ADENOCARCINOMA
OF KIDNEY
IN A CHILD
Tumor Rupture Following Trauma ROBERT
M. STRIMER,
JOHN R. RICHARDSON,
M.D. JR., M.D.
From the Urology Section, Dartmouth-Hitchcock Medical Center, Hanover, New Hampshire
ABSTRACT - A ten-year-old white girl with Stage I clear cell adenocarcinoma of the kidney was seen after local trauma had produced a renal laceration with rupture of the tumor. She was treated with nephrectomy followed by a second exploration several months later. No adjunctive therapy was used.
Adenocarcinoma of the kidney is rare in children. It represents less than 6 per cent of all childhood renal malignancy and less than 0.5 per cent of all renal adenocarcinoma in children.1~2 Most of these tumors present with hematuria and a palpable abdominal or flank mass, but up to 13 per cent of cases are discovered following trauma. 1 Our patient had a traumatically ruptured renal tumor. Case Report This ten-year-old white girl was in excellent health until January 24,1974, when she fell from a chair in school, striking her right flank. Immediate and persistent flank and abdominal pain prompted medical attention, and urinalysis revealed marked microscopic hematuria. At the time of her admission to a local hospital, physical examination revealed marked flank and right upper quadrant abdominal tenderness without a palpable mass. Flat plate of the abdomen revealed loss of detail of the right psoas margin and a 4-cm. calcified mass in the right upper quadrant (Fig. 1A). An intravenous pyelogram obtained the following morning revealed displacement and efficement of the lower pole calyces of the right kidney by this calcified mass (Fig. 1B). The left kidney was normal. The patient was transferred to Mary Hitchcock Memorial Hospital. On admission, vital signs showed a temperature of 100.6” F. rectally, pulse rate of 100 per minute, and blood pressure of 110/60 mm. Hg. Findings on abdominal examination revealed
UROLOGY
/ MAY 1975 I
VOLUME
V, NUMBER 5
hypoactive bowel sounds with right flank and right upper quadrant tenderness, but no palpable mass. Urinalysis showed 10 to 15 red blood cells and 0 to 1 white blood cell per high-power field. Hemoglobin was 12.3 Gm. per 100 ml., and the white blood cell count was 8,600. We suspected that this patient had a right renal tumor, possibly damaged in her fall. Selective right renal arteriogram demonstrated neovascularity and tumor vessels within the calcified mass (Fig. 1C). There was an obvious defect in the tumor which we believed represented traumatic rupture (Fig. 1D). Because of the rarity of other renal malignancies, a diagnosis of probable Wilms’ tumor was made. Since the tumor appeared to be ruptured, immediate surgery appeared warranted. The child was explored through a right subcostal transperitoneal incision; right radical nephrectomy was carried out, excising the kidney, Gerota’s fascia, and nodal tissue at and below the renal pedicle en bloc. There was a laceration of the lower pole of the kidney through the tumor with surrounding retroperitoneal hematoma and, apparently, spillage of tumor substance both inside and outside of Gerota’s fascia. On gross examination this tumor measured 3.5 cm. in diameter and was pale and white on cut section. There was a dense fibrous pseudocapsule with focal calcification. The tumor itself was comprised primarily of clear cells arranged in sheets and papillary configurations (Fig. 2). No vascular, nodal, or perinephric tumor spread was
649
FIGURE 1. (A) Flat plate of abdomen. (B) lntravenous pyelogram showing distorted lower pole calyces of right kidney. (C) Selective right renal artetiogram showing tumor vessels in lower pole. (D) Late phase of selective arteriogram showing tumor rupture.
identified. Except for the ruptured area, the capsule was intact. A diagnosis of adenocarcinema of the kidney was made. Pending pathologic diagnosis, the child had been placed on the National Wilms’ Tumor Protocol and had already had her first postsurgical radiation treatment when the diagnosis was made. Radiation was stopped when the true nature of the tumor was known because the relative radioresistance of the adenocarcinoma and the large extent of potential spillage which made the dose and field necessary for curative
therapy unacceptable. No chemotherapy appeared to be indicated. We believed that all of the gross tumor had been removed and after careful deliberation elected not to give the child adjunctive therapy at this time, but to perform a second exploration of the area in four to six months. At that time we could identify and treat any localized recurrence. It is noteworthy that a chest film revealed normal findings, with no evidence of metastatic disease. The patient was readmitted four and one-half months after surgery, with a weight gain of 10
UROLOGY
/ MAY 1975 / VOLUME
V, NUMBER 5
FIGURE 2. Typical microscopic adenocarcinoma of kidney.
appearance
of
pounds during an uneventful convalescence. Abdominal examination revealed no masses or tenderness. Laboratory studies revealed excellent renal function and a normal blood count. Results of a chest x-ray film were normal, Abdominal exploration was carried out through the old incision and revealed no evidence of tumor recurrence. No further therapy was advised at this time. Comment The most recent review of the literature lists only 84 cases of childhood adenocarcinoma of the kidney with adequate documentation. 1 These cases show no sex predilection, and their age range is three months to eighteen years, with a mean of 9.2 years. Clinical presentation included gross hematuria in 40 per cent and a mass noted by the patient or family in 32 per cent. Evaluation
UROLOGY
I MAY 1975 / VOLUME V, NUMBER 5
revealed microscopic hematuria in another 14 per cent and a palpable mass on careful physical examination by the physician in 64 per cent of cases. Findings on intravenous pyelograms were positive in 97 per cent, with a mass seen in 86 per cent and nonfunction of the kidney in 11 per cent. Calcification was discovered radiologically in 24 per cent of the cases. We could find no other case of a ruptured adenocarcinoma of the kidney in childhood. The proper treatment of childhood adenocarcinema of the kidney appears to be surgical. Most collected series show no demonstrable benefit from radiation therapy. ‘3’There is practically no experience with adjunctive chemotherapy in these cases. Radical nephrectomy has not shown any statistically demonstrable benefit over simple nephrectomy. ’ We believe, however, in this case with tumor rupture and spillage that we should attempt to remove as much material as possible from the renal area. The prognosis appears to correlate best with the clinical stage of the tumor at surgery. Stage I disease, limited to the kidney, appears to carry a survival rate of 75 to 80 per cent for five years. ‘7’ The significance of renal vein involvement is problematic. Local spread by extension or nodal involvement appears to indicate a survival rate of about 40 per cent at five years. Distant metastases at diagnosis appears to ensure a 100 per cent mortality rate at five years. Some authors believe that the clear cell tumor and the pseudocapsule formation are favorable prognostic factors3 The effect of traumatic rupture of the tumor, such as occurred in our case, remains to be seen. The findings at reexploration as well as the localized nature of the tumor, cell type, and pseudocapsule appear to represent favorable prognostic factors. Hitchcock Clinic Hanover, New Hampshire 03755 (DR. RICHARDSON) References CASTELLANOS, R. D., ARON, B. S., and EVANS, A. T.: Renal adenocarcinoma in children, J. Urol. 111: 534 (1974). ARON, B. S., and GROSS,M.: Renal adenocarcinoma in infancy and childhood: evaluation of treatment and prognosis, ibid. 102: 497 (1969). DEHNER, L. P., LEESTMA,J. E., and PRICE,E. B., JR.: Renal cell carcinoma in children: a clinicopathologic study of 15 cases and review of the literature, J. Pediatr. 76: 358 (1970).
651
OF KIDNEY
IN A CHILD
Tumor Rupture Following Trauma ROBERT
M. STRIMER,
JOHN R. RICHARDSON,
M.D. JR., M.D.
From the Urology Section, Dartmouth-Hitchcock Medical Center, Hanover, New Hampshire
ABSTRACT - A ten-year-old white girl with Stage I clear cell adenocarcinoma of the kidney was seen after local trauma had produced a renal laceration with rupture of the tumor. She was treated with nephrectomy followed by a second exploration several months later. No adjunctive therapy was used.
Adenocarcinoma of the kidney is rare in children. It represents less than 6 per cent of all childhood renal malignancy and less than 0.5 per cent of all renal adenocarcinoma in children.1~2 Most of these tumors present with hematuria and a palpable abdominal or flank mass, but up to 13 per cent of cases are discovered following trauma. 1 Our patient had a traumatically ruptured renal tumor. Case Report This ten-year-old white girl was in excellent health until January 24,1974, when she fell from a chair in school, striking her right flank. Immediate and persistent flank and abdominal pain prompted medical attention, and urinalysis revealed marked microscopic hematuria. At the time of her admission to a local hospital, physical examination revealed marked flank and right upper quadrant abdominal tenderness without a palpable mass. Flat plate of the abdomen revealed loss of detail of the right psoas margin and a 4-cm. calcified mass in the right upper quadrant (Fig. 1A). An intravenous pyelogram obtained the following morning revealed displacement and efficement of the lower pole calyces of the right kidney by this calcified mass (Fig. 1B). The left kidney was normal. The patient was transferred to Mary Hitchcock Memorial Hospital. On admission, vital signs showed a temperature of 100.6” F. rectally, pulse rate of 100 per minute, and blood pressure of 110/60 mm. Hg. Findings on abdominal examination revealed
UROLOGY
/ MAY 1975 I
VOLUME
V, NUMBER 5
hypoactive bowel sounds with right flank and right upper quadrant tenderness, but no palpable mass. Urinalysis showed 10 to 15 red blood cells and 0 to 1 white blood cell per high-power field. Hemoglobin was 12.3 Gm. per 100 ml., and the white blood cell count was 8,600. We suspected that this patient had a right renal tumor, possibly damaged in her fall. Selective right renal arteriogram demonstrated neovascularity and tumor vessels within the calcified mass (Fig. 1C). There was an obvious defect in the tumor which we believed represented traumatic rupture (Fig. 1D). Because of the rarity of other renal malignancies, a diagnosis of probable Wilms’ tumor was made. Since the tumor appeared to be ruptured, immediate surgery appeared warranted. The child was explored through a right subcostal transperitoneal incision; right radical nephrectomy was carried out, excising the kidney, Gerota’s fascia, and nodal tissue at and below the renal pedicle en bloc. There was a laceration of the lower pole of the kidney through the tumor with surrounding retroperitoneal hematoma and, apparently, spillage of tumor substance both inside and outside of Gerota’s fascia. On gross examination this tumor measured 3.5 cm. in diameter and was pale and white on cut section. There was a dense fibrous pseudocapsule with focal calcification. The tumor itself was comprised primarily of clear cells arranged in sheets and papillary configurations (Fig. 2). No vascular, nodal, or perinephric tumor spread was
649
FIGURE 1. (A) Flat plate of abdomen. (B) lntravenous pyelogram showing distorted lower pole calyces of right kidney. (C) Selective right renal artetiogram showing tumor vessels in lower pole. (D) Late phase of selective arteriogram showing tumor rupture.
identified. Except for the ruptured area, the capsule was intact. A diagnosis of adenocarcinema of the kidney was made. Pending pathologic diagnosis, the child had been placed on the National Wilms’ Tumor Protocol and had already had her first postsurgical radiation treatment when the diagnosis was made. Radiation was stopped when the true nature of the tumor was known because the relative radioresistance of the adenocarcinoma and the large extent of potential spillage which made the dose and field necessary for curative
therapy unacceptable. No chemotherapy appeared to be indicated. We believed that all of the gross tumor had been removed and after careful deliberation elected not to give the child adjunctive therapy at this time, but to perform a second exploration of the area in four to six months. At that time we could identify and treat any localized recurrence. It is noteworthy that a chest film revealed normal findings, with no evidence of metastatic disease. The patient was readmitted four and one-half months after surgery, with a weight gain of 10
UROLOGY
/ MAY 1975 / VOLUME
V, NUMBER 5
FIGURE 2. Typical microscopic adenocarcinoma of kidney.
appearance
of
pounds during an uneventful convalescence. Abdominal examination revealed no masses or tenderness. Laboratory studies revealed excellent renal function and a normal blood count. Results of a chest x-ray film were normal, Abdominal exploration was carried out through the old incision and revealed no evidence of tumor recurrence. No further therapy was advised at this time. Comment The most recent review of the literature lists only 84 cases of childhood adenocarcinoma of the kidney with adequate documentation. 1 These cases show no sex predilection, and their age range is three months to eighteen years, with a mean of 9.2 years. Clinical presentation included gross hematuria in 40 per cent and a mass noted by the patient or family in 32 per cent. Evaluation
UROLOGY
I MAY 1975 / VOLUME V, NUMBER 5
revealed microscopic hematuria in another 14 per cent and a palpable mass on careful physical examination by the physician in 64 per cent of cases. Findings on intravenous pyelograms were positive in 97 per cent, with a mass seen in 86 per cent and nonfunction of the kidney in 11 per cent. Calcification was discovered radiologically in 24 per cent of the cases. We could find no other case of a ruptured adenocarcinoma of the kidney in childhood. The proper treatment of childhood adenocarcinema of the kidney appears to be surgical. Most collected series show no demonstrable benefit from radiation therapy. ‘3’There is practically no experience with adjunctive chemotherapy in these cases. Radical nephrectomy has not shown any statistically demonstrable benefit over simple nephrectomy. ’ We believe, however, in this case with tumor rupture and spillage that we should attempt to remove as much material as possible from the renal area. The prognosis appears to correlate best with the clinical stage of the tumor at surgery. Stage I disease, limited to the kidney, appears to carry a survival rate of 75 to 80 per cent for five years. ‘7’ The significance of renal vein involvement is problematic. Local spread by extension or nodal involvement appears to indicate a survival rate of about 40 per cent at five years. Distant metastases at diagnosis appears to ensure a 100 per cent mortality rate at five years. Some authors believe that the clear cell tumor and the pseudocapsule formation are favorable prognostic factors3 The effect of traumatic rupture of the tumor, such as occurred in our case, remains to be seen. The findings at reexploration as well as the localized nature of the tumor, cell type, and pseudocapsule appear to represent favorable prognostic factors. Hitchcock Clinic Hanover, New Hampshire 03755 (DR. RICHARDSON) References CASTELLANOS, R. D., ARON, B. S., and EVANS, A. T.: Renal adenocarcinoma in children, J. Urol. 111: 534 (1974). ARON, B. S., and GROSS,M.: Renal adenocarcinoma in infancy and childhood: evaluation of treatment and prognosis, ibid. 102: 497 (1969). DEHNER, L. P., LEESTMA,J. E., and PRICE,E. B., JR.: Renal cell carcinoma in children: a clinicopathologic study of 15 cases and review of the literature, J. Pediatr. 76: 358 (1970).
651
