Scand J Urol Nephrol 13: 323-327, 1979
URINARY BLADDER TUMOURS IN CHILDREN Case RPport o f I r i t w t c d Papillorntr
M. Lorentzen and N . Rohr From the Institirtr c?f Pathology, tind Sitrgiccrl Department ctf U r o l o g y , 0den.w Uniivrsity Hospitctl. Denmur-k
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
(Submitted for publication September 4. 1978)
Ahstrtict. A short review is given of tumours of the urinary
bladder in children, and the first case of an inverted papilloma in a child is reported. The turnour was situated in the trigone of the bladder and was successfully extirpated, with no recurrence at follow-up 9 months later. It was histologically typical of these tumours. The 105 patients described in the literature had an average age of 55 years, whereas the present case is the first to be described arising during the first two decades of life. The most frequent symptoms of such tumours are haematuria and urinary obstruction. It is suggested that the treatment of choice is transurethral excision. The term Brunnian adenoma is recommended for use with this tumour, in order to avoid the impression given by the name papilloma of the bladder, that the tumour is malignant.
Tumours of the bladder in children are rare, and predominantly of mesodermal origin. Rhabdomyosarcoma is the most frequent neoplasm, arising mainly from the region of the trigone (Batsakis, 1963; Williams & Schistad, 1964; Ray, Grabstald, Exelby & Whitmore, 1973). Epithelial tumours of the bladder in children are extremely uncommon. Mauerrnayer, Tauber & Steuer (1977) recently collected 82 epithelial tumours of the bladder in the first two decades of life, from the literature. The tumours have been described as ordinary papillomas or carcinomas, grade 1-11. No reports have previously been published of inverted papilloma of the bladder in children or young adults. One such case is presented here in an I I-year-old boy.
CASE REPORT An 11-year-old boy was first admitted to hospital in December 1976 for appendicitis. However, the appendix was found to be normal at operation.
The patient was again admitted in September 1977, with a 4-month history of intermittent haematuria, predominantly termina, and at times associated with pain. There had been no previous urological complaints. The physical examination was negative; the blood pressure normal, as were the haemoglobin and sedimentation rate. Renal function was normal, as evaluated by the Se-creatinine and Se-urea. Analysis of the urine revealed innumerable erythrocytes, a few leukocytes, but no bacteria. An excretory pyelogram showed normal kidneys and ureters, but revealed a filling defect in the middle left half of the bladder (Fig. I ) . A mass at the same site was observed during ultrasonic scanning (Fig. 2). A polypoid, pedunculated and intensely red tumour, measuring about 2 cm was seen a little to the left of the internal urethral orifice, and below the left ureteral orifice during cystoscopy. The surface of the tumour was cauliflower-like and not papillary. Both ureteral orifices were normal, as was the mucosa outside the turnour area. A cystotomy with extirpation of the tumour was performed. No evidence of recurrence was found at cystoscopic examination 9 months later. and the patient has been free from urological complaints.
PATHOLOGY The polypoid greyish brown specimen measured 1 . 5 1~ cm, the surface being smooth or slightly lobulated. Microscopically, the surface of the lesion was covered by transitional epithelium, forming anastomosing and arborizing cords or broad sheets in the stroma (Figs. 3 and 4). The epithelium was of varying thickness and displayed moderate hyperplasia with slight nuclear enlargement and irregularity. Focal squamous metaplasia could be observed together with a few mitotic figures. Microcysts were present within the epithelial cords towards the surface, eventually displaying ‘secretory dotting’ of the inner cell lining 2X
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
324
M . LorentZen and N . Rohr
Fig. I . Cystogram showing filling defect at the site of the tumour.
(Fig. 5). These cells and the content of the cysts, which was of a homogeneous eosinophilic nature, also showed diastase-resistant PAS-positive staining. The connective tissue showed engorgement of the vessels, haemorrhage, oedema and slight to moderate diffuse infiltration by inflammatory cells, predominantly lymphocytes, but eosinophils were also encountered. Muscle fibres could not be identified at the base of the tumour.
Fig. 2. Sonogram with polypoid tumour of the urinary
bladder.
Fig. 3. Low-power view showing the surface of the tumour with anastomosing epithelial cords in the stroma. (Haematoxylin & Eosin)
DISCUSSION Tumours in the lower urinary tract are rare in children and young adults. Most such tumours are malignant, of mesodermal origin and most often situated in the trigone of the bladder. Rhabdomyosarcoma is the most common neoplasm, and 75 % of these tumours are diagnosed before the age of 5 years. The incidence in boys is twice that of girls. Urinary retention is the predominant initial symptom (Tefft & Jaffe, 1973). Epithelial tumours of the bladder are considerably less common than mesodermal tumours. Reports of isolated cases comprise the major part of the literature. In 1924, Deming referred to a questionable case of papilloma among 66 bladder tumours in the first decade of life. Forty primary epithelial tumours in the first two decades of life were found among 10 000 bladder neoplasms in the Bladder Tumor Registry at the Armed Forces Institute of Pathology (Javadpour & Mostofi, 1969). Mauermayer et al. (1977) reviewed 82 cases of epithelial tumours of the
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
Fig. 4 . Higher magnification of epithelial cords and nests in loose stroma. (H & E. x 130)
bladder in the first two decades of life from the literature after 1950. Epithelial tumours of the bladder in children differ in biologic behaviour from their counterparts in adults. They are usually solitary, papillary, and morphologically of low grade malignancy. Most tumours are situated on the lateral walls of the bladder, and designated papillomas or carcinomas grade 1-11; there have been no reports of grade HI carcinoma. Infiltrative growth pattern was seen in 5 of 40 cases presented by Javadpour & Mostofi (1969). These were three papillary carcinomas, one squamous cell carcinoma and one adenocarcinorna. The patient with the adenocarcinoma died with widespread metastases 4 months after the diagnosis had been established. Recurrence is extremely rare, but has been reported in a 9-year-old boy, who had five recur-
rences of multiple benign papillary tumours (Li, Kim & Brendler, 1972). Of the 82 cases reviewed by Mauermayer et al. in 1977, 14 patients were below the age of 10 years. They found a preponderance of males, with a ratio of six males to one female and they also found haematuria to be the most frequent symptom, occurring in over 90% of the cases. Since the report on a case of inverted papilloma of the bladder in a 63-year-old man by Potts & Hirst in 1963, 105 cases have been listed in the literature (Hasselstrom, 1975; Henderson, Allen & Bourne, 1975; De Meester, Farrow & Utz, 1975; Kim & Reiner, 1978; Car0 & Tessler, 1978). The case published by Kim & Reiner in 1978 was an incidental finding in a 75-year-old woman, who died from congestive heart failure due to myocardial infarction,
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
326
M . Lorrtitzeti trnd N . Rohr
Fig. 5 . Microcysts within cords containing colloid material. ( H & E, X320)
10 hours after admission to hospital. At autopsy, the bladder was found to contain a polyp, which showed the characteristic histological features of inverted papilloma. Potts & Hirst (1963) pointed out the distinctive morphological appearance of inverted papilloma, which has since been confirmed by others. It is of importance to stress the histological features in differentiating these tumours from papillary urothelial tumours of the bladder. Car0 & Tessler (1978) emphasized the necessity of this while reviewing 104 cases and comparing these with transitional cell papilloma. Our case complies with the diagnostic criteria of Henderson et al. (1975). which are as follows: 1) an inverted configuration: 2 ) a covering layer of urothelium; 3) uniformity of the epithelial cells; 4) very infrequent or absent mitoses: 5 ) mic-
rocyst formation; 6) occasional squamous metaplasia. Macroscopically, the majority of reported tumours have been pedunculated, polypoid lesions located most often to the trigone or neck of the bladder. The average age of the 105 patients described in the literature is 55 years; hitherto, the youngest patient (reported by Assor & Taylor in 1970) was a 26-year-old man. Most patients have been men; only 14 cases have been reported in women. The most frequent symptoms are haematuria and urinary obstruction; symptoms of prostatism have also been reported in 9 of 15 men, who had evidence of prostatic hypertrophy (De Meester et al.. 1975). All cases so far have run a benign course, without signs of malignant transformation. Many cases have
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
been followed f o r several y e a r s , a n d only o n e patient has had a recurrence. 2 years after resection (De M e e s t e r et al., 1975). We think, as o t h e r s . t h a t simple local excision by t h e transurethral route is t h e treatment o f c h o i c e , w h e n e v e r possible. M o s t a u t h o r s regard t h e lesions as benign neoplasms, and we a g r e e with o t h e r s in t h e histological r e s e m b l a n c e t o Brunn's nests. Owing t o this similarity, a n d b e c a u s e t h e t e r m papilloma o f t h e bladder c o n n o t e s potential malignant c h a n g e , Kim & Reiner ( 1978) h a v e proposed t h e designation Brunnian a d e n o m a .
ACKNOWLEDGEMENT The authors would like to express their thanks to the Paediatric Department, Kolding Hospital, for the loan of material regarding this case.
REFERENCES Assor, D. & Taylor, J. N. 1970. Inverted papilloma of the b1adder.J Urol 101. 715. Batsakis, J . G . 1963. Urogenital rhabdomyosarcoma: histogenesis and classification. ./ Urol YO. 180.
Caro, D. J . & Tessler, A . 1978. Inverted papilloma of the bladder. Ccim,er42, 708. De Meester, L. J . , Farrow, G. M. & Utz. D. C. 1975. Inverted papilloma of the urinary bladder. Ctrncer 36. 505. Deming, C. L. 1924. imary bladder tumours in the first Fnrcol 0h.stc.r 39, 432. decade of life. Siri Hasselstrom, K . 1975.' Inverteret blaerepapillom. Ugc'sXr Lit'ger 137, 2834. Henderson, D. W., Allen, P. W. & Bourne, A. J. 1975. Inverted urinary papilloma. Vircli Arch Ahr A 366. 177. Javadpour. N . & Mostofi, F. K. 1969. Primary epithelial tumours of the bladder in the first two decades of 1ife.J Urol 101, 706. Kim, Y . H. & Reiner, L. 1978. Brunnian adenoma (inverted papilloma) of the urinary bladder: report of a case. Hiini Ptitliol Y. 229. Li, R . , Kim, K . & Brendler, H. 1972. Multiple and recurrent epithelial tumours of the bladder in a child. J Urol 108, 644. Mauermayer, W., Tauber, R. & Steuer, G. 1977. Epitheliale harnblasentumoren im kindesalter und beim jugendlichen. Urologe A 16. 286. Potts. I . F. & Hirst, E . 1963. Inverted papilloma of the bladder. J Urol YO, 175. Ray, B., Grabstald, H., Exelby, P. R . & Whitmore, W. F. 1973. Bladder tumours in children. Urology 2 . 426. Tefft, M. & Jaffe, N . 1973. Sarcoma of the bladder and prostate in children. C ' u ~ i c e r 3 2 .1161. Williams, D. I . & Schistad. G. 1964. Lower urinary tract tumours in children. Brir J Urol36. 51,
B
URINARY BLADDER TUMOURS IN CHILDREN Case RPport o f I r i t w t c d Papillorntr
M. Lorentzen and N . Rohr From the Institirtr c?f Pathology, tind Sitrgiccrl Department ctf U r o l o g y , 0den.w Uniivrsity Hospitctl. Denmur-k
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
(Submitted for publication September 4. 1978)
Ahstrtict. A short review is given of tumours of the urinary
bladder in children, and the first case of an inverted papilloma in a child is reported. The turnour was situated in the trigone of the bladder and was successfully extirpated, with no recurrence at follow-up 9 months later. It was histologically typical of these tumours. The 105 patients described in the literature had an average age of 55 years, whereas the present case is the first to be described arising during the first two decades of life. The most frequent symptoms of such tumours are haematuria and urinary obstruction. It is suggested that the treatment of choice is transurethral excision. The term Brunnian adenoma is recommended for use with this tumour, in order to avoid the impression given by the name papilloma of the bladder, that the tumour is malignant.
Tumours of the bladder in children are rare, and predominantly of mesodermal origin. Rhabdomyosarcoma is the most frequent neoplasm, arising mainly from the region of the trigone (Batsakis, 1963; Williams & Schistad, 1964; Ray, Grabstald, Exelby & Whitmore, 1973). Epithelial tumours of the bladder in children are extremely uncommon. Mauerrnayer, Tauber & Steuer (1977) recently collected 82 epithelial tumours of the bladder in the first two decades of life, from the literature. The tumours have been described as ordinary papillomas or carcinomas, grade 1-11. No reports have previously been published of inverted papilloma of the bladder in children or young adults. One such case is presented here in an I I-year-old boy.
CASE REPORT An 11-year-old boy was first admitted to hospital in December 1976 for appendicitis. However, the appendix was found to be normal at operation.
The patient was again admitted in September 1977, with a 4-month history of intermittent haematuria, predominantly termina, and at times associated with pain. There had been no previous urological complaints. The physical examination was negative; the blood pressure normal, as were the haemoglobin and sedimentation rate. Renal function was normal, as evaluated by the Se-creatinine and Se-urea. Analysis of the urine revealed innumerable erythrocytes, a few leukocytes, but no bacteria. An excretory pyelogram showed normal kidneys and ureters, but revealed a filling defect in the middle left half of the bladder (Fig. I ) . A mass at the same site was observed during ultrasonic scanning (Fig. 2). A polypoid, pedunculated and intensely red tumour, measuring about 2 cm was seen a little to the left of the internal urethral orifice, and below the left ureteral orifice during cystoscopy. The surface of the tumour was cauliflower-like and not papillary. Both ureteral orifices were normal, as was the mucosa outside the turnour area. A cystotomy with extirpation of the tumour was performed. No evidence of recurrence was found at cystoscopic examination 9 months later. and the patient has been free from urological complaints.
PATHOLOGY The polypoid greyish brown specimen measured 1 . 5 1~ cm, the surface being smooth or slightly lobulated. Microscopically, the surface of the lesion was covered by transitional epithelium, forming anastomosing and arborizing cords or broad sheets in the stroma (Figs. 3 and 4). The epithelium was of varying thickness and displayed moderate hyperplasia with slight nuclear enlargement and irregularity. Focal squamous metaplasia could be observed together with a few mitotic figures. Microcysts were present within the epithelial cords towards the surface, eventually displaying ‘secretory dotting’ of the inner cell lining 2X
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
324
M . LorentZen and N . Rohr
Fig. I . Cystogram showing filling defect at the site of the tumour.
(Fig. 5). These cells and the content of the cysts, which was of a homogeneous eosinophilic nature, also showed diastase-resistant PAS-positive staining. The connective tissue showed engorgement of the vessels, haemorrhage, oedema and slight to moderate diffuse infiltration by inflammatory cells, predominantly lymphocytes, but eosinophils were also encountered. Muscle fibres could not be identified at the base of the tumour.
Fig. 2. Sonogram with polypoid tumour of the urinary
bladder.
Fig. 3. Low-power view showing the surface of the tumour with anastomosing epithelial cords in the stroma. (Haematoxylin & Eosin)
DISCUSSION Tumours in the lower urinary tract are rare in children and young adults. Most such tumours are malignant, of mesodermal origin and most often situated in the trigone of the bladder. Rhabdomyosarcoma is the most common neoplasm, and 75 % of these tumours are diagnosed before the age of 5 years. The incidence in boys is twice that of girls. Urinary retention is the predominant initial symptom (Tefft & Jaffe, 1973). Epithelial tumours of the bladder are considerably less common than mesodermal tumours. Reports of isolated cases comprise the major part of the literature. In 1924, Deming referred to a questionable case of papilloma among 66 bladder tumours in the first decade of life. Forty primary epithelial tumours in the first two decades of life were found among 10 000 bladder neoplasms in the Bladder Tumor Registry at the Armed Forces Institute of Pathology (Javadpour & Mostofi, 1969). Mauermayer et al. (1977) reviewed 82 cases of epithelial tumours of the
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
Fig. 4 . Higher magnification of epithelial cords and nests in loose stroma. (H & E. x 130)
bladder in the first two decades of life from the literature after 1950. Epithelial tumours of the bladder in children differ in biologic behaviour from their counterparts in adults. They are usually solitary, papillary, and morphologically of low grade malignancy. Most tumours are situated on the lateral walls of the bladder, and designated papillomas or carcinomas grade 1-11; there have been no reports of grade HI carcinoma. Infiltrative growth pattern was seen in 5 of 40 cases presented by Javadpour & Mostofi (1969). These were three papillary carcinomas, one squamous cell carcinoma and one adenocarcinorna. The patient with the adenocarcinoma died with widespread metastases 4 months after the diagnosis had been established. Recurrence is extremely rare, but has been reported in a 9-year-old boy, who had five recur-
rences of multiple benign papillary tumours (Li, Kim & Brendler, 1972). Of the 82 cases reviewed by Mauermayer et al. in 1977, 14 patients were below the age of 10 years. They found a preponderance of males, with a ratio of six males to one female and they also found haematuria to be the most frequent symptom, occurring in over 90% of the cases. Since the report on a case of inverted papilloma of the bladder in a 63-year-old man by Potts & Hirst in 1963, 105 cases have been listed in the literature (Hasselstrom, 1975; Henderson, Allen & Bourne, 1975; De Meester, Farrow & Utz, 1975; Kim & Reiner, 1978; Car0 & Tessler, 1978). The case published by Kim & Reiner in 1978 was an incidental finding in a 75-year-old woman, who died from congestive heart failure due to myocardial infarction,
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
326
M . Lorrtitzeti trnd N . Rohr
Fig. 5 . Microcysts within cords containing colloid material. ( H & E, X320)
10 hours after admission to hospital. At autopsy, the bladder was found to contain a polyp, which showed the characteristic histological features of inverted papilloma. Potts & Hirst (1963) pointed out the distinctive morphological appearance of inverted papilloma, which has since been confirmed by others. It is of importance to stress the histological features in differentiating these tumours from papillary urothelial tumours of the bladder. Car0 & Tessler (1978) emphasized the necessity of this while reviewing 104 cases and comparing these with transitional cell papilloma. Our case complies with the diagnostic criteria of Henderson et al. (1975). which are as follows: 1) an inverted configuration: 2 ) a covering layer of urothelium; 3) uniformity of the epithelial cells; 4) very infrequent or absent mitoses: 5 ) mic-
rocyst formation; 6) occasional squamous metaplasia. Macroscopically, the majority of reported tumours have been pedunculated, polypoid lesions located most often to the trigone or neck of the bladder. The average age of the 105 patients described in the literature is 55 years; hitherto, the youngest patient (reported by Assor & Taylor in 1970) was a 26-year-old man. Most patients have been men; only 14 cases have been reported in women. The most frequent symptoms are haematuria and urinary obstruction; symptoms of prostatism have also been reported in 9 of 15 men, who had evidence of prostatic hypertrophy (De Meester et al.. 1975). All cases so far have run a benign course, without signs of malignant transformation. Many cases have
Scand J Urol Nephrol Downloaded from informahealthcare.com by University of Alberta on 12/30/14 For personal use only.
been followed f o r several y e a r s , a n d only o n e patient has had a recurrence. 2 years after resection (De M e e s t e r et al., 1975). We think, as o t h e r s . t h a t simple local excision by t h e transurethral route is t h e treatment o f c h o i c e , w h e n e v e r possible. M o s t a u t h o r s regard t h e lesions as benign neoplasms, and we a g r e e with o t h e r s in t h e histological r e s e m b l a n c e t o Brunn's nests. Owing t o this similarity, a n d b e c a u s e t h e t e r m papilloma o f t h e bladder c o n n o t e s potential malignant c h a n g e , Kim & Reiner ( 1978) h a v e proposed t h e designation Brunnian a d e n o m a .
ACKNOWLEDGEMENT The authors would like to express their thanks to the Paediatric Department, Kolding Hospital, for the loan of material regarding this case.
REFERENCES Assor, D. & Taylor, J. N. 1970. Inverted papilloma of the b1adder.J Urol 101. 715. Batsakis, J . G . 1963. Urogenital rhabdomyosarcoma: histogenesis and classification. ./ Urol YO. 180.
Caro, D. J . & Tessler, A . 1978. Inverted papilloma of the bladder. Ccim,er42, 708. De Meester, L. J . , Farrow, G. M. & Utz. D. C. 1975. Inverted papilloma of the urinary bladder. Ctrncer 36. 505. Deming, C. L. 1924. imary bladder tumours in the first Fnrcol 0h.stc.r 39, 432. decade of life. Siri Hasselstrom, K . 1975.' Inverteret blaerepapillom. Ugc'sXr Lit'ger 137, 2834. Henderson, D. W., Allen, P. W. & Bourne, A. J. 1975. Inverted urinary papilloma. Vircli Arch Ahr A 366. 177. Javadpour. N . & Mostofi, F. K. 1969. Primary epithelial tumours of the bladder in the first two decades of 1ife.J Urol 101, 706. Kim, Y . H. & Reiner, L. 1978. Brunnian adenoma (inverted papilloma) of the urinary bladder: report of a case. Hiini Ptitliol Y. 229. Li, R . , Kim, K . & Brendler, H. 1972. Multiple and recurrent epithelial tumours of the bladder in a child. J Urol 108, 644. Mauermayer, W., Tauber, R. & Steuer, G. 1977. Epitheliale harnblasentumoren im kindesalter und beim jugendlichen. Urologe A 16. 286. Potts. I . F. & Hirst, E . 1963. Inverted papilloma of the bladder. J Urol YO, 175. Ray, B., Grabstald, H., Exelby, P. R . & Whitmore, W. F. 1973. Bladder tumours in children. Urology 2 . 426. Tefft, M. & Jaffe, N . 1973. Sarcoma of the bladder and prostate in children. C ' u ~ i c e r 3 2 .1161. Williams, D. I . & Schistad. G. 1964. Lower urinary tract tumours in children. Brir J Urol36. 51,
B
