operation when a tumor is clearly present and when the operation is feasible, my own experience warrants the conclusion that a small tumor may be removed just as safely by the lumbar route and that the ultimate results are just as good as when the more extensive transperitoneal operation
HYPERNEPHROMAS TOO EARLY TO DIAGNOSE
715
is used. Also the lumbar route allows one greater freedom of surgical action. If for example, a stone is found or a benign condition which requires postoperative drainage, it can be easily provided without danger of peritonitis. Hence, in these early cases in which the preoperative diagnosis is uncertain, I feel that a lumbar exploratory is the advisable procedure. Finally, fancy diagnoses are to be shunned. If, for example, the roentgenologist wishes to gamble on the diagnosis of a benign cyst, his written report should state clearly that hypernephroma is much more common, that benign cyst and malignant tumor may give the same roentgen picture and that renal exploration is indicated. The experienced urologist knows this, but not all surgeons who do renal surgery are experienced urologists and they lean heavily on the roentgenologist's report and judgment. The general use of intravenous urography by medical diagnosticians and others who are not trained in urology makes this problem even more serious. I shall illustrate these principles by 4 cases.
Case 1. Mrs. R. M., first seen February 1932, was a healthy woman 48 years old who had had hematuria for only 4 days. The bleeding was rather profuse, and accompanied by a dull ache in the left flank and back. The family physician, Dr. Whitehouse, referred her to me. Physical examination revealed only one suggestive sign: the lower pole of the left kidney was palpable, in spite of the fact that the patient was obese and the right kidney was not felt. The hematuria was traced to the left kidney. Urographic study revealed no deformity of either kidney. Retrograde pyelograms were taken in the Women's Hospital and the Sinai Hospital, 2 weeks apart. As you can see (fig. 1), there is no filling defect, and both roentgenographic reports state there is no sign of a renal tumor. In the face of this statement, repeated by two sets of capable observers, what course should I take? It would not look well to operate on a patient in the face of two x-ray reports, both denying any evidence of a renal tumor. Nevertheless, because of the hematuria from the left kidney, and because the left kidney was unexplainably palpable, the situation seemed too suspicious to warrant procrastination. Hence, within 10 days after the second confirmatory cystoscopic study, I explored the left kidney by a lumbar incision. In the upper pole, there was a hypernephroma as large as an orange, pushing the kidney down. The nephrectomy was done over 7 years ago, and the patient is still well. Case 2. In January 1932, a woman 52 years old consulted me. She had painless hematuria which had been present only 3 days. Her family physician,
716
LAWRENCE R. WHARTON
Dr. Akehurst, had examined the urine on former occasions and found nothing abnormal. There had been no other urogenital symptoms. The family doctor promptly demanded an explanation of the hematuria. On physical examination, the only positive finding was that the lower pole of the right kidney was palpable. No mass was evident. Since the lower pole of the right kidney is palpable in a large percent of women, this finding had no significance. Cystoscopy showed that the hematuria was coming from the right kidney and the
FIG. 1
FIG. 2
FIG. 1. Case 1. Normal pyelogram in a patient who had had hematuria for only 4 days. Only positive finding was a palpable left kidney which was source of hematuria. Exploratory laparotomy revealed a hypemephroma 7 cm. in diameter in upper pole. Operation: March 1932; patient is well. FIG. 2. Case 2. A characteristic deformity of right kidney in a woman who had had hematuria only 3 days. The roentgenologist's diagnosis was renal cyst. At nephrectomy a hypemepliroma 2 x 3 cm. was removed. Operation: January 1932; patient is well.
pyelogram revealed a definite filling defect in the median calices of the right kidney,-unmistakable evidence of a tumor (fig. 2). Although the diagnosis made by the roentgenologists was a cyst of the right kidney, I determined to explore this kidney immediately. Operation revealed a small hypernephroma, measuring 3 x 5 cm. The nephrectomy was easy, done by the lumbar route. The patient is still well, over 7 years later.
This case is shown because of the early lesion, the short period of bleeding and to commend the intelligent and prompt action of both the family
HYPERNEPHROMAS TOO EARLY TO DIAGNOSE
717
doctor and the patient. The situation seemed clear to me, although one might have been confused and led to procrastination by the report of the roentgenologists, who diagnosed a cyst of the kidney. The danger of making such a disgnosis and the possible harmful influence it may have is shown by the next case.
Case 3. Mrs. B. was aged 53 years. 1 never saw this patient, but am permitted to report the case by the courtesy of the resident surgeon of the Women's Hospital, Dr. Sellers. The patient was admitted to the public ward in February 1939, complaining of pain in the right upper abdomen. This had been present for about 2 years. A cholecystectomy done in 1937 had not affected it. When the gall bladder was removed in 1937, the surgeon palpated the right kidney and found it normal. There was an indefinite history of some bleeding in December 1938, but the patient did not know whether it came from the rectum, vagina or bladder. Urological and cystoscopic studies made in March 1939 were negative, except for the urogram of the right kidney and ureter (fig. 3), which shows a slight deformity of the upper calyx. The roentgenologists made a diagnosis of cyst of the right kidney. In view of this diagnosis, therefore, and because of the utter absence of any related symptoms or hematuria, the consultant advised that the woman be sent home for observation. This policy, however, did not suit the family doctor, who asked that she be readmitted and operated upon. This was done, and on March 25, 1939, the resident explored and removed the right kidney, finding an early hypernephroma, 2 cm. in diameter, in the upper pole. These last 2 cases demonstrate that the roentgenologic diagnosis of a renal cyst in a questionable case may confuse the situation and lead to unfortunate delay in treatment.
Case 4. This case presented our most difficult problem and taught me more than all of the others. A woman 48 years old, had had painless and profuse hematuria for 2 weeks. Her family physician, Dr. Howard Warner, recommended urologic study and referred her to me. She was rather heavy and muscular, and general examination was entirely negative. The hematuria was traced to the left kidney. The only other suggestive finding was shown in the left urogram. There is a suggestive deformity in the next to the highest calyx. This calyx is bent downward at a right angle, increasing the space between it and the highest calyx. All the other calices are symmetrical. Also, when some of the sodium bromide solution is allowed to drain out, this asymmetrical calyx empties first, as though it were under pressure. There was no evidence of stone, infection, obstruction or any other urological disorder (fig. 4).
718
LAWRENCE R. WHARTON
Because of the importance of this problem, I submitted these films and the clinical data to several urologists; only one agreed with me that the patient probably had a renal tumor, but all stated that, if that were their kidney, they would want it removed. The entire situation was explained to both the patient and her husband. The patient was given a full course of roentgen therapy, during which the bleeding ceased, but later returned. Ten days later, I explored the kidney transperitoneally.
FIG.3
FIG.4
FIG. 3. Case 3. The patient complained of epigastric pain. She had no urological symptoms and no hematuria. Pyelogram showed this early deformity of highest calyx, again diagnosed benign cyst. The family doctor insisted that an exploratory operation be carried out. A small hypernephroma 2 cm. in diameter was found in upper pole. Operation: March 1939. This case illustrates danger of presuming that one can differentiate a cyst from a hypernephroma. FIG. 4. Case 4. Marked, painless hematuria from left kidney. Note slight deformity of second calyx. It is bent downward, and space between it and highest calyx is increased. Operation disclosed no tumor, two stones that cast no shadow in the x-ray, and multiple renal hemorrhages. Operation: March 1939.
Because of the heavy abdominal muscles and the large amount of intraperitoneal fat, the operation was difficult. A lumbar exploration might have been equally arduous. The kidney itself was completely normal on inspection except for marked hyperemia; on palpation, in the suspected area one could feel an area of softening. Fearing that this might be a liquefied or softened tumor, and knowing that biopsy would be fatal, if it were a tumor, I removed the kidney. There was no tumor. The softened area was a collection of fat,
HYPERNEPHROMAS TOO EARLY TO DIAGNOSE
719
and the bleeding was due either to multiple and diffuse renal hemorrhages or to two small calculi which had not cast a shadow in the x-ray film. The convalescence was normal and the patient is well. She understands the situation completely, and is delighted that she did not have a malignant tumor, although we all regretted that a mistake in diagnosis had been made.
It was from this case that I learned that preoperative irradiation may add a confusing factor in some cases, and that a lumbar exploratory may give one greater liberty in performing a conservative operation. SUMMARY
Diagnosis of early renal malignancy may be difficult. We know what happens to those patients in whom we defer operation until the diagnosis is certain. In my own experience, practically all of my 5 year survivals are found in the group in which I operated, in spite of the fact that the diagnosis was still questionable. All but one had a malignant tumor; in one case, I explored and removed a kidney, finding only stones and renal hemorrhage. On the basis of my own experience, I have adopted the following general principles in these cases:The diagnosis of early renal tumor must occasionally be based on the general clinical picture, or on suggestive evidence, in spite of the absence of any single infallible sign. When there is presumptive evidence of renal tumor, the proper treatment is exploration. Clinical data must be accurate and interpreted with judgement, in order that mistakes may be avoided. It is, however, a more serious error to postpone an operation till the situation is clear, than to perform an exploratory and find no hypernephroma. The decision requires fine judgement, and the surgeon who saves lives will occasionally explore and find no tumor. Generally, however, there is some pathological condition to explain the bleeding; it is rarely idiopathic. It is usually impossible to differentiate a benign cyst from a hypernephroma and it is usually unsafe to presume that this differentiation can be made. Preoperative irradiation may also confuse the picture in a questionable case, and should be reserved for proved tumors. The lumbar route is preferable to the transperitoneal, for purposes of exploring the kidney. The transperitoneal operation is indicated for cases in which the presence of a tumor is established and in which the situation lends itself to that procedure.
1201 N. Calvert St., Baltimore, Md.