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Seminal vesicle cyst and ipsilateral renal agenesis
SEMINAL
VESICLE
IPSILATERAL ROBERT NORMAN
CYST AND
RENAL
E. DONOHUE,
AGENESIS
M.D.”
F. GREENSLADE,
From the Department of Urology, Christchurch, New Zealand
F.R.C.S.,
F.R.A.C.S.
Christchurch
Hospital,
ABSTRACT-A case of seminal vesicle cyst and ipsilateral renal agenesis is presented, the literature summarized, the embryology reviewed, and the diferential diagnosis discussed. Our reasons for surgical therapy are stated.
The differential diagnosis of a paraprostatic mass is always challenging. It involves a detailed knowledge of the embryology of the genitourinary tract. A patient with such a mass is described, the embryology of the area presented, and the world literature reviewed. Case Report A forty-one-year-old farmer, in excellent health, noted the urge to void at 9:00 A.M. but was unable to urinate. At noon, he was still unable to void. At 4:00 P.M. he was in a great deal of distress and began passing small amounts of urine incontinently. At 6:00 P.M. he spontaneously passed thick bloody urine per urethra. There were no clots; he denied any pain or burning. A sample of the fluid was collected, and he visited his doctor who confirmed the presence of blood and placed him on sulfisoxazole (Gantrisin) for ten days. His urine became clear over the next three voidings; all of which were initiated without difficulty. Ten days later at a return visit he was referred .to a urologist. He had undergone appendectomy twenty years before for acute appendicitis. He denied any history of urinary tract disease or symptoms preceding the episode of hematuria.
*Present address: University of Colorado Medical Center, Division of Urology, 4200 East Ninth Avenue, Denver, Colorado 80220.
66
His sexual function was normal; his ejacuIate He is the father of two was never bloody. children. On physical examination his temperature was respiration 18, pulse SO, and 98.6”F. (37°C.) blood pressure 130190 mm. Hg. The only positive physical findings were a healed appendectomy scar in the right lower quadrant and a large paraprostatic cystic mass on the right, slightly above the prostate, nonmovable and nontender. The penis and urethral meatus were normal, and the scrotal examination revealed normal testis, epididymis, and vas bilaterally. The prostate felt normal in size and consistency. Laboratory data were as follows: blood count, 4,000 per cubic millimeter; hemoglobin, 15.5 Gm. per 100 ml.; hematocrit, 45; urinalysis revealed no white or red blood cells; urine culture showed no growth. An intravenous pyelogram revealed a hypertrophied but normal left kidney and ureter but no evidence of a kidney on the right. On the excretory cystographic phase no mass was noted. The bladder outline appeared normal (Fig. 1A). Cystoscopy revealed the absence of the right hemitrigone, a smooth bladder wall with no evidence of distal obstruction, and a large extrinsic mass on the right side pushing on the bladder. An aortogram was performed. No renal artery was noted on the right, and the views of the pelvis on the films did not show any abnormal blood vessels (Fig. 1B).
UROLOGY
/
JULY
1973
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VOLUME
II, NUMBER
1
FIGURE 1. (A) Film during intravenous pyelogram reveals absent right collecting collecting system, and no evidence of extrinsic mass on right side of bladder. (B) of right ie&Z artery.
At exploration a midline transperitoneal incision was made, the posterior peritoneum was opened, and the mass was dissected free from the right posterolateral wall of the bladder. The vas was noted entering the mass. It could not be separated from it and was interupted and ligated. The other end of the vas entered the mass. During the procedure, the mass was entered, and about 30 cc. of blood-tinged fluid was removed. There was no odor. Smear did not reveal any sperm; the culture was negative. The mass was excised, and a culture for tuberculosis was negative. The peritoneum was closed over the raw area and the wound closed. Postoperatively, he suffered with urinary retention, and in an attempt to catheterize him, his urethra was perforated. He underwent immediate laparotomy, suprapubic cystotomy, and insertion of paravesical drains. His course from then on was benign. The pathologic report revealed the presence of a cyst, lined with low cuboidal epithelium consistent with seminal vesicle cyst (Fig. 2). At follow-up three months later, the patient was asymptomatic and voiding satisfactorily. Embryology As the embryo ages, the mesonephros (the second of the three organs in the developing urinary system in man) atrophies and disappears, but its tubules and ducts persist and form parts of the adult genital system in both sexes. The
UROLOGY
/ JULY 1973 / VOLUME II, NUMBER 1
system, hypertrophied left Aortogram reveals absence
miillerian duct, developing later than the mesonephric duct, also contributes to the adult genital system (Tables I and II). TABLE
I.
Mesonephric
Female
duct Male
Cephalic tubules Tubules of epoophoron
Caudal tubules Paroophoron
Efferent ducts of testis; lobules of head of epididymis Ductus aberrans (organ of Haller); paradidymis (organ of Giraldes)
Mesonephric duct Longitudinal duct epoophoron
of
TABLE
II.
Canal of epididymis; ductus deferens; ejaculatory duct; seminal vesicle arises as a diverticulum
Miillerian
Female Upper vertical segment Uterus, tubes, and fimbriae Intermediate horizontal segment Fundus, body of uterus Lower vertical segment Uterovaginal canal (fused)
duct Male
Appendix
testis
Disappears Prostatic
utricle
67
FIGURE 2. epithelium,
(A) Low-power view of specimen. (B) High-power consistent with seminal vesicle cyst. Comment
Cyst of the seminal vesicle is itself a rare entity. Until 1971 only 20 cases had been accepted according to Block.2 Smith” reported the first case, a mass containing 5,000 cc. of fluid which he treated by transrectal drainage. Englisch* described four genital cysts: (1) wolffian duct cyst, along the vas deferens; (2) miillerian duct cyst, midline; (3) sinus pocularis cyst, atresia of the utricular orifice; and (4) Zinner5 seminal vesicle cyst, anterolateral. described the first seminal vesicle cyst associated with ipsilateral renal agenesis and discussed the possible cause of the combined picture. TABLE III.
Author
Age
(years)
view of specimen;
The mesonephric duct turns abruptly just before it enters the cloaca, and the ureteric bud arises just proximal to the turn. The seminal vesicle outpouches at thirteen weeks, in close proximity to the ureteric bud which had developed previously at four weeks. Any malformation of the bud could easily also affect the seminal vesicle outpouching, and then combined anomalies result. Zinner4 believed that congenital atresia of the ejaculatory duct was the cause of the retention cyst of the seminal vesicle. Deming6 in discussing the seminal vesicle cyst with normal ipsilateral renal development stated that it was unilateral, single, contained old blood probably secondary to distention and consequent bleeding
World literature cases
Chief Complaint
Intravenous Pyelogram
18
Frequency
Not done
Heetdeers and Delambre’
23
Absent left kidney
Davidson and BeardI
22
Urgency; frequency Urethral verrucae
Kimachi and Wiesenfeld14
27
Frequency
Absent right kidney
Harts
27
Asymptomatic
Hal-V0
28
Asymptomatic
41
Dysuria; hematuria
Absent left kidney; hypertrophied right kidney Absent right kidney; hypertrophied left kidney Absent right kidney; hypertrophied left kidney
68
and Greenslade
Absent right kidney; hypertrophied left kidney
UROLOGY
Diagnosis
Cystoscopy
ZinnerS
Donohue
cyst lined with low cuboidal
/
Seminal vesicle cyst
Mass; absent left hemitrigone Absent left hemitrigone Absent right hemitrigone; extrinsic mass Absent right hemitrigone Absent left hemitrigone
Seminal vesicle cyst Seminal vesicle cyst
Absent right hemitrigone
Seminal vesicle cyst
Absent right hemitrigone
Seminal vesicle cyst
JULY
1973
/
Seminal vesicle cyst Seminal vesicle cyst
VOLUME
II, NUMBER
1
into the cyst and believed that it was caused by either a congenital or acquired obstructive process in the ejaculatory duct. Heetdeers and Delambre7 believed that there was a discontinuity between the seminal vesicle and the ejaculatory duct either acquired or congenital. Patton* believed that the mesonephric duct failed to acquire a normal opening into the cloaca, and after birth secretion occurred and a mechanical effect the same as obstruction was produced, a cystic mass resulted. Hartg*10 considered that all these cysts resulted from an angulation of the mesonephric duct at its cloaca1 end with faulty development of the ureteral diverticulum and its neighbor, the seminal vesicle. Tritsch” established a correlation between recurrent prostatism and seminal vesicle cyst formation caused, he believed, by recurrent ejaculatory duct obstruction and consequent back pressure. Rieser and Griffin12 described
Reviewing the 7 cases described of seminal vesicle cyst and ipsilateral renal agenesis, 2 patients were asymptomatic and 1 had an external penile problem (Table III). The presence, at cystoscopy, of a hemitrigone on the same side does not rule out the possibility of renal agenesis. Campbell, in 1951, describing 248 cases of renal agenesis, noted that 24 had a hemitrigone and an atretic ureter on the ipsilateral side. Our patient, the oldest in the series, did not have any cystic involvement of the ipsilateral epididymis as described by Rieser and Griffin,‘2 and he was the first patient with documented hematuria. In evaluating the patient, we believe that angiography is superior to needle aspiration especially in the light of the recent report by Smith, Webb, and Price13 of carcinoma in the seminal vesicle cyst. We also consider that surgical exploration with excision is mandatory for the same reason.
four genital cysts: (1) mtillerian duct cyst, (2) cyst of the seminal vesicle, (3) retention cyst of the prostate, and (4) diverticulum of ampulla/ ejaculatory duct. The differential diagnosis included the position of the mass, the presence or absence of sperm in it, and a normal or enlarged ipsilateral epididymis:
References
Midline Miillerian duct cyst Large No sperm Testis and epididymis normal Lateral Seminal vesicle cyst Large Epididymis enlarged Sperm Seminal vesicle absent on seminal vesiculogram Prostatic cyst No sperm Testis and epididymis normal Diverticulum of ejaculatory duct or ampulla Sperm Enlarged epididymis Both seminal vesicles normal on seminal vesiculogram
UROLOGY
/
JULY I973
/
VOLUME
II, NUMBER
1
Anatomy, 33rd ed., Davis, D. V., and Davis, 1. GRAY, H.: F., Eds., London, Longmans, Green and Co. Ltd., 1962. Personal communication, 1972. 2. BLOCK, N. L.: 3. SMITH, N. R.: Hydrocele of the seminal vesicle, Lancet 2: 558 (1872). 4. ENGLISCH, R.: ijber Zysten an der hinteren blasenwand bei Mannern, Medical Jahr of Vienna, 1875. Ein Fall von intravesikaler Samenblase5. ZINNER, A.: zyste, Wien. Med. Wochenschr. 64: 605 (1914). 6. DEMING, C. L.: Cysts of the seminal vesicle, Trans. Am. Assoc. Genitourin. Surg. 28: 301 (1935). 7. HEETDEERS, D. R., and DELAMBRE, L. C.: Cyst of the seminal vesicle, J. Urol. 93: 725 (1965). 8. PATTON, B.: Quoted in Heetdeers, D. R., and Delambre, L. c., ibid. 9. HART, J. B.: A case of cyst of hydrops of the seminal vesicle, ibid. 86: 137 (1961). Case of cyst of seminal vesicle, ibid. 96: 247 10. IDEM: (1966). Bietrag zur Pathogenese der Spermato11. TRITSCH, H.: celen und der Samenblasencysten, Huartartzt 9: 538 (1958). Cysts of the prostate, J. 12. RIESER, C., and GRIFFIN, T.: Urol. 91: 282 (1964). Carcinoma of 13. SMITH, G. A., WEBB, E., and PRICE, W.: the seminal vesicle, ibid. 97: 743 (1967). Cyst of the seminal 14. KIMCHI, D., and WIESENFELD, A.: vesicle associated with ipsilateral renal agenesis. Case report, ibid. 89: 906 (1963). 15. DAVIDSON, A. C., and BEARD, J. H.: Seminal vesicle cyst, in association with ipsilateral renal agenesis and lumbar scoliosis, South. Med. J. 62: 608 (1969).
69
VESICLE
IPSILATERAL ROBERT NORMAN
CYST AND
RENAL
E. DONOHUE,
AGENESIS
M.D.”
F. GREENSLADE,
From the Department of Urology, Christchurch, New Zealand
F.R.C.S.,
F.R.A.C.S.
Christchurch
Hospital,
ABSTRACT-A case of seminal vesicle cyst and ipsilateral renal agenesis is presented, the literature summarized, the embryology reviewed, and the diferential diagnosis discussed. Our reasons for surgical therapy are stated.
The differential diagnosis of a paraprostatic mass is always challenging. It involves a detailed knowledge of the embryology of the genitourinary tract. A patient with such a mass is described, the embryology of the area presented, and the world literature reviewed. Case Report A forty-one-year-old farmer, in excellent health, noted the urge to void at 9:00 A.M. but was unable to urinate. At noon, he was still unable to void. At 4:00 P.M. he was in a great deal of distress and began passing small amounts of urine incontinently. At 6:00 P.M. he spontaneously passed thick bloody urine per urethra. There were no clots; he denied any pain or burning. A sample of the fluid was collected, and he visited his doctor who confirmed the presence of blood and placed him on sulfisoxazole (Gantrisin) for ten days. His urine became clear over the next three voidings; all of which were initiated without difficulty. Ten days later at a return visit he was referred .to a urologist. He had undergone appendectomy twenty years before for acute appendicitis. He denied any history of urinary tract disease or symptoms preceding the episode of hematuria.
*Present address: University of Colorado Medical Center, Division of Urology, 4200 East Ninth Avenue, Denver, Colorado 80220.
66
His sexual function was normal; his ejacuIate He is the father of two was never bloody. children. On physical examination his temperature was respiration 18, pulse SO, and 98.6”F. (37°C.) blood pressure 130190 mm. Hg. The only positive physical findings were a healed appendectomy scar in the right lower quadrant and a large paraprostatic cystic mass on the right, slightly above the prostate, nonmovable and nontender. The penis and urethral meatus were normal, and the scrotal examination revealed normal testis, epididymis, and vas bilaterally. The prostate felt normal in size and consistency. Laboratory data were as follows: blood count, 4,000 per cubic millimeter; hemoglobin, 15.5 Gm. per 100 ml.; hematocrit, 45; urinalysis revealed no white or red blood cells; urine culture showed no growth. An intravenous pyelogram revealed a hypertrophied but normal left kidney and ureter but no evidence of a kidney on the right. On the excretory cystographic phase no mass was noted. The bladder outline appeared normal (Fig. 1A). Cystoscopy revealed the absence of the right hemitrigone, a smooth bladder wall with no evidence of distal obstruction, and a large extrinsic mass on the right side pushing on the bladder. An aortogram was performed. No renal artery was noted on the right, and the views of the pelvis on the films did not show any abnormal blood vessels (Fig. 1B).
UROLOGY
/
JULY
1973
/
VOLUME
II, NUMBER
1
FIGURE 1. (A) Film during intravenous pyelogram reveals absent right collecting collecting system, and no evidence of extrinsic mass on right side of bladder. (B) of right ie&Z artery.
At exploration a midline transperitoneal incision was made, the posterior peritoneum was opened, and the mass was dissected free from the right posterolateral wall of the bladder. The vas was noted entering the mass. It could not be separated from it and was interupted and ligated. The other end of the vas entered the mass. During the procedure, the mass was entered, and about 30 cc. of blood-tinged fluid was removed. There was no odor. Smear did not reveal any sperm; the culture was negative. The mass was excised, and a culture for tuberculosis was negative. The peritoneum was closed over the raw area and the wound closed. Postoperatively, he suffered with urinary retention, and in an attempt to catheterize him, his urethra was perforated. He underwent immediate laparotomy, suprapubic cystotomy, and insertion of paravesical drains. His course from then on was benign. The pathologic report revealed the presence of a cyst, lined with low cuboidal epithelium consistent with seminal vesicle cyst (Fig. 2). At follow-up three months later, the patient was asymptomatic and voiding satisfactorily. Embryology As the embryo ages, the mesonephros (the second of the three organs in the developing urinary system in man) atrophies and disappears, but its tubules and ducts persist and form parts of the adult genital system in both sexes. The
UROLOGY
/ JULY 1973 / VOLUME II, NUMBER 1
system, hypertrophied left Aortogram reveals absence
miillerian duct, developing later than the mesonephric duct, also contributes to the adult genital system (Tables I and II). TABLE
I.
Mesonephric
Female
duct Male
Cephalic tubules Tubules of epoophoron
Caudal tubules Paroophoron
Efferent ducts of testis; lobules of head of epididymis Ductus aberrans (organ of Haller); paradidymis (organ of Giraldes)
Mesonephric duct Longitudinal duct epoophoron
of
TABLE
II.
Canal of epididymis; ductus deferens; ejaculatory duct; seminal vesicle arises as a diverticulum
Miillerian
Female Upper vertical segment Uterus, tubes, and fimbriae Intermediate horizontal segment Fundus, body of uterus Lower vertical segment Uterovaginal canal (fused)
duct Male
Appendix
testis
Disappears Prostatic
utricle
67
FIGURE 2. epithelium,
(A) Low-power view of specimen. (B) High-power consistent with seminal vesicle cyst. Comment
Cyst of the seminal vesicle is itself a rare entity. Until 1971 only 20 cases had been accepted according to Block.2 Smith” reported the first case, a mass containing 5,000 cc. of fluid which he treated by transrectal drainage. Englisch* described four genital cysts: (1) wolffian duct cyst, along the vas deferens; (2) miillerian duct cyst, midline; (3) sinus pocularis cyst, atresia of the utricular orifice; and (4) Zinner5 seminal vesicle cyst, anterolateral. described the first seminal vesicle cyst associated with ipsilateral renal agenesis and discussed the possible cause of the combined picture. TABLE III.
Author
Age
(years)
view of specimen;
The mesonephric duct turns abruptly just before it enters the cloaca, and the ureteric bud arises just proximal to the turn. The seminal vesicle outpouches at thirteen weeks, in close proximity to the ureteric bud which had developed previously at four weeks. Any malformation of the bud could easily also affect the seminal vesicle outpouching, and then combined anomalies result. Zinner4 believed that congenital atresia of the ejaculatory duct was the cause of the retention cyst of the seminal vesicle. Deming6 in discussing the seminal vesicle cyst with normal ipsilateral renal development stated that it was unilateral, single, contained old blood probably secondary to distention and consequent bleeding
World literature cases
Chief Complaint
Intravenous Pyelogram
18
Frequency
Not done
Heetdeers and Delambre’
23
Absent left kidney
Davidson and BeardI
22
Urgency; frequency Urethral verrucae
Kimachi and Wiesenfeld14
27
Frequency
Absent right kidney
Harts
27
Asymptomatic
Hal-V0
28
Asymptomatic
41
Dysuria; hematuria
Absent left kidney; hypertrophied right kidney Absent right kidney; hypertrophied left kidney Absent right kidney; hypertrophied left kidney
68
and Greenslade
Absent right kidney; hypertrophied left kidney
UROLOGY
Diagnosis
Cystoscopy
ZinnerS
Donohue
cyst lined with low cuboidal
/
Seminal vesicle cyst
Mass; absent left hemitrigone Absent left hemitrigone Absent right hemitrigone; extrinsic mass Absent right hemitrigone Absent left hemitrigone
Seminal vesicle cyst Seminal vesicle cyst
Absent right hemitrigone
Seminal vesicle cyst
Absent right hemitrigone
Seminal vesicle cyst
JULY
1973
/
Seminal vesicle cyst Seminal vesicle cyst
VOLUME
II, NUMBER
1
into the cyst and believed that it was caused by either a congenital or acquired obstructive process in the ejaculatory duct. Heetdeers and Delambre7 believed that there was a discontinuity between the seminal vesicle and the ejaculatory duct either acquired or congenital. Patton* believed that the mesonephric duct failed to acquire a normal opening into the cloaca, and after birth secretion occurred and a mechanical effect the same as obstruction was produced, a cystic mass resulted. Hartg*10 considered that all these cysts resulted from an angulation of the mesonephric duct at its cloaca1 end with faulty development of the ureteral diverticulum and its neighbor, the seminal vesicle. Tritsch” established a correlation between recurrent prostatism and seminal vesicle cyst formation caused, he believed, by recurrent ejaculatory duct obstruction and consequent back pressure. Rieser and Griffin12 described
Reviewing the 7 cases described of seminal vesicle cyst and ipsilateral renal agenesis, 2 patients were asymptomatic and 1 had an external penile problem (Table III). The presence, at cystoscopy, of a hemitrigone on the same side does not rule out the possibility of renal agenesis. Campbell, in 1951, describing 248 cases of renal agenesis, noted that 24 had a hemitrigone and an atretic ureter on the ipsilateral side. Our patient, the oldest in the series, did not have any cystic involvement of the ipsilateral epididymis as described by Rieser and Griffin,‘2 and he was the first patient with documented hematuria. In evaluating the patient, we believe that angiography is superior to needle aspiration especially in the light of the recent report by Smith, Webb, and Price13 of carcinoma in the seminal vesicle cyst. We also consider that surgical exploration with excision is mandatory for the same reason.
four genital cysts: (1) mtillerian duct cyst, (2) cyst of the seminal vesicle, (3) retention cyst of the prostate, and (4) diverticulum of ampulla/ ejaculatory duct. The differential diagnosis included the position of the mass, the presence or absence of sperm in it, and a normal or enlarged ipsilateral epididymis:
References
Midline Miillerian duct cyst Large No sperm Testis and epididymis normal Lateral Seminal vesicle cyst Large Epididymis enlarged Sperm Seminal vesicle absent on seminal vesiculogram Prostatic cyst No sperm Testis and epididymis normal Diverticulum of ejaculatory duct or ampulla Sperm Enlarged epididymis Both seminal vesicles normal on seminal vesiculogram
UROLOGY
/
JULY I973
/
VOLUME
II, NUMBER
1
Anatomy, 33rd ed., Davis, D. V., and Davis, 1. GRAY, H.: F., Eds., London, Longmans, Green and Co. Ltd., 1962. Personal communication, 1972. 2. BLOCK, N. L.: 3. SMITH, N. R.: Hydrocele of the seminal vesicle, Lancet 2: 558 (1872). 4. ENGLISCH, R.: ijber Zysten an der hinteren blasenwand bei Mannern, Medical Jahr of Vienna, 1875. Ein Fall von intravesikaler Samenblase5. ZINNER, A.: zyste, Wien. Med. Wochenschr. 64: 605 (1914). 6. DEMING, C. L.: Cysts of the seminal vesicle, Trans. Am. Assoc. Genitourin. Surg. 28: 301 (1935). 7. HEETDEERS, D. R., and DELAMBRE, L. C.: Cyst of the seminal vesicle, J. Urol. 93: 725 (1965). 8. PATTON, B.: Quoted in Heetdeers, D. R., and Delambre, L. c., ibid. 9. HART, J. B.: A case of cyst of hydrops of the seminal vesicle, ibid. 86: 137 (1961). Case of cyst of seminal vesicle, ibid. 96: 247 10. IDEM: (1966). Bietrag zur Pathogenese der Spermato11. TRITSCH, H.: celen und der Samenblasencysten, Huartartzt 9: 538 (1958). Cysts of the prostate, J. 12. RIESER, C., and GRIFFIN, T.: Urol. 91: 282 (1964). Carcinoma of 13. SMITH, G. A., WEBB, E., and PRICE, W.: the seminal vesicle, ibid. 97: 743 (1967). Cyst of the seminal 14. KIMCHI, D., and WIESENFELD, A.: vesicle associated with ipsilateral renal agenesis. Case report, ibid. 89: 906 (1963). 15. DAVIDSON, A. C., and BEARD, J. H.: Seminal vesicle cyst, in association with ipsilateral renal agenesis and lumbar scoliosis, South. Med. J. 62: 608 (1969).
69