- Email: [email protected]
Bilateral Carcinoma in Polycystic Renal Disease—An Unique Case
THE .JOURNAL OF UROLOGY
Vol. 71, No. 6, June 1954 Printed in U.S.A.
BILATERAL CARCINOMA IN POLYCYSTIC RENAL DISEASE-AN UNIQUE CASE CAPT. ANTHONY A. BORSKI (MC, USA) AND COL. JAMES C. KIMBROUGH (MC, USA) From the Urology Service, Walter Reed Army Hospital, Washington, D. C.
A review of the literature fails to reveal any reported cases of bilateral renal carcinoma in polycystic renal disease. The occurrence of unilateral renal carcinoma in polycystic disease, however was reviewed in 1940 by Melicow and Gile 1 and in 1952 by ,Johnson2 and nine such cases were found. The following is believed to be the only known case of bilateral renal carcinoma in polycystic renal disease to be reported to date: A 38-year-old white man was admitted to vValter Reed Army Hospital on March 17, 1948 with intermittent headaches of 2 years' duration and a painless mass in the right flank. In 1946 a routine physical examination revealed hypertension and a mass in the right flank, but there were no investigative studies made at that time. The headaches had been increasing in intensity and ·were usually worse in the morning and subsided somewhat during the day. There was no eye, ear, nose or throat disease to account for these headaches and the mass in the right flank caused no symptoms. The patient was admitted to the Urology Service for study of the right flank mass. Family history was pertinent in that one sister had a thyroid tumor and another sister and one nephew were known to have brain tumors. There was no family history of polycystic renal disease. Physical examination showed the patient to be well developed, well nourished and in no distress. His weight was 174 pounds and his blood pressure 140/90. The entire physical examination was within normal limits except that both kidneys were enlarged to palpation and the right kidney was larger and more irregular than the left. The impression was that the patient had polycystic disease of the kidneys. Laboratory studies showed the following: Blood urea nitrogen, 15.8; urea clearance, 89 per cent; urine concentration, 1.022; CO2 volume per cent, 56.2 (normal 53~72); total protein, 6.8 per cent; AG ratio, 3.2. Chest x-ray was normal. Excretory urogram showed enlarged kidneys with some distortion of calyces, especially the right inferior calyx, but the typical spidering effect of polycystic kidneys was not present. On March 30, the right kidney was explored. It was noted that the lower pole was a solid mass of tissue. There were many cysts present throughout the kidney and there was a multicystic surface. Several large cysts were unroofed and others were aspirated. A frozen section of tissue was reported as being doubtful. At this stage of surgery, the patient was doing poorly, and in view of the fact that no positive diagnosis was made other than that of polycystic kidney the operation was completed. Permanent sections of biopsy material showed adenocarcinoma; therefore, a right nephrectomy was performed on April 5 (figs. 1 and 1
2
Melicow, M. M. and Gile, Harold: J. Urol., 43: 767-773, 1940. Johnson, W. F.: J. Urol., 60: 10-12, 1953. 677
678
ANTH:ONY A. BORSKI AND JAMES C. KIMBROUGH
Fm. 1. Right kidney showing multiple cysts and multinodular tumor masses.
2, A). Radiation therapy to the right flank was started. On April 20, the patient noticed an unsteady gait and severe headaches but without visual disturbances. An electro-encephalogram was reported as being normal. Eye, ear, nose and throat consultation was negative. During the next 3 months there were no marked changes. In August vomiting of a nonprojectile type developed and without nausea. The electro-encephalogram was repeated and revealed left side amplitude asymmetry which was considered evidence of metastatic carcinoma. A neurosurgical consultant advised against exploration and, therefore, radiation to the brain was begun on September 10. The symptoms abated, but in N ovember, headaches and vomiting recurred and radiation was once again started. Blood pressure at this time was 220/120. Spinal tap on December 14 revealed pressure 260 mm. water and protein, 154 mg./100 cc. On December 21 his blood pressure was 140/100. He received a total of 4800 roentgens through three different portals to the brain. In January 1949, he was asymptomatic and all studies were within normal limits except for the large left kidney which showed distortion of calyces. Typical spidering was not noted. The patient was discharged to outpatient care. Second admission: The patient was again admitted to Walter Reed Army Hospital on June 19, 1951, for surgical treatment of the mass in the left flank. In the course of follow-up care, the left kidney was found to have at least one large cyst and several smaller ones. The patient was asymptomatic except for occasional right flank pain. Physical examination showed the blood pressure to be 172/110. There was a 15 by 15 by 15 cm. mass in the left flank which was irregular, nontender, and which moved with respirations. The remainder of the physical examination was essentially negative. Laboratory studies showed both the admission urinalysis and complete blood count to be normal. The phenolsulfonphthalein test revealed a 49 per cent excretion in 60 minutes. On June 26, a urinalysis showed 3 plus albumin; blood urea nitrogen, 26. Skeletal and chest x-rays were negative. An excretory urogram re-
BILATERAL CARCINOMA IN POLYCYSTIC KIDNEY
679
Fm. 2. A, low power section of right kidney showing adenocarcinoma. B, low power section of left kidney showing adenocarcinoma.
vealed an absence of the right renal shadow and fractional visualization of the left kidney. On June 21, 1951, the left kidney was explored and many cysts were punctured. There were some firm areas which were suggestive of tumor and a biopsy specimen was taken. The postoperative course was uneventful. The pathological diagnosis was that of clear cell carcinoma of the kidney (fig. 2, B). The patient was started on radiation therapy to the left kidney. The amount of radiation received was 5688 roentgens through three portals to the left kidney. Third admission: On December 8, 1952, the patient was again admitted to the hospital with intermittent chills and fever of 3 days' duration. He had also had severe intermittent left lumbar pains for several months. Three days prior to admission he had a fever of 103F with profuse night s,veats. There were no urinary symptoms.
680
ANTHONY A. BORSKI AND JAMES C. KIMBROUGH
A physical examination revealed the patient to be well developed, but with an appearance of being older than his 43 years. Blood pressure was 160/95; temperature, 102F. He had slight abdominal tenderness and there was a large mass in the left flank which was slightly tender. Laboratory studies showed the admission urinalysis to have a 1 plus albumin and a specific gravity of 1.016. The white blood count was 5,500. Urine culture revealed Escherichia coli and Streptococcus fecalis. The blood urea nitrogen was normal. Skeletal survey and chest x-rays were also normal. An excretory urogram failed to reveal any visualization of dye. Phenolsulfonphthalein test showed 28 per cent excretion in 30 minutes. The patient was treated with streptomycin and penicillin and in 48 hours he was asymptomatic. Because of polycystic disease and carcinoma in the remaining kidney, no further diagnostic tests were performed. He was discharged on December 17, 1952, to be followed in the Outpatient Service. DISCUSSION
The congenital nature of polycystic disease of the kidneys is undisputed. Pathogenesis is unknown and many theories have been advanced as to the etiology, but the most widely accepted theory is that of Hildebrandt. 3 The disease results as a failure of union between metanephroblastemic elements and the collecting tubules originating in its metanephric duct. Cystic formation results from blockage of glomerular secretion. Nodular cystic enlargement of the kidneys is predominately bilateral. In cases thought to be unilateral, histologic study will almost always disclose this anomalous development in the opposite kidney. When the renal cysts enlarge, they cause compression atrophy of the renal parenchyma and consequently renal failure ensues. 4 There is no known causal relationship between polycystic disease and carcinoma of the kidney. Simultaneous occurrence of these conditions is considered to be a coincidence. The prognosis of polycystic disease is especially poor when the disease is recognized in childhood and is also poor in adults, although some patients live to an old age. When one kidney is removed, survival is not likely for more than a year or two, although survival for longer periods has been reported. Treatment of uncomplicated cases is fundamentally the same as for nephritis. Hematuria may demand nephrectomy if conservative measures fail to stop the bleeding. Rovsing 5 has relieved pain in some cases and also has improved renal function by cyst puncture. Goldstein 6 has used cyst puncture and marsupialization of the kidneys to the wound. In this manner the cysts could continue to be aspirated postoperatively. Young employed aspiration and injections of 5 per cent sodium morrhuate solution and Fish7 injected 50 per cent dextose solution. 3 Campbell, Meredith: Clinical Pediatric Urology: Philadelphia: W. B. Saunders Co., 1951. 4 Bobbitt, R. M.: J. Urol., 50: 131-140, 1943. 5 Rovsing, T.: Am. J. Urol. 8: 120-124, 1912. 6 Goldstein, A. E.: J. Urol., 34: 536-547, 1935. 7 Cahill, G. F. and Fish, G. W.: Trans. Am. Assoc. Gfmito-Urin. Surg., 38: 81, 1941.
BILATERAL CARCINOMA IN POLYCYSTIC KIDNEY
681
Acute symptoms may be relieved and adequate drainage maintained by occasional careful dilatation of the ureters and renal pelvic lavage. Death is almost always due to uremia. SUMMARY
A case of bilateral carcinoma in polycystic kidneys is presented. Despite the extremely poor prognosis, the patient has survived 5 years and is asymptomatic at the present time. A review of the literature fails to disclose any similar case. ADDENDUM
The patient was re-admitted on December 15, 1953 complaining of nausea and vomiting, constant occipital headache, and difficulty in maintaining his equilibrium. A physical examination revealed abnormal bilateral deep tendon reflexes, more pronounced on the left side. His blood pressure was 240/140 and there was a firm, nontender, fixed 20 cm. mass in the left upper quadrant. The electroencephalogram revealed an abnormal record ,vith a slow wave focus in the left parieto-occipital area. There was nothing unusual in the laboratory data except that the blood urea nitrogen determination was 27 mg. per cent. The patient was given 1,942 roentgens to the occipital lobes, but the headaches persisted throughout the course of therapy. On February 7, he had a severe occipital headache and died shortly thereafter. Several tumor areas in the polycystic left kidney were found at autopsy and an examination of the brain showed a 2 cm. tumor nodule in the cerebellum which had ruptured into the fourth ventricle. There was also a cyst in the cerebellum which was filled with old blood and necrosis. This cystic region, the pathologist felt, probably represented the cerebral metastases which had been destroyed several years earlier by previous radiation therapy. Pathological diagnosis: Cerebral metastases, secondary to adenocarcinoma of the polycystic kidney.
Vol. 71, No. 6, June 1954 Printed in U.S.A.
BILATERAL CARCINOMA IN POLYCYSTIC RENAL DISEASE-AN UNIQUE CASE CAPT. ANTHONY A. BORSKI (MC, USA) AND COL. JAMES C. KIMBROUGH (MC, USA) From the Urology Service, Walter Reed Army Hospital, Washington, D. C.
A review of the literature fails to reveal any reported cases of bilateral renal carcinoma in polycystic renal disease. The occurrence of unilateral renal carcinoma in polycystic disease, however was reviewed in 1940 by Melicow and Gile 1 and in 1952 by ,Johnson2 and nine such cases were found. The following is believed to be the only known case of bilateral renal carcinoma in polycystic renal disease to be reported to date: A 38-year-old white man was admitted to vValter Reed Army Hospital on March 17, 1948 with intermittent headaches of 2 years' duration and a painless mass in the right flank. In 1946 a routine physical examination revealed hypertension and a mass in the right flank, but there were no investigative studies made at that time. The headaches had been increasing in intensity and ·were usually worse in the morning and subsided somewhat during the day. There was no eye, ear, nose or throat disease to account for these headaches and the mass in the right flank caused no symptoms. The patient was admitted to the Urology Service for study of the right flank mass. Family history was pertinent in that one sister had a thyroid tumor and another sister and one nephew were known to have brain tumors. There was no family history of polycystic renal disease. Physical examination showed the patient to be well developed, well nourished and in no distress. His weight was 174 pounds and his blood pressure 140/90. The entire physical examination was within normal limits except that both kidneys were enlarged to palpation and the right kidney was larger and more irregular than the left. The impression was that the patient had polycystic disease of the kidneys. Laboratory studies showed the following: Blood urea nitrogen, 15.8; urea clearance, 89 per cent; urine concentration, 1.022; CO2 volume per cent, 56.2 (normal 53~72); total protein, 6.8 per cent; AG ratio, 3.2. Chest x-ray was normal. Excretory urogram showed enlarged kidneys with some distortion of calyces, especially the right inferior calyx, but the typical spidering effect of polycystic kidneys was not present. On March 30, the right kidney was explored. It was noted that the lower pole was a solid mass of tissue. There were many cysts present throughout the kidney and there was a multicystic surface. Several large cysts were unroofed and others were aspirated. A frozen section of tissue was reported as being doubtful. At this stage of surgery, the patient was doing poorly, and in view of the fact that no positive diagnosis was made other than that of polycystic kidney the operation was completed. Permanent sections of biopsy material showed adenocarcinoma; therefore, a right nephrectomy was performed on April 5 (figs. 1 and 1
2
Melicow, M. M. and Gile, Harold: J. Urol., 43: 767-773, 1940. Johnson, W. F.: J. Urol., 60: 10-12, 1953. 677
678
ANTH:ONY A. BORSKI AND JAMES C. KIMBROUGH
Fm. 1. Right kidney showing multiple cysts and multinodular tumor masses.
2, A). Radiation therapy to the right flank was started. On April 20, the patient noticed an unsteady gait and severe headaches but without visual disturbances. An electro-encephalogram was reported as being normal. Eye, ear, nose and throat consultation was negative. During the next 3 months there were no marked changes. In August vomiting of a nonprojectile type developed and without nausea. The electro-encephalogram was repeated and revealed left side amplitude asymmetry which was considered evidence of metastatic carcinoma. A neurosurgical consultant advised against exploration and, therefore, radiation to the brain was begun on September 10. The symptoms abated, but in N ovember, headaches and vomiting recurred and radiation was once again started. Blood pressure at this time was 220/120. Spinal tap on December 14 revealed pressure 260 mm. water and protein, 154 mg./100 cc. On December 21 his blood pressure was 140/100. He received a total of 4800 roentgens through three different portals to the brain. In January 1949, he was asymptomatic and all studies were within normal limits except for the large left kidney which showed distortion of calyces. Typical spidering was not noted. The patient was discharged to outpatient care. Second admission: The patient was again admitted to Walter Reed Army Hospital on June 19, 1951, for surgical treatment of the mass in the left flank. In the course of follow-up care, the left kidney was found to have at least one large cyst and several smaller ones. The patient was asymptomatic except for occasional right flank pain. Physical examination showed the blood pressure to be 172/110. There was a 15 by 15 by 15 cm. mass in the left flank which was irregular, nontender, and which moved with respirations. The remainder of the physical examination was essentially negative. Laboratory studies showed both the admission urinalysis and complete blood count to be normal. The phenolsulfonphthalein test revealed a 49 per cent excretion in 60 minutes. On June 26, a urinalysis showed 3 plus albumin; blood urea nitrogen, 26. Skeletal and chest x-rays were negative. An excretory urogram re-
BILATERAL CARCINOMA IN POLYCYSTIC KIDNEY
679
Fm. 2. A, low power section of right kidney showing adenocarcinoma. B, low power section of left kidney showing adenocarcinoma.
vealed an absence of the right renal shadow and fractional visualization of the left kidney. On June 21, 1951, the left kidney was explored and many cysts were punctured. There were some firm areas which were suggestive of tumor and a biopsy specimen was taken. The postoperative course was uneventful. The pathological diagnosis was that of clear cell carcinoma of the kidney (fig. 2, B). The patient was started on radiation therapy to the left kidney. The amount of radiation received was 5688 roentgens through three portals to the left kidney. Third admission: On December 8, 1952, the patient was again admitted to the hospital with intermittent chills and fever of 3 days' duration. He had also had severe intermittent left lumbar pains for several months. Three days prior to admission he had a fever of 103F with profuse night s,veats. There were no urinary symptoms.
680
ANTHONY A. BORSKI AND JAMES C. KIMBROUGH
A physical examination revealed the patient to be well developed, but with an appearance of being older than his 43 years. Blood pressure was 160/95; temperature, 102F. He had slight abdominal tenderness and there was a large mass in the left flank which was slightly tender. Laboratory studies showed the admission urinalysis to have a 1 plus albumin and a specific gravity of 1.016. The white blood count was 5,500. Urine culture revealed Escherichia coli and Streptococcus fecalis. The blood urea nitrogen was normal. Skeletal survey and chest x-rays were also normal. An excretory urogram failed to reveal any visualization of dye. Phenolsulfonphthalein test showed 28 per cent excretion in 30 minutes. The patient was treated with streptomycin and penicillin and in 48 hours he was asymptomatic. Because of polycystic disease and carcinoma in the remaining kidney, no further diagnostic tests were performed. He was discharged on December 17, 1952, to be followed in the Outpatient Service. DISCUSSION
The congenital nature of polycystic disease of the kidneys is undisputed. Pathogenesis is unknown and many theories have been advanced as to the etiology, but the most widely accepted theory is that of Hildebrandt. 3 The disease results as a failure of union between metanephroblastemic elements and the collecting tubules originating in its metanephric duct. Cystic formation results from blockage of glomerular secretion. Nodular cystic enlargement of the kidneys is predominately bilateral. In cases thought to be unilateral, histologic study will almost always disclose this anomalous development in the opposite kidney. When the renal cysts enlarge, they cause compression atrophy of the renal parenchyma and consequently renal failure ensues. 4 There is no known causal relationship between polycystic disease and carcinoma of the kidney. Simultaneous occurrence of these conditions is considered to be a coincidence. The prognosis of polycystic disease is especially poor when the disease is recognized in childhood and is also poor in adults, although some patients live to an old age. When one kidney is removed, survival is not likely for more than a year or two, although survival for longer periods has been reported. Treatment of uncomplicated cases is fundamentally the same as for nephritis. Hematuria may demand nephrectomy if conservative measures fail to stop the bleeding. Rovsing 5 has relieved pain in some cases and also has improved renal function by cyst puncture. Goldstein 6 has used cyst puncture and marsupialization of the kidneys to the wound. In this manner the cysts could continue to be aspirated postoperatively. Young employed aspiration and injections of 5 per cent sodium morrhuate solution and Fish7 injected 50 per cent dextose solution. 3 Campbell, Meredith: Clinical Pediatric Urology: Philadelphia: W. B. Saunders Co., 1951. 4 Bobbitt, R. M.: J. Urol., 50: 131-140, 1943. 5 Rovsing, T.: Am. J. Urol. 8: 120-124, 1912. 6 Goldstein, A. E.: J. Urol., 34: 536-547, 1935. 7 Cahill, G. F. and Fish, G. W.: Trans. Am. Assoc. Gfmito-Urin. Surg., 38: 81, 1941.
BILATERAL CARCINOMA IN POLYCYSTIC KIDNEY
681
Acute symptoms may be relieved and adequate drainage maintained by occasional careful dilatation of the ureters and renal pelvic lavage. Death is almost always due to uremia. SUMMARY
A case of bilateral carcinoma in polycystic kidneys is presented. Despite the extremely poor prognosis, the patient has survived 5 years and is asymptomatic at the present time. A review of the literature fails to disclose any similar case. ADDENDUM
The patient was re-admitted on December 15, 1953 complaining of nausea and vomiting, constant occipital headache, and difficulty in maintaining his equilibrium. A physical examination revealed abnormal bilateral deep tendon reflexes, more pronounced on the left side. His blood pressure was 240/140 and there was a firm, nontender, fixed 20 cm. mass in the left upper quadrant. The electroencephalogram revealed an abnormal record ,vith a slow wave focus in the left parieto-occipital area. There was nothing unusual in the laboratory data except that the blood urea nitrogen determination was 27 mg. per cent. The patient was given 1,942 roentgens to the occipital lobes, but the headaches persisted throughout the course of therapy. On February 7, he had a severe occipital headache and died shortly thereafter. Several tumor areas in the polycystic left kidney were found at autopsy and an examination of the brain showed a 2 cm. tumor nodule in the cerebellum which had ruptured into the fourth ventricle. There was also a cyst in the cerebellum which was filled with old blood and necrosis. This cystic region, the pathologist felt, probably represented the cerebral metastases which had been destroyed several years earlier by previous radiation therapy. Pathological diagnosis: Cerebral metastases, secondary to adenocarcinoma of the polycystic kidney.