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Paraganglioma of urethra
PARAGANGLIOMA OF URETHRA ROBERT A. BADALAMENT, M.D. PAUL KENWORTHY, M.D. ARTHUR PELLEGRINI, M.D. JOSEPH R. DRAGO, M.D.
From the Department of Surgery, Division of Urology and Department of Pathology, The Ohio State University, Columbus, Ohio
ABSTRACT--Paraganglioma of the urethra is a rare tumor; only 4 cases have been reported previously. We report a fifth case of urethral paraganglioma and review the literature.
Approximately 10 percent of pheochromocytomas involve extra-adrenal sites. 1 Extra-adrenal pheochromocytomas or paragangliomas most commonly involve the organs of Zuckerkandl and paraganglia of the neck, thorax, or abdomen. 2 Additionally, paragangliomas may infrequently involve the urinary tract. In 1953, Zimmerman, Biron, and MacMahon ~ reported the first case of paraganglioma of the urinary bladder. Currently, 119 cases of bladder paraganglioma have been reported2; the estimated incidence is 0.06 percent. 4 In 1979, Mehta and associates5 described the first patient with paraganglioma of the prostatic urethra, which in this case was metastatic to the retroperitoneum. In 1983 Altavilla, Cavazzini, and Russo ~ reported the second case of paraganglioma of the prostatic urethra. Several months later, a case of a pendulous urethral paraganglioma was reported by Bryant and coworkers. 7 Recently, Dennis et al. 8 reported a case of a prostatic paraganglioma which was diagnosed preoperatively and successfully managed by radical retropubic prostateetomy. Our extensive search and personal review of the literature did not identify additional case reports. Herein, we report a fifth case of urethral paraganglioma and review the previously reported cases.
76
.Case Report A seventy-two-year-old white man to the Ohio State University urology ~ symptoms of bladder outlet obstructi ing of urinary frequency (voiding eve utes), decreased force and caliber. stream, nocturia (five times nightly), tion of incomplete bladder emptyin~ symptoms had progressed since a rig] hemisphere stroke three months e though the stroke produced left uppel weakness and a fearful affect, no otl lae were apparent. The remainder medical history was noncontributory hypertension which was well contr atenolol. On physical examination the p~ n o r m o t e n s i v e and had unremar] dominal and genital examinations. rectal examination, sphincter tone w the prostate was symmmetrically enl indurated, and measured approxima cm. Cystometrogram was performed small-capacity, hypertonic bladder void residual volume of 100 mL. ( revealed a pedunculated urethral m from the prostatic urethra. Additk structive lateral lobe e n l a r g e m e
UROLOGY
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JULY 1991
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V O L U M E XXXVII
required. The combination of surgery and oxybutynin resulted in a moderate subjective improvement in his voiding symptoms.
elicate fibrovascular stroma (hematoxylin original magnification x 100).
and moderately severe bladder trans were noted. ~fter discontinuation of oral anticoagulants, !~atient underwent outpatient transurethral i~)val of the urethral polyp and ineisional atotomy. The stalk of polyp was grasped old cup biopsy forceps and extracted; i}!)2seewasthen fulgurated with Neodymium ium aluminum garnet (nd-YAG) laser. The [ent was sent home with a urethral catheter eh he removed twenty-four hours later. ilraganglioma was diagnosed on histologie ~ination. Grossly, the nonfixed specimen ialed a pink smooth polypoid mass measuril.1 by 0.5 by 0.4 era. Microscopically, the !i)r was composed of zellballen or nests of iwith round uniform nuelei separated by a ,'ular stroma (Fig. 1). The cells granular cytoplasm and rare ,'sent. The neoplasm was eovml cell mueosa. Numerous imstudies were p e r f o r m e d . enolase showed a diffuse inpositivity, ehromogranin demse strong positivity, S-100 positive cells, and pan-keratin asional positive cell. HHF-35 n), prostatic specific antigen, ,'id phosphatase studies were :postoperatively, the patient ~e of the urethral lesion at eys~tion, and urinary eateeholadeterminations were normal. ~ertension remained well cone modification of atenolol was
1991
/
VOLUME XXXVIII, NUMBER 1
Comment Coupland 9'1° demonstrated that the distribution of paraganglia is more widespread in the fetus and infant than in the adult. He described numerous paraganglia in association with sympathetic nerve fibers in the bladder, perivesical region, and prostate of a newborn. However, only 1 case of a normal paraganglion occurring in the prostate of an adult has been reported, u A single normal paraganglion was discovered incidentally in a prostate fragment of an eightynine-year-old white man undergoing transurethral prostatectomy for benign hyperplasia. Other cell types of neural crest origin have been reported to occur within prostate tissue. Argentaffin and argyrophil cells~2,~3 as well as eareinoid tumors of the prostate have been identified.13-15 The patient described by Mehta and assoeiates 5 was a twenty-nine-year-old man who presented with pitting edema of the right leg and asymmetric enlargement of the right lobe of the prostate. Transrectal needle biopsy of the prostate produced a specimen that was identified as a primary neural tumor. At surgical exploration the patient had a large pelvic mass, encasing the right ureter and adjacent pelvic vessels, with prominent eontralateral pelvic lymphadenopathy. A wedge biopsy specimen was interpreted as a malignant paraganglioma. The patient was treated with cyclic chemotherapy (consisting of vincristine, eyclophosphamide, doxorubicin and actinomyein D) for eighteen months. At twenty-four months following surgery, his disease remained stable. A1tavilla et al. 6 questioned if this report represented a primary malignant prostatic urethral paraganglioma since "no clear evidence of its prostatic origin was given." In the case reported by Altavilla and coworkers, 6 a sixty-three-year-old white man presented with urinary retention and hypertension. Twenty-four-hour urinary excretion of normetanephrine, metanephrine, and vanillylmandelic acid w e r e slightly i n c r e a s e d preoperatively. During the one year following transbladder resection of the prostate, blood pressure determinations and urinary catecholamine metabolites remained normal. Bryant and colleagues7 described a case involving a sixty-seven-year-old white man who
77
presented with nonobstructive prostatic asymmetry and hypertension, which was well controlled by medical management. The patient underwent endoscopic removal of a 1-cm urethral polyp originating from the ventral portion of the anterior urethra near the penoscrotal junction. Additionally, needle biopsy of the prostate was performed and demonstrated benign hyperplasia. In the six-month period of follow-up, the patient had no cystoseopie evidence of recurrence, and his hypertension remained essentially unchanged. Dennis and associates 8 described a thirtyfive-year-old white man with a history of mild untreated hypertension, which was exacerbated by abdominal straining and urination. Urinary cateeholamine metabolite determinations revealed an elevated vanillylmandelie acid and norepinephrine. Although digital rectal examination of the prostate was normal,laliodinemeta-iodobenzylguanidin scan, computerized tomography, magnetic resonance imaging, and pelvic angiography confirmed the presence of a prostatic mass. Following preoperative alphaadrenergic blockade, a radical retropubic prostatectomy and bilateral pelvic lymph node dissection were p e r f o r m e d . The p a t h o l o g i c examination showed the paraganglioma was confined to the prostate. Postoperatively, the patient's hypertension resolved, and he remains free of disease one year after prostatectomy. The data c o n c e r n i n g u r e t h r a l paragangliomas are too limited to draw definitive conclusions concerning optimal management. However, the reported experience with bladder paragangliomas may be extrapolated into this clinical setting. The only reliable criterion of malignancy is the presence of metastases. Of the 119 eases of bladder paragangliomas, malignant behavior was noted in 16 patients (13 %).2 This may represent the minimum rate of malignancy, since most reported cases have
78
relatively short follow-up. Patients with malig~ nant bladder paragangliomas tended to be younger; three-fourths were younger than forty years old. 2 Thus, we advocate that urethrai paragangliomas be managed with extended foi: low-up, including serial blood pressure deter~ minations, catecholamine metabolite deter~i minations, and cystoseopy. 456 West Tenth Avenue'! Columbus, Ohio 432101
/DR. DRAG0!
References 1. Rossi P, Young IS, and Pante WS: Techniques, usefulne,. and hazards of arteriography of pheochromocytomas, review 99 cases, JAMA 205:75 (1968). 2. Peterson RO: Urinary bladder, miscellaneous neoplast pheochromoeytomas, in Biello LA, Robinson S, and Barishek VN (Eds) Urologic Pathology, Philadelphia, JB Lippincott Co. chaI 4, 1986, pp 373-377. 3. Zimmerman IJ, Biron RE, and MaeMahon HE: Pheoehrti~ mocytoma of the urinary bladder, N Engl J Med 249:25 (1953)i( 4. Leestma JE, and Price EB Jr Paraganglioma of the urinar~ bladder, Cancer 28:1063 (1971). 5. Mehta M, Nadel NS, Lonni Y, and Ali h Malignant para~ ganglioma of the prostate and retroperitoneum, J Urol 121: 37~
(1979). 6. Altavilla G, Cavazzini L, and Russo R: Secreting benig~ paraganglioma of the prostatic urethra, Tumori 69:79 (1983); ~i 7. Bryant KR, Thompson IM, Ortiz R, and Spence C~t Urethral paraganglioma presenting as a urethral polyp, I Ur~ 130:571 (1983). ~i 8. Dennis PJ, et al: Phe0chromocytoma of the prostate: an u!l usual location, J Urol 141:130 (1989). :!! 9. Coupland RE: Prenatal development of abdominal par aortic bodies in man, J Anat 86:357 (1952). 10. Coupland RE: Post-natal fate of abdominal para-aori bodies in man, J Anat 88:455 (1954). 11. Freedman SR, and Goldman RL: Normal paragangliaii the human prostate, J Urol 113:874 (1975). 12. Kazzas BA: Argentaffin and argyrophil ceils in the pr~ rate, J Pathol 12:189 (1974). 13. Capella C, et al: The endocrine component of prostaf carcinomas mixed adenoearcinomas-earcinoid tumors and n0i tumor prostate. Histochemical and ultrastruetural identificatic of endocrine cells, Histopathology 5:175 (1981). 14. Sylora HO, et al: Primary carcinoid of the urethra, J rJr~ 114:150 (1975). 15. Wasserstein PW, and Goldman RL: Primary carcinoidi the prostate, Urology 13:318 (1979).
UROLOGY
/
JULY 1991
/
VOLUME XXXVIII, NUMB~ I
From the Department of Surgery, Division of Urology and Department of Pathology, The Ohio State University, Columbus, Ohio
ABSTRACT--Paraganglioma of the urethra is a rare tumor; only 4 cases have been reported previously. We report a fifth case of urethral paraganglioma and review the literature.
Approximately 10 percent of pheochromocytomas involve extra-adrenal sites. 1 Extra-adrenal pheochromocytomas or paragangliomas most commonly involve the organs of Zuckerkandl and paraganglia of the neck, thorax, or abdomen. 2 Additionally, paragangliomas may infrequently involve the urinary tract. In 1953, Zimmerman, Biron, and MacMahon ~ reported the first case of paraganglioma of the urinary bladder. Currently, 119 cases of bladder paraganglioma have been reported2; the estimated incidence is 0.06 percent. 4 In 1979, Mehta and associates5 described the first patient with paraganglioma of the prostatic urethra, which in this case was metastatic to the retroperitoneum. In 1983 Altavilla, Cavazzini, and Russo ~ reported the second case of paraganglioma of the prostatic urethra. Several months later, a case of a pendulous urethral paraganglioma was reported by Bryant and coworkers. 7 Recently, Dennis et al. 8 reported a case of a prostatic paraganglioma which was diagnosed preoperatively and successfully managed by radical retropubic prostateetomy. Our extensive search and personal review of the literature did not identify additional case reports. Herein, we report a fifth case of urethral paraganglioma and review the previously reported cases.
76
.Case Report A seventy-two-year-old white man to the Ohio State University urology ~ symptoms of bladder outlet obstructi ing of urinary frequency (voiding eve utes), decreased force and caliber. stream, nocturia (five times nightly), tion of incomplete bladder emptyin~ symptoms had progressed since a rig] hemisphere stroke three months e though the stroke produced left uppel weakness and a fearful affect, no otl lae were apparent. The remainder medical history was noncontributory hypertension which was well contr atenolol. On physical examination the p~ n o r m o t e n s i v e and had unremar] dominal and genital examinations. rectal examination, sphincter tone w the prostate was symmmetrically enl indurated, and measured approxima cm. Cystometrogram was performed small-capacity, hypertonic bladder void residual volume of 100 mL. ( revealed a pedunculated urethral m from the prostatic urethra. Additk structive lateral lobe e n l a r g e m e
UROLOGY
/
JULY 1991
/
V O L U M E XXXVII
required. The combination of surgery and oxybutynin resulted in a moderate subjective improvement in his voiding symptoms.
elicate fibrovascular stroma (hematoxylin original magnification x 100).
and moderately severe bladder trans were noted. ~fter discontinuation of oral anticoagulants, !~atient underwent outpatient transurethral i~)val of the urethral polyp and ineisional atotomy. The stalk of polyp was grasped old cup biopsy forceps and extracted; i}!)2seewasthen fulgurated with Neodymium ium aluminum garnet (nd-YAG) laser. The [ent was sent home with a urethral catheter eh he removed twenty-four hours later. ilraganglioma was diagnosed on histologie ~ination. Grossly, the nonfixed specimen ialed a pink smooth polypoid mass measuril.1 by 0.5 by 0.4 era. Microscopically, the !i)r was composed of zellballen or nests of iwith round uniform nuelei separated by a ,'ular stroma (Fig. 1). The cells granular cytoplasm and rare ,'sent. The neoplasm was eovml cell mueosa. Numerous imstudies were p e r f o r m e d . enolase showed a diffuse inpositivity, ehromogranin demse strong positivity, S-100 positive cells, and pan-keratin asional positive cell. HHF-35 n), prostatic specific antigen, ,'id phosphatase studies were :postoperatively, the patient ~e of the urethral lesion at eys~tion, and urinary eateeholadeterminations were normal. ~ertension remained well cone modification of atenolol was
1991
/
VOLUME XXXVIII, NUMBER 1
Comment Coupland 9'1° demonstrated that the distribution of paraganglia is more widespread in the fetus and infant than in the adult. He described numerous paraganglia in association with sympathetic nerve fibers in the bladder, perivesical region, and prostate of a newborn. However, only 1 case of a normal paraganglion occurring in the prostate of an adult has been reported, u A single normal paraganglion was discovered incidentally in a prostate fragment of an eightynine-year-old white man undergoing transurethral prostatectomy for benign hyperplasia. Other cell types of neural crest origin have been reported to occur within prostate tissue. Argentaffin and argyrophil cells~2,~3 as well as eareinoid tumors of the prostate have been identified.13-15 The patient described by Mehta and assoeiates 5 was a twenty-nine-year-old man who presented with pitting edema of the right leg and asymmetric enlargement of the right lobe of the prostate. Transrectal needle biopsy of the prostate produced a specimen that was identified as a primary neural tumor. At surgical exploration the patient had a large pelvic mass, encasing the right ureter and adjacent pelvic vessels, with prominent eontralateral pelvic lymphadenopathy. A wedge biopsy specimen was interpreted as a malignant paraganglioma. The patient was treated with cyclic chemotherapy (consisting of vincristine, eyclophosphamide, doxorubicin and actinomyein D) for eighteen months. At twenty-four months following surgery, his disease remained stable. A1tavilla et al. 6 questioned if this report represented a primary malignant prostatic urethral paraganglioma since "no clear evidence of its prostatic origin was given." In the case reported by Altavilla and coworkers, 6 a sixty-three-year-old white man presented with urinary retention and hypertension. Twenty-four-hour urinary excretion of normetanephrine, metanephrine, and vanillylmandelic acid w e r e slightly i n c r e a s e d preoperatively. During the one year following transbladder resection of the prostate, blood pressure determinations and urinary catecholamine metabolites remained normal. Bryant and colleagues7 described a case involving a sixty-seven-year-old white man who
77
presented with nonobstructive prostatic asymmetry and hypertension, which was well controlled by medical management. The patient underwent endoscopic removal of a 1-cm urethral polyp originating from the ventral portion of the anterior urethra near the penoscrotal junction. Additionally, needle biopsy of the prostate was performed and demonstrated benign hyperplasia. In the six-month period of follow-up, the patient had no cystoseopie evidence of recurrence, and his hypertension remained essentially unchanged. Dennis and associates 8 described a thirtyfive-year-old white man with a history of mild untreated hypertension, which was exacerbated by abdominal straining and urination. Urinary cateeholamine metabolite determinations revealed an elevated vanillylmandelie acid and norepinephrine. Although digital rectal examination of the prostate was normal,laliodinemeta-iodobenzylguanidin scan, computerized tomography, magnetic resonance imaging, and pelvic angiography confirmed the presence of a prostatic mass. Following preoperative alphaadrenergic blockade, a radical retropubic prostatectomy and bilateral pelvic lymph node dissection were p e r f o r m e d . The p a t h o l o g i c examination showed the paraganglioma was confined to the prostate. Postoperatively, the patient's hypertension resolved, and he remains free of disease one year after prostatectomy. The data c o n c e r n i n g u r e t h r a l paragangliomas are too limited to draw definitive conclusions concerning optimal management. However, the reported experience with bladder paragangliomas may be extrapolated into this clinical setting. The only reliable criterion of malignancy is the presence of metastases. Of the 119 eases of bladder paragangliomas, malignant behavior was noted in 16 patients (13 %).2 This may represent the minimum rate of malignancy, since most reported cases have
78
relatively short follow-up. Patients with malig~ nant bladder paragangliomas tended to be younger; three-fourths were younger than forty years old. 2 Thus, we advocate that urethrai paragangliomas be managed with extended foi: low-up, including serial blood pressure deter~ minations, catecholamine metabolite deter~i minations, and cystoseopy. 456 West Tenth Avenue'! Columbus, Ohio 432101
/DR. DRAG0!
References 1. Rossi P, Young IS, and Pante WS: Techniques, usefulne,. and hazards of arteriography of pheochromocytomas, review 99 cases, JAMA 205:75 (1968). 2. Peterson RO: Urinary bladder, miscellaneous neoplast pheochromoeytomas, in Biello LA, Robinson S, and Barishek VN (Eds) Urologic Pathology, Philadelphia, JB Lippincott Co. chaI 4, 1986, pp 373-377. 3. Zimmerman IJ, Biron RE, and MaeMahon HE: Pheoehrti~ mocytoma of the urinary bladder, N Engl J Med 249:25 (1953)i( 4. Leestma JE, and Price EB Jr Paraganglioma of the urinar~ bladder, Cancer 28:1063 (1971). 5. Mehta M, Nadel NS, Lonni Y, and Ali h Malignant para~ ganglioma of the prostate and retroperitoneum, J Urol 121: 37~
(1979). 6. Altavilla G, Cavazzini L, and Russo R: Secreting benig~ paraganglioma of the prostatic urethra, Tumori 69:79 (1983); ~i 7. Bryant KR, Thompson IM, Ortiz R, and Spence C~t Urethral paraganglioma presenting as a urethral polyp, I Ur~ 130:571 (1983). ~i 8. Dennis PJ, et al: Phe0chromocytoma of the prostate: an u!l usual location, J Urol 141:130 (1989). :!! 9. Coupland RE: Prenatal development of abdominal par aortic bodies in man, J Anat 86:357 (1952). 10. Coupland RE: Post-natal fate of abdominal para-aori bodies in man, J Anat 88:455 (1954). 11. Freedman SR, and Goldman RL: Normal paragangliaii the human prostate, J Urol 113:874 (1975). 12. Kazzas BA: Argentaffin and argyrophil ceils in the pr~ rate, J Pathol 12:189 (1974). 13. Capella C, et al: The endocrine component of prostaf carcinomas mixed adenoearcinomas-earcinoid tumors and n0i tumor prostate. Histochemical and ultrastruetural identificatic of endocrine cells, Histopathology 5:175 (1981). 14. Sylora HO, et al: Primary carcinoid of the urethra, J rJr~ 114:150 (1975). 15. Wasserstein PW, and Goldman RL: Primary carcinoidi the prostate, Urology 13:318 (1979).
UROLOGY
/
JULY 1991
/
VOLUME XXXVIII, NUMB~ I