Scand J Urol Nephrol9: 285-288, 1975
BLADDER PAPILLOMA IN A CHILD Case Report
Sigvald Refsum Jr and Sigvald B. Refsum d. y.
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
From the Department of Surgery, Sarpsborg sykehus, Sarpsborg, and the Institute of Pathology, University of Oslo, Rikshospitakt, Oslo, Norway
(Submitted for publication June 10, 1974)
Absfract. A case of bladder papilloma in a 12-year-old boy is presented. He had had painless, intermittent hematuria. An excretory urogram revealed a tilling defect in the bladder. A walnut-sized, pedunculated papilloma was removed by transvesical extirpation. Histological examination showed a papillary bladder tumour, grade 1 (Bergkvist et al., 1%5). Our patient also had a Cushing syndrome and later a bilateral adrenalectomy was done. The coexistence of two such rare conditions may not be purely coincidental. Control cystoscopies have shown no sign of tumoral recurrence.
Bladder tumours are very rare in children. Reports in the literature are mostly of single cases. When seen, they are predominantly of mesochymal origin, mainly malignant rhabdomyosarcoma and myxoma (Siegel & Pincus, 1%9; Williams & Schistad, 1961). Bladder tumours of epithelial origin are extremely rare, and it is commonly accepted that in Europe and the USA epithelial bladder tumours virtually do not exist before the age of thirty (Stenwig, Miller & Poppe, 1973). Some authors even believe that the villous papilloma of adult pathology does not occur before adolescence (Higgins, Williams & Nash, 1951). However, in recent years a few cases have been reported. In 1955 Lowry, Soanes & Forbes reported a case of grade 1 papillary carcinoma of the urinary bladder in a 6-year-old boy, and they stated that this was the fifth case in the literature till then. In 1957 Waller & Roll reported two small grade I papillary carcinomas in the bladder of a 15-year-old girl. In 1%1 Kohler added a case of grade 1 transitional cell carcinoma in an Wyear-
old boy. He reviewed the bladder tumours on file at the Michael Reese Hospital in Chicago for the years 1941 to 1%1 and found two primary tumours in the young age group, one in a 12-year-old boy with a papillary transitional cell carcinoma grade I to 2, and the other in a 17-year-old boy with a grade 1 papillary carcinoma. In 1969 Firstater, Heyman & Loewenthal reported a case of bladder papilloma in a 10-year-old Israeli girl, which they believed was the second reported case of a benign epithelial tumour in a child, not counting the cases of what they termed malignant epithelial neoplasms previously reported in children. In 1%9 Siegel & Pincus also reported a case of bladder papilloma, in an 8-year-old boy. The rareness of this type of tumour in children prompts us to report our case.
CASE REPORT A 12-year-old boy (born February 13, 1960) was referred for examination because of intermittent, painless hematuria. This had been observed four or five times since the beginning of September 1972, with the last episode 4 days prior to admission. Each time the hematuria had lasted a day o r two, the urine once being macroscopically red and the other times containing, coagula. An intravenous urography was done and revealed an irregular filling defect in the bottom of the bladder on the left side, resembling a bladder papilloma (Figs. I and 2). The kidneys and ureters were normal. On admission to the hospital (January 1 1 . 1973) he was found to be short and overweight with a somewhat peculiar appearance, with adiposity mainly localized to Scand J Urol Nephrol9
286
S. Refsum Jr and S. B . Refsum d . y . Cystoscopy 4, 9 and 16 months later has disclosed no sign of papilloma recurrence. Half a year after his first admission he had developed a characteristic Cushing syndrome. He had for the last 2-3 years been gaining weight and he had at the same time stopped growing. He was found to have hyperplasia of the adrenal glands, and a bilateral adrenalectomy was performed. Histological examination showed cortical hyperplasia of the adrenal glands. He is now receiving hormone substitution therapy, and his Cushing appearance has already almost disappeared.
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
DISCUSSION
Fig. 1 . Excretory urogram showing an irregular filling defect in the bladder.
the trunk and neck and relatively slender extremities with some striae. The face was reddish with some acne. The blood pressure was 140/75. No oedema. Examination of the heart, lungs and abdomen revealed normal findings. Hb 102%. ESR I I mm. Kreatinin 1.00.8 mg%. Urinanalysis: Specific gravity 1027, protein negative, sugar negative, microscopically C k l red blood cells, no bacteria, no casts. Normal ECG and chest X-rays. Cystoscopy was performed under general anaesthesia. Just inside the internal urethral orifrce a large tumour that looked like a papilloma was seen. Suprapubic exploration of the urinary bladder was done, and a rather broad-based, somewhat pedunculated papilloma about the size of a walnut was found, just behind and lateral to the orifice of the left ureter. The papilloma was excised together with the normal mucosa and submucosa surrounding it. The defect in the mucosa was closed with sutures. Recovery was uneventful and the patient was discharged 12 days after operation. Microscopically (Laboratorium for Patologi, P I124/ 73). a papillary tumour was seen (Fig. 3). The papillae were covered with multilayered cylindrical cells with a tendency to flattening off at the surface. The nuclei varied slightly in size and only a few were in mitosis. There was no hyperchromasia or any atypia of the nuclei, and the cytoplasm was fairly abundant (Fig. 4). The interstitial stroma was scanty with no sign of any leucocyte infiltration. The border between the tumour and the underlying stroma was sharp with no sign of any dissolution of the cell borders or infiltration down into the underlying stroma. At the line of resection a normal bladder urothelium was found in all the sections examined. A diagnosis of papillary bladder tumour, grade 1 (Bergkvist, Ljungqvist & Morberger. 1%5) was made. Scand J Urol Nephrol9
The incidence of papillary bladder tumours is increasing in many countries, Norway included (Stenwig et al., 1!373). Although epithelial bladder tumours are reported to be practically non-existent before the age of thirty (Stenwig et al., 1973), the Cancer Registry of Norway nevertheless contains 12 cases of papillary bladder tumours in persons 20 years and younger (Einar Pedersen, personal communication). The condition might therefore not be as extremely rare in children as reports from other countries have suggested. Although the term papilloma implies benignity and should be curable, the papillary bladder tumours in adult life are seldom benign and recurrences are common. This is the reason why particularly American authors use the term papillary carcinoma, grade I , instead of bladder papilloma. We prefer to use the more non-committal term papillary tumour of the bladder together with a grading of the cellular atypia, according to Bergkvist’s classification. The epithelial tumours of the urinary bladder occurring during the first two decades of life
Fig. 2. Cystogram showing the filling defect caused
by the bladder papilloma.
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
Bladder papilloma in a child
287
Fig. 3. Papillary bladder tumour with free line of resection. ~ 3 . 5 .
Fig. 4. Papillary bladder tumour, Bergkvist grade I . x 140. Scand J Oral Nephrol9
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
288
S . Refsum Jr and S . B . Refsum d . y .
differ markedly from their more malignant counterparts in the older age groups. The former are of low grade malignancy (grade 1 o r 2) morphologically and show no evidence of invasion. They run a benign clinical course. No recurrences have been reported in children (Siegel & Pincus, 1969). The common symptom in all the reported cases is hematuria, and it is also the initial symptom. We would therefore like to stress the importance of a thorough urological examination in all cases of hematuria also in children. Concerning the etiology of epithelial bladder tumours, certain carcinogenic agents (betanaphthylamine, xenylamine and others) are known, and there is also a correlation to smoking (Stenwig et al., 1973). In some African countries there is a much higher incidence of epithelial bladder tumour occurring approximately 20 years earlier in life. In these countries it is often seen in connection with bilharziasis. In our patient none of these etiological factors were present. It is interesting, however, that the boy also had a Cushing syndrome. The coexist-
Scand J Vrol Nephml9
ence of two such rare conditions may not be purely coincidental.
REFERENCES Bergkvist, A., Ljungqvist, A. & Morberger, G. 1%5. Classifications of bladder tumours based on the cellular pattern. Actu Chir Scund J30, 371. Firstater, M., Heyman, I. & Loewenthal, M. 1969. Bladder papilloma in a child: Case Report. J Urol 101, 57.
Higgins, T. T., Williams, D. I. & Nash, D. F. E. 1951. Tumours of the bladder. In The urology of childhood, 183. Butterworth, London. Kohler, F. P. 1%1. Carcinoma of the bladder in the second decade. J Urol 85, 284. Lowry, E. C., Soanes, W. A. & Forbes, K. A. 1955. Carcinoma of the bladder in children: Case report. J Urol73, 307. Siegel, W. H. & Pincus, M. B. 1%9. Epithelial bladder tumours in children.J Urol101, 55. Stenwig, A. E., Miller, A. & Poppe, E. 1973. Svulster i urinblaeren. Tidsskr Nor Laegeforen 93, 1948. Waller, J. I. & Roll, W. A. 1957. Bladder carcinoma in a teenaged girl. J Urol78, 764. Williams, D. I. & Schistad, G. 1%1. Lower urinary tract tumours in children. Br J Urol36, 51.
BLADDER PAPILLOMA IN A CHILD Case Report
Sigvald Refsum Jr and Sigvald B. Refsum d. y.
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
From the Department of Surgery, Sarpsborg sykehus, Sarpsborg, and the Institute of Pathology, University of Oslo, Rikshospitakt, Oslo, Norway
(Submitted for publication June 10, 1974)
Absfract. A case of bladder papilloma in a 12-year-old boy is presented. He had had painless, intermittent hematuria. An excretory urogram revealed a tilling defect in the bladder. A walnut-sized, pedunculated papilloma was removed by transvesical extirpation. Histological examination showed a papillary bladder tumour, grade 1 (Bergkvist et al., 1%5). Our patient also had a Cushing syndrome and later a bilateral adrenalectomy was done. The coexistence of two such rare conditions may not be purely coincidental. Control cystoscopies have shown no sign of tumoral recurrence.
Bladder tumours are very rare in children. Reports in the literature are mostly of single cases. When seen, they are predominantly of mesochymal origin, mainly malignant rhabdomyosarcoma and myxoma (Siegel & Pincus, 1%9; Williams & Schistad, 1961). Bladder tumours of epithelial origin are extremely rare, and it is commonly accepted that in Europe and the USA epithelial bladder tumours virtually do not exist before the age of thirty (Stenwig, Miller & Poppe, 1973). Some authors even believe that the villous papilloma of adult pathology does not occur before adolescence (Higgins, Williams & Nash, 1951). However, in recent years a few cases have been reported. In 1955 Lowry, Soanes & Forbes reported a case of grade 1 papillary carcinoma of the urinary bladder in a 6-year-old boy, and they stated that this was the fifth case in the literature till then. In 1957 Waller & Roll reported two small grade I papillary carcinomas in the bladder of a 15-year-old girl. In 1%1 Kohler added a case of grade 1 transitional cell carcinoma in an Wyear-
old boy. He reviewed the bladder tumours on file at the Michael Reese Hospital in Chicago for the years 1941 to 1%1 and found two primary tumours in the young age group, one in a 12-year-old boy with a papillary transitional cell carcinoma grade I to 2, and the other in a 17-year-old boy with a grade 1 papillary carcinoma. In 1969 Firstater, Heyman & Loewenthal reported a case of bladder papilloma in a 10-year-old Israeli girl, which they believed was the second reported case of a benign epithelial tumour in a child, not counting the cases of what they termed malignant epithelial neoplasms previously reported in children. In 1%9 Siegel & Pincus also reported a case of bladder papilloma, in an 8-year-old boy. The rareness of this type of tumour in children prompts us to report our case.
CASE REPORT A 12-year-old boy (born February 13, 1960) was referred for examination because of intermittent, painless hematuria. This had been observed four or five times since the beginning of September 1972, with the last episode 4 days prior to admission. Each time the hematuria had lasted a day o r two, the urine once being macroscopically red and the other times containing, coagula. An intravenous urography was done and revealed an irregular filling defect in the bottom of the bladder on the left side, resembling a bladder papilloma (Figs. I and 2). The kidneys and ureters were normal. On admission to the hospital (January 1 1 . 1973) he was found to be short and overweight with a somewhat peculiar appearance, with adiposity mainly localized to Scand J Urol Nephrol9
286
S. Refsum Jr and S. B . Refsum d . y . Cystoscopy 4, 9 and 16 months later has disclosed no sign of papilloma recurrence. Half a year after his first admission he had developed a characteristic Cushing syndrome. He had for the last 2-3 years been gaining weight and he had at the same time stopped growing. He was found to have hyperplasia of the adrenal glands, and a bilateral adrenalectomy was performed. Histological examination showed cortical hyperplasia of the adrenal glands. He is now receiving hormone substitution therapy, and his Cushing appearance has already almost disappeared.
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
DISCUSSION
Fig. 1 . Excretory urogram showing an irregular filling defect in the bladder.
the trunk and neck and relatively slender extremities with some striae. The face was reddish with some acne. The blood pressure was 140/75. No oedema. Examination of the heart, lungs and abdomen revealed normal findings. Hb 102%. ESR I I mm. Kreatinin 1.00.8 mg%. Urinanalysis: Specific gravity 1027, protein negative, sugar negative, microscopically C k l red blood cells, no bacteria, no casts. Normal ECG and chest X-rays. Cystoscopy was performed under general anaesthesia. Just inside the internal urethral orifrce a large tumour that looked like a papilloma was seen. Suprapubic exploration of the urinary bladder was done, and a rather broad-based, somewhat pedunculated papilloma about the size of a walnut was found, just behind and lateral to the orifice of the left ureter. The papilloma was excised together with the normal mucosa and submucosa surrounding it. The defect in the mucosa was closed with sutures. Recovery was uneventful and the patient was discharged 12 days after operation. Microscopically (Laboratorium for Patologi, P I124/ 73). a papillary tumour was seen (Fig. 3). The papillae were covered with multilayered cylindrical cells with a tendency to flattening off at the surface. The nuclei varied slightly in size and only a few were in mitosis. There was no hyperchromasia or any atypia of the nuclei, and the cytoplasm was fairly abundant (Fig. 4). The interstitial stroma was scanty with no sign of any leucocyte infiltration. The border between the tumour and the underlying stroma was sharp with no sign of any dissolution of the cell borders or infiltration down into the underlying stroma. At the line of resection a normal bladder urothelium was found in all the sections examined. A diagnosis of papillary bladder tumour, grade 1 (Bergkvist, Ljungqvist & Morberger. 1%5) was made. Scand J Urol Nephrol9
The incidence of papillary bladder tumours is increasing in many countries, Norway included (Stenwig et al., 1!373). Although epithelial bladder tumours are reported to be practically non-existent before the age of thirty (Stenwig et al., 1973), the Cancer Registry of Norway nevertheless contains 12 cases of papillary bladder tumours in persons 20 years and younger (Einar Pedersen, personal communication). The condition might therefore not be as extremely rare in children as reports from other countries have suggested. Although the term papilloma implies benignity and should be curable, the papillary bladder tumours in adult life are seldom benign and recurrences are common. This is the reason why particularly American authors use the term papillary carcinoma, grade I , instead of bladder papilloma. We prefer to use the more non-committal term papillary tumour of the bladder together with a grading of the cellular atypia, according to Bergkvist’s classification. The epithelial tumours of the urinary bladder occurring during the first two decades of life
Fig. 2. Cystogram showing the filling defect caused
by the bladder papilloma.
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
Bladder papilloma in a child
287
Fig. 3. Papillary bladder tumour with free line of resection. ~ 3 . 5 .
Fig. 4. Papillary bladder tumour, Bergkvist grade I . x 140. Scand J Oral Nephrol9
Scand J Urol Nephrol Downloaded from informahealthcare.com by McMaster University on 01/07/15 For personal use only.
288
S . Refsum Jr and S . B . Refsum d . y .
differ markedly from their more malignant counterparts in the older age groups. The former are of low grade malignancy (grade 1 o r 2) morphologically and show no evidence of invasion. They run a benign clinical course. No recurrences have been reported in children (Siegel & Pincus, 1969). The common symptom in all the reported cases is hematuria, and it is also the initial symptom. We would therefore like to stress the importance of a thorough urological examination in all cases of hematuria also in children. Concerning the etiology of epithelial bladder tumours, certain carcinogenic agents (betanaphthylamine, xenylamine and others) are known, and there is also a correlation to smoking (Stenwig et al., 1973). In some African countries there is a much higher incidence of epithelial bladder tumour occurring approximately 20 years earlier in life. In these countries it is often seen in connection with bilharziasis. In our patient none of these etiological factors were present. It is interesting, however, that the boy also had a Cushing syndrome. The coexist-
Scand J Vrol Nephml9
ence of two such rare conditions may not be purely coincidental.
REFERENCES Bergkvist, A., Ljungqvist, A. & Morberger, G. 1%5. Classifications of bladder tumours based on the cellular pattern. Actu Chir Scund J30, 371. Firstater, M., Heyman, I. & Loewenthal, M. 1969. Bladder papilloma in a child: Case Report. J Urol 101, 57.
Higgins, T. T., Williams, D. I. & Nash, D. F. E. 1951. Tumours of the bladder. In The urology of childhood, 183. Butterworth, London. Kohler, F. P. 1%1. Carcinoma of the bladder in the second decade. J Urol 85, 284. Lowry, E. C., Soanes, W. A. & Forbes, K. A. 1955. Carcinoma of the bladder in children: Case report. J Urol73, 307. Siegel, W. H. & Pincus, M. B. 1%9. Epithelial bladder tumours in children.J Urol101, 55. Stenwig, A. E., Miller, A. & Poppe, E. 1973. Svulster i urinblaeren. Tidsskr Nor Laegeforen 93, 1948. Waller, J. I. & Roll, W. A. 1957. Bladder carcinoma in a teenaged girl. J Urol78, 764. Williams, D. I. & Schistad, G. 1%1. Lower urinary tract tumours in children. Br J Urol36, 51.
