British Journal of Urology (197% 41, 401-404 0
Malignant Phaeochromocytoma of the Urinary Bladder-Report of Two Cases P. JAVAHERI and J. RAAFAT
The Departments of Surgery and Internal Medicine, Pahlavi University, School of Medicine, Shiraz, Iran
Malignant phaeochromocytoma of the urinary bladder is an extremely rare occurrence, only 4 patients so far having been reported. Two new cases have been observed here at the Pahlavi University. Pelvic angiography was performed for the first time in this condition. Case Reports Case 1. G. A., a 44-year-old white male was admitted to Nemazee Hospital, Pahlavi University on 2nd October with a history of painless haematuria of 2 months duration. Physical examination revealed blood pressure of 180/100. IVP demonstrated a large filling defect in the bladder (Fig. 1). Pelvic angiography indicated innumerable pathological vessels and tumour stain (Fig. 2, A and B). Cystoscopy revealed a nodular tumour in the dome of the bladder 2 x 2 cm in diameter. At operation a large vesical tumour was found. Interestingly enough, the major part of this mass extended outside the bladder wall. Partial cystectomy and resection of two large lymph nodes along the external iliac artery were performed. Histological examination of the bladder tumour and the resected lymph nodes disclosed phaeochromocytoma. Postoperatively, the BP returned to I20/70 and remained at that level for 3 months. He was lost to follow-up and a year later died suddenly.
Fig. 1. IVP. Case 1 : large filling defect in the bladder.
Case 2. S. M., a 24-year-old female known to be hypertensive for 3 years was admitted to Pahlavi University, Saadi Hospital on 7th March 1973. She complained of headache, dizziness, palpitation, shortness of breath, profuse sweating, hot flushing, nausea and vomiting. These symptoms occurred primarily during the last month of each of the last 3 pregnancies during the last 3 years. Following her last delivery (40 days prior to admission), the clinical symptoms became persistent and were particularly aggravated at the time of urination. She had 2 episodes of fainting while urinating. Her blood pressure ranged between 150/110 to 250/130. Pelvic examination disclosed the presence of an orange-size tumour. Bimanual palpation of this mass caused sudden increase of blood pressure and rapid pulse rate. Urinalysis was normal and 24 hours excretion of urinary vanillyl mandelic acid was 18.6 mg. IVP presented medial deviation of the left ureter suggestive of an extrinsic mass laterally (Fig. 3). Cystoscopy demonstrated intact bladder mucosa with no gross pathology inside.
401
402
BRITISH JOURNAL OF UROLOGY
Fig. 2. Pelvic angiography. Case 1 : demonstrating pathologic tumour vessels (A) and tumour stain (B).
Fig. 3. IVP. Case 2: medial deviation of the left ureter suggestive of an extrinsic mass laterally.
MALIGNANT PHAEOCHROMOCYTOMA OF THE URINARY BLADDER
403
Both adrenal glands appeared normal on exploration. The palpated pelvic tumour arose from the external surface of the bladder without invasion of the vesical mucosa. Partial cystectomy along with resection of 2 enlarged adjacent lymph nodes were performed. Histological examination of the resected mass and the lymph nodes disclosed phaeochromocytoma. Her clinical symptoms completely subsided after the operation and her BP returned to 110/70.She is now on her I-year follow-up and has remained normotensive and asymptomatic.
Discussion
Since Zimmerman, Biron and MacMahon (1953) presented the first case of phaeochromocytoma of the urinary bladder, 38 cases have been reported in the literature, (Cabanas, Faulconer and Fekete, 1973). Four of these 38 cases have been malignant. The first malignant case, (Pugh, Gresham and Mullaney, 1969) was a 36-year-old female who died shortly after cystoscopy and a t autopsy she was found to have phaeochromocytoma of the bladder with metastasis to the regional lymph nodes and the liver. The second case (Higgins and Tresidder, 1966) was a 14-year-old boy with haematuria and sustained hypertension. Partial cystectomy and resection of metastatic regional lymph nodes were performed. The patient was known to be normotensive and doing well at 9 months after operation. The third malignant case (Moloney, Cowdell and Lewis, 1966) was a 26-year-old male with metastasis to lymph nodes, lungs and bones. Partial cystectomy was done; but he died 4 years after the treatment. The fourth patient (Scott and Eversole, 1960) was a 14-year-old white boy who had sustained mild hypertension. Total cystectomy, prostatectomy, seminal vesiculectomy and urinary diversion were performed. A regional lymph node was invaded with the same type of tumour. The patient was known to be alive and well 14 months after surgery. Our 2 cases bring the total number of malignant cases of phaeochromocytoma of the bladder t o 6. The only evidence of malignancy in this disease is the presence of metastasis because histological changes and tissue infiltration also may be seen in tumours with quite benign behaviour (Doctor, Phadke and Sirsat, 1972). Vesical phaeochromocytoma originates from the paraganglia related to autonomic nerves which are mainly located in the muscular layer of the bladder wall, (Bourne and Beltaos, 1967).The mucosal layer may look intact in this type of bladder tumour. Cystoscopy may or may not reveal the presence of tumour. In our first case cystoscopy revealed the mass while in the second case cystoscopy failed to show any pathology. Tumours of phaeochromocytoma arise from chromaffin tissue and in 10% of the cases are multifocal (Higgins and Tresidder, 1966). Multifocal tumours should not be mistaken for metastasis. The tumour is defined as multifocal if, besides the original tumour, another mass is located at sites where chromaffin tissue naturally is present. The metastasis of phaeochromocytoma invades tissues that normally do not have chromaffin elements. Partial cystectomy is the recommended type of treatment. Since the tumour is situated between the muscular fibres of bladder wall and does not necessarily invade the mucosal layers, transurethral resection does not seem to be the satisfactory treatment. Radiation has been used in the treatment of malignant phaeochromocytoma (Higgins and Tresidder, 1966). It is too early to judge the effectiveness of this therapy. Summary
2 new patients with malignant phaeochromocytoma of the urinary bladder were presented. 4 other cases with the same disease reported in literature were reviewed and discussed. References BOURNE, R. B. and BELTAOS, E. (1967). Pheochromocytoma of the bladder. Case report and summary of literature. Journal of UroloEy, 98, 361-364.
404
BRlTlSH JOURNAL OF UROLOGY
CABANAS, V. Y., FAULCONER, R. J. and FEKETE, A. M. (1973). Pheochromocytoma presenting as ureterocele. Journal of Urology, 110, 389-390. DOCTOR,V. M., PHADKE, A. G. and SIRSAT,M. V. (1972). Pheochromocytoma of the urinary bladder. British Journal of Urology, 44, 351-355. HIGGINS. P. M. and TRESIDDER, G. C. (1966). Phaeochromocytoma of the urinary bladder. British Medical Journal, 2,274211.
MOLONEY, G . E.. COWDELL, R. H. and LEwrs, C. L. (1966). Malignant phaeochromocytoma of the bladder. British Journal of Urology, 38, 461-470. PUGH,R. C. B., GRESHAM, G. A. and MULLANEY, J. (1960). Pheochromocytoma of the urinary bladder. Journal of Pathology and Bacteriology, 79, 89-107. SCOTT, W.W . and EVERSOLE, S. L. (1960). Pheochromocytoma of the urinary bladder. Journal of Urology, 83, 656-664.
ZrMMERMAN, I. J., BIRON,R. E. and MACMAHON, H. E. (1953). Pheochrornocytoma of the urinary bladder. New England Journal of Medicine, 25-26,249.
The Authors Parviz Javaheri, MD, Assistant Professor of Surgery. J. Raafat, MD, Senior Resident in Medicine. Address for reprints: Parviz Javaheri, MD, Nemazee Hospital, Shiraz, Iran.
Malignant Phaeochromocytoma of the Urinary Bladder-Report of Two Cases P. JAVAHERI and J. RAAFAT
The Departments of Surgery and Internal Medicine, Pahlavi University, School of Medicine, Shiraz, Iran
Malignant phaeochromocytoma of the urinary bladder is an extremely rare occurrence, only 4 patients so far having been reported. Two new cases have been observed here at the Pahlavi University. Pelvic angiography was performed for the first time in this condition. Case Reports Case 1. G. A., a 44-year-old white male was admitted to Nemazee Hospital, Pahlavi University on 2nd October with a history of painless haematuria of 2 months duration. Physical examination revealed blood pressure of 180/100. IVP demonstrated a large filling defect in the bladder (Fig. 1). Pelvic angiography indicated innumerable pathological vessels and tumour stain (Fig. 2, A and B). Cystoscopy revealed a nodular tumour in the dome of the bladder 2 x 2 cm in diameter. At operation a large vesical tumour was found. Interestingly enough, the major part of this mass extended outside the bladder wall. Partial cystectomy and resection of two large lymph nodes along the external iliac artery were performed. Histological examination of the bladder tumour and the resected lymph nodes disclosed phaeochromocytoma. Postoperatively, the BP returned to I20/70 and remained at that level for 3 months. He was lost to follow-up and a year later died suddenly.
Fig. 1. IVP. Case 1 : large filling defect in the bladder.
Case 2. S. M., a 24-year-old female known to be hypertensive for 3 years was admitted to Pahlavi University, Saadi Hospital on 7th March 1973. She complained of headache, dizziness, palpitation, shortness of breath, profuse sweating, hot flushing, nausea and vomiting. These symptoms occurred primarily during the last month of each of the last 3 pregnancies during the last 3 years. Following her last delivery (40 days prior to admission), the clinical symptoms became persistent and were particularly aggravated at the time of urination. She had 2 episodes of fainting while urinating. Her blood pressure ranged between 150/110 to 250/130. Pelvic examination disclosed the presence of an orange-size tumour. Bimanual palpation of this mass caused sudden increase of blood pressure and rapid pulse rate. Urinalysis was normal and 24 hours excretion of urinary vanillyl mandelic acid was 18.6 mg. IVP presented medial deviation of the left ureter suggestive of an extrinsic mass laterally (Fig. 3). Cystoscopy demonstrated intact bladder mucosa with no gross pathology inside.
401
402
BRITISH JOURNAL OF UROLOGY
Fig. 2. Pelvic angiography. Case 1 : demonstrating pathologic tumour vessels (A) and tumour stain (B).
Fig. 3. IVP. Case 2: medial deviation of the left ureter suggestive of an extrinsic mass laterally.
MALIGNANT PHAEOCHROMOCYTOMA OF THE URINARY BLADDER
403
Both adrenal glands appeared normal on exploration. The palpated pelvic tumour arose from the external surface of the bladder without invasion of the vesical mucosa. Partial cystectomy along with resection of 2 enlarged adjacent lymph nodes were performed. Histological examination of the resected mass and the lymph nodes disclosed phaeochromocytoma. Her clinical symptoms completely subsided after the operation and her BP returned to 110/70.She is now on her I-year follow-up and has remained normotensive and asymptomatic.
Discussion
Since Zimmerman, Biron and MacMahon (1953) presented the first case of phaeochromocytoma of the urinary bladder, 38 cases have been reported in the literature, (Cabanas, Faulconer and Fekete, 1973). Four of these 38 cases have been malignant. The first malignant case, (Pugh, Gresham and Mullaney, 1969) was a 36-year-old female who died shortly after cystoscopy and a t autopsy she was found to have phaeochromocytoma of the bladder with metastasis to the regional lymph nodes and the liver. The second case (Higgins and Tresidder, 1966) was a 14-year-old boy with haematuria and sustained hypertension. Partial cystectomy and resection of metastatic regional lymph nodes were performed. The patient was known to be normotensive and doing well at 9 months after operation. The third malignant case (Moloney, Cowdell and Lewis, 1966) was a 26-year-old male with metastasis to lymph nodes, lungs and bones. Partial cystectomy was done; but he died 4 years after the treatment. The fourth patient (Scott and Eversole, 1960) was a 14-year-old white boy who had sustained mild hypertension. Total cystectomy, prostatectomy, seminal vesiculectomy and urinary diversion were performed. A regional lymph node was invaded with the same type of tumour. The patient was known to be alive and well 14 months after surgery. Our 2 cases bring the total number of malignant cases of phaeochromocytoma of the bladder t o 6. The only evidence of malignancy in this disease is the presence of metastasis because histological changes and tissue infiltration also may be seen in tumours with quite benign behaviour (Doctor, Phadke and Sirsat, 1972). Vesical phaeochromocytoma originates from the paraganglia related to autonomic nerves which are mainly located in the muscular layer of the bladder wall, (Bourne and Beltaos, 1967).The mucosal layer may look intact in this type of bladder tumour. Cystoscopy may or may not reveal the presence of tumour. In our first case cystoscopy revealed the mass while in the second case cystoscopy failed to show any pathology. Tumours of phaeochromocytoma arise from chromaffin tissue and in 10% of the cases are multifocal (Higgins and Tresidder, 1966). Multifocal tumours should not be mistaken for metastasis. The tumour is defined as multifocal if, besides the original tumour, another mass is located at sites where chromaffin tissue naturally is present. The metastasis of phaeochromocytoma invades tissues that normally do not have chromaffin elements. Partial cystectomy is the recommended type of treatment. Since the tumour is situated between the muscular fibres of bladder wall and does not necessarily invade the mucosal layers, transurethral resection does not seem to be the satisfactory treatment. Radiation has been used in the treatment of malignant phaeochromocytoma (Higgins and Tresidder, 1966). It is too early to judge the effectiveness of this therapy. Summary
2 new patients with malignant phaeochromocytoma of the urinary bladder were presented. 4 other cases with the same disease reported in literature were reviewed and discussed. References BOURNE, R. B. and BELTAOS, E. (1967). Pheochromocytoma of the bladder. Case report and summary of literature. Journal of UroloEy, 98, 361-364.
404
BRlTlSH JOURNAL OF UROLOGY
CABANAS, V. Y., FAULCONER, R. J. and FEKETE, A. M. (1973). Pheochromocytoma presenting as ureterocele. Journal of Urology, 110, 389-390. DOCTOR,V. M., PHADKE, A. G. and SIRSAT,M. V. (1972). Pheochromocytoma of the urinary bladder. British Journal of Urology, 44, 351-355. HIGGINS. P. M. and TRESIDDER, G. C. (1966). Phaeochromocytoma of the urinary bladder. British Medical Journal, 2,274211.
MOLONEY, G . E.. COWDELL, R. H. and LEwrs, C. L. (1966). Malignant phaeochromocytoma of the bladder. British Journal of Urology, 38, 461-470. PUGH,R. C. B., GRESHAM, G. A. and MULLANEY, J. (1960). Pheochromocytoma of the urinary bladder. Journal of Pathology and Bacteriology, 79, 89-107. SCOTT, W.W . and EVERSOLE, S. L. (1960). Pheochromocytoma of the urinary bladder. Journal of Urology, 83, 656-664.
ZrMMERMAN, I. J., BIRON,R. E. and MACMAHON, H. E. (1953). Pheochrornocytoma of the urinary bladder. New England Journal of Medicine, 25-26,249.
The Authors Parviz Javaheri, MD, Assistant Professor of Surgery. J. Raafat, MD, Senior Resident in Medicine. Address for reprints: Parviz Javaheri, MD, Nemazee Hospital, Shiraz, Iran.
