Vol. 121, March Printed in U.S.A.
JOURNAL OF UROLOGY
G,.;;:;y.:i;;;\c.t © 1979 by The Williams & Wilkins Co.
WILMS TUMOR IN ADOLESCENCE KAMLA SHAH, SANTOSH WASAN
AND
STEWART LOTT
From the Department o{Therapeutic Radiology, the Ontario Cancer Treatment and Research Foundation and Queen's University, Kingston, Ontario, Canada
ABSTRACT
A review of the literature reveals that Wilms tumor is rare in adolescence. At the time of diagnosis 78 per cent of children are less than 5 years old. Of 17 cases of Wilms tumor seen at the Ontario Cancer Foundation Clinic in Kingston from 1973 to 1975, 3 children were between 16 and 17 years old. Of 316 cases registered in Ontario with the Ontario Cancer Treatment and Research Foundation only 5 were in the 15 to 19-year age group. The possibility of Wilms tumor in patients beyond the usual age group is not considered as a differential diagnosis because of its relative rarity. Patients may present with features suggestive of neuroblastoma, renal cell carcinoma, hydronephrosis, cholecystitis, appendicitis and twisted ovarian cyst as seen in our 3 patients. In comparison, a review of 84 reported cases of renal cell carcinoma in children from 1934 to 1974 showed 5 cases in the 15 to 18-year age group. invasion was present in a few foci. Thus, this represented a Wilms tumor with capsular invasion but no extension into the renal vein or renal pelvis. For 6 days 0.5 mg. actinomycin D was given intravenously followed by radiotherapy to a dose of 3,000 rad in 20 treatments for 4 weeks to the right side of the abdomen. In May 1973 bilateral pulmonary lesions developed. Both lungs were irradiated to a dose of 1,200 rad plus 1,800 rad to CASE REPORTS metastases in the lungs in 20 treatments for 5 weeks. ChemoCase 1. A 17-year-old girl presented to the emergency therapy was advised but refused by the patient. Psychological department on November 15, 1971 with abdominal pain 1 day problems and drug addiction made management of this pain duration and no nausea, vomiting or diarrhea. Examina- tient difficult. In June severe chest pain developed owing to tion revealed tenderness in the right renal angle and right metastases in the pleurae and lungs. The patient received a lower quadrant of the abdomen but no abdominal mass. There further palliative course of radiotherapy and was started on was tenderness on rectal examination. Differential diagnosis vincristine but she died on October 25. No postmortem examiincluded appendicitis and ovarian cyst. Laparotomy was done nation was done. Case 2. A 15-year-old girl presented to the emergency immediately, a large amount of serosanguineous fluid was drained from the peritoneal cavity, the appendix was removed department in March 1974 with a 4-day history of progressive and the ovaries showed no disease. The patient appeared pale abdominal pain. The pain was of sudden onset, localized to 1 day postoperatively and there was a sharp decrease in the the right side of the abdomen, radiated to the right flank and hemoglobin. She was re-explored and a large amount of was associated with nausea and chills. Examination revealed serosanguineous fluid was removed from the pelvic cavity. A a soft abdomen with normal bowel sounds and tenderness in marked cystic enlargement of the right kidney was found and the right upper and lower quadrants, right flank and costowas considered to be hydronephrosis. A postoperative excre- vertebral angle. Hemoglobin was 13.3 gm. and white blood tory urogram (IVP) showed a large mass in the upper pole of count was 15,000. The patient was treated initially for a the right kidney, displacing the calices, and several small urinary tract infection. At hospitalization the bilirubin was calcifications in the mass. The presence of a neuroblastoma 1.5 mg. per cent but within 24 hours it increased to 2.2 mg. was suspected. A renal arteriogram showed a large mass per cent and the pain worsened. At this stage the clinical enveloping the right kidney consistent with either neuroblas- diagnosis was thought to be ascending cholangitis. On March 3 a laparotomy revealed a soft, smooth, cystic toma or renal cell carcinoma. On January 7, 1972 a transabdominal nephrectomy was right renal mass. Frozen section of the aspirated blood-stained done. Gross examination revealed a 404 gm. renal mass material revealed a malignancy suggestive ofWilms tumor. A measuring 14 x 7 x 6 cm. Cut sections revealed that the right nephrectomy was done. The specimen consisted of a upper two-thirds of the kidney were replaced by a mass and right kidney, weighing 445 gm. and containing a 7 cm. tumor normal renal tissue was recognized in the lower third. The mass in its middle portion. On cut surface the tumor appeared upper half of the tumor showed hemorrhage and necrosis, lobulated with yellowish areas and areas of hemorrhagic while the lower half contained a grayish white firm tumor necrosis. There was no invasion of the capsule, renal vein or with a lobulated appearance. It also showed areas of hemor- renal pelvis. Microscopically, this was a poorly differentiated rhage and necrosis. There was no evidence of capsular, renal tumor with sheets of undifferentiated mesenchymal cells lying in a sparse connective tissue stroma. In some of the section vein or ureteral invasion. Microscopic examination revealed a highly cellular tumor the tumor showed poorly formed tubules representing epithecomposed mainly of undifferentiated mesenchymal cells. lial elements. An IVP, chest x-rays and tomograms of the There was an occasional tubular structure representing epi- chest showed no evidence of metastases. Postoperative therapy included 1 mg. actinomycin D intrathelial elements. No striated muscle was found. Capsular venously during the procedure followed by 0.5 mg. actinomyAccepted for publication June 27, 1978. cin D intravenously daily for 5 days. From March 17 to April Wilms tumor (nephroblastoma) is said to be a rare tumor in adolescence. 1 At the Ontario Cancer Foundation Clinic in Kingston 17 cases of Wilms tumor were seen from 1943 to 1975. From 1972 to 1975, 2 girls and 1 boy between 16 and 17 years old were seen at our clinic. Herein we present these 3 cases to illustrate several factors involving the diagnosis and their implications in the management of these patients.
365
366
SHAH, W ASAN AND LOTT
2, 1975, 3,000 rad were given to the tumor bed in 15 treatments for 3 weeks. On April 3 the patient was started on chemotherapy and was given 0.9 mg. actinomycin D intravenously and 2 mg. vincristine intravenously on alternate weeks. The last treatment was given on May 22. Psychological aspects included problems of coping with the reality of the disease. The patient showed little insight into the necessity of continuing treatments despite repeated explanations. She was lost to followup after May 1975, at which time she was well and had no evidence of disease. Case 3. A 16-year-old boy presented on November 11, 1975 with a 7-week history of pain in the left lower quadrant of the abdomen, malaise, headaches and a mass in the left upper quadrant. Differential diagnosis included splenomegaly, Wilms tumor, renal cell carcinoma and renal cyst. An aortogram and left renal arteriogram suggested Wilms tumor because of the presence of a mass, hypervascularity and lack of calcification in the mass. A laparotomy on November 20 revealed a huge mass arising from the left kidney, which was adherent to the spleen and colon. The kidney, spleen and part of the colon were resected. There was a 12 cm. necrotic, friable renal mass destroying a large portion of the normal renal parenchyma. Microscopically, this was a Wilms tumor characterized by a proliferation ofmesenchymal cells in a lobulated fashion. The intervening stroma was myxomatous. A few areas of tubular differentiation were noted. There was no evidence of capsular invasion. Postoperative therapy included 15 µ,g./kg. actinomycin D intravenously daily for 5 days. A severe generalized pustular rash, fever and bleeding from the rectum occurred. The platelet count decreased from 300,000 to 52,000 and later to almost a few hundred platelets. Actinomycin D was stopped at that stage. From January 14 to February 5, 1976, 3,000 rad were given to the tumor bed in 17 treatments for 3½ weeks. A week after completion of radiotherapy the patient was started on alternate weekly administration of 1 mg. actinomycin D intravenously and 2 mg. vincristine intravenously. In August the vincristine was stopped because of neuropathy and the actinomycin D was stopped because of hair loss, mucosal reactions, skin rash and acne. In January 1977 there was no evidence of recurrence and the chest x-ray was clear. DIAGNOSIS
The possibility of a Wilms tumor in patients beyond the usual age group is often dismissed or not considered because of its relative rarity. As in our 3 cases patients may present with features suggestive of neuroblastoma, renal cell carcinoma, hydronephrosis, urinary tract infection, cholecystitis, appendicitis and twisted ovarian cysts. Case 1 had had 3 laparotomies and a 7-week delay from the onset of symptoms to nephrectomy. DISCUSSION
Age at nephrectomy. The peak incidence of Wilms tumor occurs between the second and sixth years of life. Of these children 78 per cent are
JOURNAL OF UROLOGY
G,.;;:;y.:i;;;\c.t © 1979 by The Williams & Wilkins Co.
WILMS TUMOR IN ADOLESCENCE KAMLA SHAH, SANTOSH WASAN
AND
STEWART LOTT
From the Department o{Therapeutic Radiology, the Ontario Cancer Treatment and Research Foundation and Queen's University, Kingston, Ontario, Canada
ABSTRACT
A review of the literature reveals that Wilms tumor is rare in adolescence. At the time of diagnosis 78 per cent of children are less than 5 years old. Of 17 cases of Wilms tumor seen at the Ontario Cancer Foundation Clinic in Kingston from 1973 to 1975, 3 children were between 16 and 17 years old. Of 316 cases registered in Ontario with the Ontario Cancer Treatment and Research Foundation only 5 were in the 15 to 19-year age group. The possibility of Wilms tumor in patients beyond the usual age group is not considered as a differential diagnosis because of its relative rarity. Patients may present with features suggestive of neuroblastoma, renal cell carcinoma, hydronephrosis, cholecystitis, appendicitis and twisted ovarian cyst as seen in our 3 patients. In comparison, a review of 84 reported cases of renal cell carcinoma in children from 1934 to 1974 showed 5 cases in the 15 to 18-year age group. invasion was present in a few foci. Thus, this represented a Wilms tumor with capsular invasion but no extension into the renal vein or renal pelvis. For 6 days 0.5 mg. actinomycin D was given intravenously followed by radiotherapy to a dose of 3,000 rad in 20 treatments for 4 weeks to the right side of the abdomen. In May 1973 bilateral pulmonary lesions developed. Both lungs were irradiated to a dose of 1,200 rad plus 1,800 rad to CASE REPORTS metastases in the lungs in 20 treatments for 5 weeks. ChemoCase 1. A 17-year-old girl presented to the emergency therapy was advised but refused by the patient. Psychological department on November 15, 1971 with abdominal pain 1 day problems and drug addiction made management of this pain duration and no nausea, vomiting or diarrhea. Examina- tient difficult. In June severe chest pain developed owing to tion revealed tenderness in the right renal angle and right metastases in the pleurae and lungs. The patient received a lower quadrant of the abdomen but no abdominal mass. There further palliative course of radiotherapy and was started on was tenderness on rectal examination. Differential diagnosis vincristine but she died on October 25. No postmortem examiincluded appendicitis and ovarian cyst. Laparotomy was done nation was done. Case 2. A 15-year-old girl presented to the emergency immediately, a large amount of serosanguineous fluid was drained from the peritoneal cavity, the appendix was removed department in March 1974 with a 4-day history of progressive and the ovaries showed no disease. The patient appeared pale abdominal pain. The pain was of sudden onset, localized to 1 day postoperatively and there was a sharp decrease in the the right side of the abdomen, radiated to the right flank and hemoglobin. She was re-explored and a large amount of was associated with nausea and chills. Examination revealed serosanguineous fluid was removed from the pelvic cavity. A a soft abdomen with normal bowel sounds and tenderness in marked cystic enlargement of the right kidney was found and the right upper and lower quadrants, right flank and costowas considered to be hydronephrosis. A postoperative excre- vertebral angle. Hemoglobin was 13.3 gm. and white blood tory urogram (IVP) showed a large mass in the upper pole of count was 15,000. The patient was treated initially for a the right kidney, displacing the calices, and several small urinary tract infection. At hospitalization the bilirubin was calcifications in the mass. The presence of a neuroblastoma 1.5 mg. per cent but within 24 hours it increased to 2.2 mg. was suspected. A renal arteriogram showed a large mass per cent and the pain worsened. At this stage the clinical enveloping the right kidney consistent with either neuroblas- diagnosis was thought to be ascending cholangitis. On March 3 a laparotomy revealed a soft, smooth, cystic toma or renal cell carcinoma. On January 7, 1972 a transabdominal nephrectomy was right renal mass. Frozen section of the aspirated blood-stained done. Gross examination revealed a 404 gm. renal mass material revealed a malignancy suggestive ofWilms tumor. A measuring 14 x 7 x 6 cm. Cut sections revealed that the right nephrectomy was done. The specimen consisted of a upper two-thirds of the kidney were replaced by a mass and right kidney, weighing 445 gm. and containing a 7 cm. tumor normal renal tissue was recognized in the lower third. The mass in its middle portion. On cut surface the tumor appeared upper half of the tumor showed hemorrhage and necrosis, lobulated with yellowish areas and areas of hemorrhagic while the lower half contained a grayish white firm tumor necrosis. There was no invasion of the capsule, renal vein or with a lobulated appearance. It also showed areas of hemor- renal pelvis. Microscopically, this was a poorly differentiated rhage and necrosis. There was no evidence of capsular, renal tumor with sheets of undifferentiated mesenchymal cells lying in a sparse connective tissue stroma. In some of the section vein or ureteral invasion. Microscopic examination revealed a highly cellular tumor the tumor showed poorly formed tubules representing epithecomposed mainly of undifferentiated mesenchymal cells. lial elements. An IVP, chest x-rays and tomograms of the There was an occasional tubular structure representing epi- chest showed no evidence of metastases. Postoperative therapy included 1 mg. actinomycin D intrathelial elements. No striated muscle was found. Capsular venously during the procedure followed by 0.5 mg. actinomyAccepted for publication June 27, 1978. cin D intravenously daily for 5 days. From March 17 to April Wilms tumor (nephroblastoma) is said to be a rare tumor in adolescence. 1 At the Ontario Cancer Foundation Clinic in Kingston 17 cases of Wilms tumor were seen from 1943 to 1975. From 1972 to 1975, 2 girls and 1 boy between 16 and 17 years old were seen at our clinic. Herein we present these 3 cases to illustrate several factors involving the diagnosis and their implications in the management of these patients.
365
366
SHAH, W ASAN AND LOTT
2, 1975, 3,000 rad were given to the tumor bed in 15 treatments for 3 weeks. On April 3 the patient was started on chemotherapy and was given 0.9 mg. actinomycin D intravenously and 2 mg. vincristine intravenously on alternate weeks. The last treatment was given on May 22. Psychological aspects included problems of coping with the reality of the disease. The patient showed little insight into the necessity of continuing treatments despite repeated explanations. She was lost to followup after May 1975, at which time she was well and had no evidence of disease. Case 3. A 16-year-old boy presented on November 11, 1975 with a 7-week history of pain in the left lower quadrant of the abdomen, malaise, headaches and a mass in the left upper quadrant. Differential diagnosis included splenomegaly, Wilms tumor, renal cell carcinoma and renal cyst. An aortogram and left renal arteriogram suggested Wilms tumor because of the presence of a mass, hypervascularity and lack of calcification in the mass. A laparotomy on November 20 revealed a huge mass arising from the left kidney, which was adherent to the spleen and colon. The kidney, spleen and part of the colon were resected. There was a 12 cm. necrotic, friable renal mass destroying a large portion of the normal renal parenchyma. Microscopically, this was a Wilms tumor characterized by a proliferation ofmesenchymal cells in a lobulated fashion. The intervening stroma was myxomatous. A few areas of tubular differentiation were noted. There was no evidence of capsular invasion. Postoperative therapy included 15 µ,g./kg. actinomycin D intravenously daily for 5 days. A severe generalized pustular rash, fever and bleeding from the rectum occurred. The platelet count decreased from 300,000 to 52,000 and later to almost a few hundred platelets. Actinomycin D was stopped at that stage. From January 14 to February 5, 1976, 3,000 rad were given to the tumor bed in 17 treatments for 3½ weeks. A week after completion of radiotherapy the patient was started on alternate weekly administration of 1 mg. actinomycin D intravenously and 2 mg. vincristine intravenously. In August the vincristine was stopped because of neuropathy and the actinomycin D was stopped because of hair loss, mucosal reactions, skin rash and acne. In January 1977 there was no evidence of recurrence and the chest x-ray was clear. DIAGNOSIS
The possibility of a Wilms tumor in patients beyond the usual age group is often dismissed or not considered because of its relative rarity. As in our 3 cases patients may present with features suggestive of neuroblastoma, renal cell carcinoma, hydronephrosis, urinary tract infection, cholecystitis, appendicitis and twisted ovarian cysts. Case 1 had had 3 laparotomies and a 7-week delay from the onset of symptoms to nephrectomy. DISCUSSION
Age at nephrectomy. The peak incidence of Wilms tumor occurs between the second and sixth years of life. Of these children 78 per cent are
