©1991 S. Karger AG, Basel 0302-2838/91/0202-0113$2 75/0
Eur Urol 1991;20:113-116
Localized Amyloidosis of the Urinary Bladder 1006016
T. Lehtonena, J. Mäkinenh, S. Wikströma “Department of Urology, Helsinki University Central Hospital, Helsinki; b Department of Pathology, Lohja Regional Hospital, Lohja, Finland
Key Words. Urinary bladder • Amyloidosis • Hematuria • Transurethral resection Abstract. Localized amyloidosis of the urinary bladder is a rare condition. Five patients, 1 with localized second ary amyloidosis, are described. The symptoms, macroscopic hematuria and tumor-like appearance in cyctoscopy, may mimic bladder cancer. Diagnosis is based on histopathological examinations with Congo red staining. In most instances, the treatment of choice is transurethral resection and electrocoagulation. Because of the risk of recur rences, a close follow-up is recommended.
Patients and Methods During the years 1968-88, 5 men with episodes of macroscopic hematuria and suspicion of bladder cancer were referred to our hos pital. All patients were examined by urinary cytology, intravenous pyelogram and cystoscopy with histologic biopsy. After establishing the diagnosis, rectoscopy with rectal biopsy in order to find other focuses of amyloidosis was performed. In 1 patient, who had a pre vious history of long-standing suppuration and pulmonary tubercu losis, liver and kidney needle biopsies were obtained. In one man with large intravesical tumor, bladder GT-scan and transurethral ultrasound examination were performed prior to the operation. All men were hospitalized for further treatment.
Results Table 1 demonstrates the previous and urological his tories and cystoscopic findings of all 5 patients. In the cystoscopic examination, all but 1 revealed localized tumor in the bladder. Only 1 patient had reddish hemor rhagic areas of 1-2 cm in diameter in different parts of the bladder wall. Histologic specimen of all patients showed amyloidosis. Patient No. 5 had long-standing suppuration after a war trauma (osteomyelitis of femur) and pulmonary tuberculosis. Patient No. 3 had had in terstitial cystitis diagnosed by the typical symptoms dysuria and suprapubic pain and a cystoscopic appear ance with petechial hémorrhagies. All patients had nega tive rectal biopsies and patient No. 5 negative liver and kidney biopsies. Urinary cytology was negative in every patient. Transurethral ultrasound and bladder CT-scan re vealed a large, superficial tumor in the anterior wall of the bladder. No signs of deeper infiltration could be seen (fig- 1)Treatment and follow-up of the patients are listed in table 2. In 1 case (patient No. 1), the bladder amyloidosis with no tumor-like appearance was treated by electro-
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Amyloidosis may be secondary to long-standing infec tions (tuberculosis, osteomyelitis) and inflammatory or immunologic disease (rheumatoid arthritis, Crohn’s dis ease). Localized amyloidosis of the bladder is a rare con dition. Until 1988, we have found reports of 54 patients with primary localized amyloidosis of the bladder [1-5]. Clinically important is that the symptoms of localized amyloidosis with macroscopic hematuria and the cystoscopic appearance may give rise to suspicion of bladder cancer. Here we present 5 patients with localized bladder amyloidosis of which 1 is of the secondary type.
Lehtonen/Mäkinen/Wikström
114
Fig. 1. a Intravesical ultrasonograph showing a large superficial tumor in the anterior wall of the bladder, b Contrast-enhanced blad der CT-scan reveals the same tumor with no infiltration outside the bladder.
Table 1. Clinical data of 5 patients with localized bladder amyloidosis Patient
Age years
Previous diseases
Urological symptoms
Cystoscopic findings
Site
EK
74
BPH
macroscopic hematuria, pollakiuria
reddish, bleeding areas, 1-2 cm in diameter
bladder dome posterior wall
VK
54
macroscopic hematuria
large tumor, 3-cm diameter
right lateral wall
VP
50
macroscopic hematuria, pollakiuria, bladder pain
tumors, 2.5-cm diameter
bladder dome
BW
71
macroscopic hematuria
tumor, 2.5 X 5 cm
anterior bladder wall
FU
67
macroscopic hematuria
hard, yellowish tumor, 3-cm diameter
right lateral wall
cystitis interstitialis
suppuration of osteo myelitis, pulmonary tbc
Table 2. Treatment and follow-up of patients with localized bladder amyloidosis Primary treatment
Treatment of recurrences
Follow-up time, years
EK VK VP BW FU
electrocoagulation bladder resection bladder resection TUR bladder resection
electrocoagulation X 3 electrocoagulation X 4; small residual tumors TUR and electrocoagulation once a year TUR X 1 ; bladder resection electrocoagulation X 1
1.5 5 22
2 4
exitus: cardiac infarction recurrent tumors; exitus: cardiac infarction small recurrent tumors no recurrences exitus: cardiac infarction Downloaded by: University of Exeter 144.173.6.94 - 6/17/2020 7:06:34 PM
Patient
Localized Amyloidosis of the Urinary Bladder
Discussion Amyloidosis can be classified into five different types according to etiology [6, 7]: (1) primary, without any evi dence of predisposing disease; (2) myeloma-associated; (3) secondary, occurring as a complication of a long standing infection, inflammation or immunologic dis ease (including tuberculosis, osteomyelitis, rheumatoid arthritis, inflammatory bowel diseases), or metabolic disease (diabetes mellitus); (4) localized, with involve ment of a single organ, and (5) primary heredofamilial. Localized amyloidosis of the bladder is quite rare; there are only 54 reports in the previous literature. According to Caldamone et al. [1], who analyzed the informations given in the literature, 6 of 44 patients had bladder amyloidosis secondary to systemic diseases. One of our 5 patients showed localized amyloidosis second ary to long-standing osteomyelitis and/or pulmonary tu berculosis without any evidence of amyloidosis in liver, kidney and rectum biopsies. Macroscopic hematuria with or without dysuria is the most common symptom of local bladder amyloidosis [8]. In our material, all the patients had macroscopic hema turia and 2 patients concomitant dysuric complaints. Macroscopic hematuria together with cystoscopic find ings aroused suspicion of bladder cancer. Cystoscopically the localized bladder amyloidosis is a yellowish, broad-based or polypoid tumor which can be
* €
Fig. 2. Microscopically normal epithelium of the urinary bladder biopsy. Large masses of amyloid just under the surface in the lamina propria (arrow), van Gieson. X 125.
single or multifocal [8]. One of our patients had a single amyloid tumor on the right anterior wall of the urinary bladder which was confirmed by transurethral ultra sound and bladder CT-scan. Another 3 patients, includ ing the patient with secondary amyloidosis, had single tumors at primary cystoscopy, but multiple, small recur rencies at follow-up. Cystoscopy of one patient with pri mary amyloidosis revealed several reddish hemorrhagic areas of 10-15 mm in diameter throughout the bladder, which is more characteristic of secondary amyloidosis [4]. The definitive diagnosis of amyloidosis is based on histopathological examinations with Congo red staining. In 4 patients including the one with secondary local amy loidosis of the bladder, the deposits of amyloidosis oc curred in the submucosa and in the muscularis, which is characteristic of primary localized amyloidosis [9], In 1 patient with a more diffuse macroscopic appearance of primary amyloidosis, mucosal ulcerations and vascular involvement of subendothelial vessels by amyloidosis were seen. This kind of histological appearance is more often seen in secondary vesical amyloidosis [10]. Transurethral resection and electrocoagulation is the treatment of choice in primary localized amyloidosis of the urinary bladder [1], Only in cases with single amyloid tumor partial cystectomy seemed to give more long standing results than in one of our patients. Because of the propensity to multiple recurrences, a follow-up with cystoscopy and repeated transurethral resections were necessary. The prognosis for patients with localized bladder amyloidosis is, however, good.
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coagulations twice a year. In 3 patients, the primary treat ment was open-bladder resection. All of these patients had recurrence of small (1-1.5 cm in diameter) amyloid tumors in different parts of the bladder wall which were treated by electrocoagulations at 1- to 3-year intervals. In 1 patient (No. 4), the primary treatment was transure thral resection of the large amyloid tumor. After a 6month follow-up, a new TUR was performed in the tumor of the same site. Because of early recurrence 4 months after the second treatment an open bladder resec tion was performed. After this operation, no recurrence was noted in the 2-year follow-up. Three of our patients died of cardiac infarction. No autopsy was performed. Histologically, amyloidosis was diagnosed by staining the specimens with van Gieson’s and Congo red stains. The specimen of patient No. 1 showed subepithelial dif fuse perivascular amyloidosis deposits. In all other pa tients, large amyloidosis deposits were situated subepithelially and were present also within the muscularis (fig. 2).
115
Lehtonen/Mäkinen/Wikström
116
1 Caldamone AA, Elbadawi A, Moshtagi A, et al: Primary local ized amyloidosis of urinary bladder. Urology 1980; 15:174189. 2 Nakajima K, Hisazumi H, Okasyo A, et al: Primary localized amyloidosis of bladder. Urology 1980;15:302-303. 3 Mead MG, Hickinbotham P, Walls J: Amyloidosis localized to the bladder. Br J Urol 1982;54:428. 4 Ahmad E, Johansson SL, Fall M: Blue spotted bladder - a man ifestation of bladder amyloidosis. Scand J Urol Nephrol 1986; 20:145-147. 5 Grainger R, O’Riordan B, Cullen A, et al: Primary amyloidosis of lower urinary tract. Urology 1988;31:14-16. 6 Cohen AS: Amyloidosis. N Engl J Med 1967;277:574-583. 7 Kyle RA, Bayrd ED: Amyloidosis: Review of 236 cases. Medi cine 1975;54:271-299.
8 Malek RS, Laurence BS, Greene F, et al: Amyloidosis of the urinary bladder. Br J Urol 1971;43:189-200. 9 Strong GH, Kelsey D, Hoch W: Primary amyloid disease of the bladder. J Urol 1974; 112:463-466. 10 Au KK, Gilbaugh JH Jr: Primary amyloidosis of the bladder. J Urol 1975;114:786-787.
T. Lehtonen Department of Urology Helsinki University Haartmaninkatu 4 SF-00290 Helsinki (Finland)
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References
Eur Urol 1991;20:113-116
Localized Amyloidosis of the Urinary Bladder 1006016
T. Lehtonena, J. Mäkinenh, S. Wikströma “Department of Urology, Helsinki University Central Hospital, Helsinki; b Department of Pathology, Lohja Regional Hospital, Lohja, Finland
Key Words. Urinary bladder • Amyloidosis • Hematuria • Transurethral resection Abstract. Localized amyloidosis of the urinary bladder is a rare condition. Five patients, 1 with localized second ary amyloidosis, are described. The symptoms, macroscopic hematuria and tumor-like appearance in cyctoscopy, may mimic bladder cancer. Diagnosis is based on histopathological examinations with Congo red staining. In most instances, the treatment of choice is transurethral resection and electrocoagulation. Because of the risk of recur rences, a close follow-up is recommended.
Patients and Methods During the years 1968-88, 5 men with episodes of macroscopic hematuria and suspicion of bladder cancer were referred to our hos pital. All patients were examined by urinary cytology, intravenous pyelogram and cystoscopy with histologic biopsy. After establishing the diagnosis, rectoscopy with rectal biopsy in order to find other focuses of amyloidosis was performed. In 1 patient, who had a pre vious history of long-standing suppuration and pulmonary tubercu losis, liver and kidney needle biopsies were obtained. In one man with large intravesical tumor, bladder GT-scan and transurethral ultrasound examination were performed prior to the operation. All men were hospitalized for further treatment.
Results Table 1 demonstrates the previous and urological his tories and cystoscopic findings of all 5 patients. In the cystoscopic examination, all but 1 revealed localized tumor in the bladder. Only 1 patient had reddish hemor rhagic areas of 1-2 cm in diameter in different parts of the bladder wall. Histologic specimen of all patients showed amyloidosis. Patient No. 5 had long-standing suppuration after a war trauma (osteomyelitis of femur) and pulmonary tuberculosis. Patient No. 3 had had in terstitial cystitis diagnosed by the typical symptoms dysuria and suprapubic pain and a cystoscopic appear ance with petechial hémorrhagies. All patients had nega tive rectal biopsies and patient No. 5 negative liver and kidney biopsies. Urinary cytology was negative in every patient. Transurethral ultrasound and bladder CT-scan re vealed a large, superficial tumor in the anterior wall of the bladder. No signs of deeper infiltration could be seen (fig- 1)Treatment and follow-up of the patients are listed in table 2. In 1 case (patient No. 1), the bladder amyloidosis with no tumor-like appearance was treated by electro-
Downloaded by: University of Exeter 144.173.6.94 - 6/17/2020 7:06:34 PM
Amyloidosis may be secondary to long-standing infec tions (tuberculosis, osteomyelitis) and inflammatory or immunologic disease (rheumatoid arthritis, Crohn’s dis ease). Localized amyloidosis of the bladder is a rare con dition. Until 1988, we have found reports of 54 patients with primary localized amyloidosis of the bladder [1-5]. Clinically important is that the symptoms of localized amyloidosis with macroscopic hematuria and the cystoscopic appearance may give rise to suspicion of bladder cancer. Here we present 5 patients with localized bladder amyloidosis of which 1 is of the secondary type.
Lehtonen/Mäkinen/Wikström
114
Fig. 1. a Intravesical ultrasonograph showing a large superficial tumor in the anterior wall of the bladder, b Contrast-enhanced blad der CT-scan reveals the same tumor with no infiltration outside the bladder.
Table 1. Clinical data of 5 patients with localized bladder amyloidosis Patient
Age years
Previous diseases
Urological symptoms
Cystoscopic findings
Site
EK
74
BPH
macroscopic hematuria, pollakiuria
reddish, bleeding areas, 1-2 cm in diameter
bladder dome posterior wall
VK
54
macroscopic hematuria
large tumor, 3-cm diameter
right lateral wall
VP
50
macroscopic hematuria, pollakiuria, bladder pain
tumors, 2.5-cm diameter
bladder dome
BW
71
macroscopic hematuria
tumor, 2.5 X 5 cm
anterior bladder wall
FU
67
macroscopic hematuria
hard, yellowish tumor, 3-cm diameter
right lateral wall
cystitis interstitialis
suppuration of osteo myelitis, pulmonary tbc
Table 2. Treatment and follow-up of patients with localized bladder amyloidosis Primary treatment
Treatment of recurrences
Follow-up time, years
EK VK VP BW FU
electrocoagulation bladder resection bladder resection TUR bladder resection
electrocoagulation X 3 electrocoagulation X 4; small residual tumors TUR and electrocoagulation once a year TUR X 1 ; bladder resection electrocoagulation X 1
1.5 5 22
2 4
exitus: cardiac infarction recurrent tumors; exitus: cardiac infarction small recurrent tumors no recurrences exitus: cardiac infarction Downloaded by: University of Exeter 144.173.6.94 - 6/17/2020 7:06:34 PM
Patient
Localized Amyloidosis of the Urinary Bladder
Discussion Amyloidosis can be classified into five different types according to etiology [6, 7]: (1) primary, without any evi dence of predisposing disease; (2) myeloma-associated; (3) secondary, occurring as a complication of a long standing infection, inflammation or immunologic dis ease (including tuberculosis, osteomyelitis, rheumatoid arthritis, inflammatory bowel diseases), or metabolic disease (diabetes mellitus); (4) localized, with involve ment of a single organ, and (5) primary heredofamilial. Localized amyloidosis of the bladder is quite rare; there are only 54 reports in the previous literature. According to Caldamone et al. [1], who analyzed the informations given in the literature, 6 of 44 patients had bladder amyloidosis secondary to systemic diseases. One of our 5 patients showed localized amyloidosis second ary to long-standing osteomyelitis and/or pulmonary tu berculosis without any evidence of amyloidosis in liver, kidney and rectum biopsies. Macroscopic hematuria with or without dysuria is the most common symptom of local bladder amyloidosis [8]. In our material, all the patients had macroscopic hema turia and 2 patients concomitant dysuric complaints. Macroscopic hematuria together with cystoscopic find ings aroused suspicion of bladder cancer. Cystoscopically the localized bladder amyloidosis is a yellowish, broad-based or polypoid tumor which can be
* €
Fig. 2. Microscopically normal epithelium of the urinary bladder biopsy. Large masses of amyloid just under the surface in the lamina propria (arrow), van Gieson. X 125.
single or multifocal [8]. One of our patients had a single amyloid tumor on the right anterior wall of the urinary bladder which was confirmed by transurethral ultra sound and bladder CT-scan. Another 3 patients, includ ing the patient with secondary amyloidosis, had single tumors at primary cystoscopy, but multiple, small recur rencies at follow-up. Cystoscopy of one patient with pri mary amyloidosis revealed several reddish hemorrhagic areas of 10-15 mm in diameter throughout the bladder, which is more characteristic of secondary amyloidosis [4]. The definitive diagnosis of amyloidosis is based on histopathological examinations with Congo red staining. In 4 patients including the one with secondary local amy loidosis of the bladder, the deposits of amyloidosis oc curred in the submucosa and in the muscularis, which is characteristic of primary localized amyloidosis [9], In 1 patient with a more diffuse macroscopic appearance of primary amyloidosis, mucosal ulcerations and vascular involvement of subendothelial vessels by amyloidosis were seen. This kind of histological appearance is more often seen in secondary vesical amyloidosis [10]. Transurethral resection and electrocoagulation is the treatment of choice in primary localized amyloidosis of the urinary bladder [1], Only in cases with single amyloid tumor partial cystectomy seemed to give more long standing results than in one of our patients. Because of the propensity to multiple recurrences, a follow-up with cystoscopy and repeated transurethral resections were necessary. The prognosis for patients with localized bladder amyloidosis is, however, good.
Downloaded by: University of Exeter 144.173.6.94 - 6/17/2020 7:06:34 PM
coagulations twice a year. In 3 patients, the primary treat ment was open-bladder resection. All of these patients had recurrence of small (1-1.5 cm in diameter) amyloid tumors in different parts of the bladder wall which were treated by electrocoagulations at 1- to 3-year intervals. In 1 patient (No. 4), the primary treatment was transure thral resection of the large amyloid tumor. After a 6month follow-up, a new TUR was performed in the tumor of the same site. Because of early recurrence 4 months after the second treatment an open bladder resec tion was performed. After this operation, no recurrence was noted in the 2-year follow-up. Three of our patients died of cardiac infarction. No autopsy was performed. Histologically, amyloidosis was diagnosed by staining the specimens with van Gieson’s and Congo red stains. The specimen of patient No. 1 showed subepithelial dif fuse perivascular amyloidosis deposits. In all other pa tients, large amyloidosis deposits were situated subepithelially and were present also within the muscularis (fig. 2).
115
Lehtonen/Mäkinen/Wikström
116
1 Caldamone AA, Elbadawi A, Moshtagi A, et al: Primary local ized amyloidosis of urinary bladder. Urology 1980; 15:174189. 2 Nakajima K, Hisazumi H, Okasyo A, et al: Primary localized amyloidosis of bladder. Urology 1980;15:302-303. 3 Mead MG, Hickinbotham P, Walls J: Amyloidosis localized to the bladder. Br J Urol 1982;54:428. 4 Ahmad E, Johansson SL, Fall M: Blue spotted bladder - a man ifestation of bladder amyloidosis. Scand J Urol Nephrol 1986; 20:145-147. 5 Grainger R, O’Riordan B, Cullen A, et al: Primary amyloidosis of lower urinary tract. Urology 1988;31:14-16. 6 Cohen AS: Amyloidosis. N Engl J Med 1967;277:574-583. 7 Kyle RA, Bayrd ED: Amyloidosis: Review of 236 cases. Medi cine 1975;54:271-299.
8 Malek RS, Laurence BS, Greene F, et al: Amyloidosis of the urinary bladder. Br J Urol 1971;43:189-200. 9 Strong GH, Kelsey D, Hoch W: Primary amyloid disease of the bladder. J Urol 1974; 112:463-466. 10 Au KK, Gilbaugh JH Jr: Primary amyloidosis of the bladder. J Urol 1975;114:786-787.
T. Lehtonen Department of Urology Helsinki University Haartmaninkatu 4 SF-00290 Helsinki (Finland)
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References
