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Cyst of seminal vesicle associated with ipsilateral renal agenesis
CYST OF SEMINAL VESICLE IPSILATERAL GERSHON
A. S. LEWIS,
WITH
RENAL AGENESIS
C. EJECKAM,
S. GOVATSOS,
ASSOCIATED
M.D.
M.D. M.D.
From the Departments of Pathology, Laboratories and Surgery, S.A. Grace General Hospital, St. John’s, Newfoundland, Canada
Medicine,
ABSTRACT-A case of cyst of seminal vesicle associated with ipsilateral renal agenesis in a twentythree-year-old man presenting primarily with rectal symptoms is reported. The usual symptoms had been urinary bladder irritation and pain on ejaculation. The embryologic development of this rare entity is discussed.
Cysts of seminal vesicle with or without ipsilatera1 renal agenesis are rare. Zinner’ reported the first case in 1914. In a review of literature in 1976, Fuselier and Peters2 found only 20 cases including their own. However, Klein, in his discussion in the clinicopathologic exercises of Massachusetts General Hospital3 quoting Bleckley and Bleich4 claimed that a search of the literature revealed that the association of seminal vesicle cysts and renal agenesis has been reported in about 100 patients. The clinical presentation of these cysts is not always diagnostic, and their existence may not be a common knowledge. We report an additional case of cyst of seminal vesicle associated with ipsilateral renal agenesis encountered in our hospital. Case Report A twenty-three-year-old man was admitted to this hospital on January 16, 1980, with complaints of difficulty with defecation for about a month and a half. He would have to strain excessively in order to evacuate, and even after that he always had a feeling of fullness in the rectum. He took laxatives without relief. Later he saw his family physician who felt a mass in
372
the rectum and referred the patient to a surgeon. Past medical history and family history showed he was one of a set of triplets and was small at birth but otherwise well. One of his sister triplets had a blockage in the coronary artery and underwent surgery at age sixteen years. Examination at the hospital revealed a physically normal male except for the rectal examination which revealed a mass which appeared to be just above the prostate gland. Further investigations were done including ultrasound which gave a suggestion of solid mass deep to the bladder, a barium enema which was noncontributory, and a chest x-ray film which was normal. Other investigations included total white cell count 8.7 x 103, hemoglobin 15.7 Gm/lOO ml, hematocrit 48, mean corpuscular volume 87.8, and platelets 275 x 103. His blood chemistry and urinalysis were normal. A diagnosis of a pelvic mass was made, and on January 18, 1980 he was operated on. Operative findings At laparotomy, a large retroperitoneal mass was found below the urinary bladder and in
UROLOGY
/ OCTOBER
1984
/ VOLUME
XXIV, NUMBER 4
FIGURE 1. (A) Section of cyst lined by columnar epithelium thrown into papillary folds. (B) Another area of cyst showing flattened epithelial lining; no inflammatory cell infiltrate. (Hematoxylin and eosin. original magnification x 60 and x 40, respectively.)
front of the rectum. On opening of the peritoneum and mobilization of the mass it was seen that the mass was lobulated and cystic. Because the impression created was of an ectopic right hydronephrotic kidney the right renal fossa was examined and revealed no kidney. The left kidney was present but was slightly enlarged. There was a vascular pedicle to the mass as well as a short tubular structure. These were clamped, ligated, and divided, and the mass was removed. The patient made an uneventful recovery. An intravenous pyelogram (IVP) was performed after full recovery, and this confirmed the absence of the right kidney. Pathology Grossly the specimen consisted of a cystic bosselated mass and measured 7 by 4 by 3 cm. There was a dilated saccular and tubular structure leading to the main mass; this measured 3.5 cm in length. On section this cyst was unilocular, and the mucosal lining in areas appeared granular while in other areas fine ridges were present. The wall was thickened and measured 0.2 cm. Microscopically, the section consisted of cystic spaces lined by columnar epithelium which had been thrown into primary and secondary papillary folds (Fig. 1A) and joined by epithelial anastomosis in areas. A few rounded basal cells were present. In other areas the lining of the cyst was flattened and consisted of flattened to low cuboidal epithelium (Fig. 1B). Secondary and tertiary cysts formed in the muscle coat due to invagination of the lining, were present. No renal, ureteric tissue, or epithelium were
UROLOGY
I
OCTOBER
1984
/ VOLUME
found. A pathologic diagnosis of cyst of seminal vesicle was made. Comment Cysts of the seminal vesicle are exceptionally rare. They are either acquired following blockage consequent to inflammation, or congenital in which case they can be accompanied by ipsilateral renal agenesis. The age range of the previously documented cases was between eighteen and forty-one years, with a peak between twenty and twenty-eight. The late age of onset in the congenital cases, however, can be attributed to the fact that though the blockage of the duct exists at birth, the production of seminal fluid and its accumulation require long periods of time to enable the cyst to enlarge enough to cause symptoms.5 Only 1 case reported by Rajfer et aLe occurred in a black male; all others were encountered in the white race. The significance of this racial difference is yet to be understood fully. However, it may reflect the readiness of one racial group as against the other to seek medical care. There is no predilection for left or right side of a seminal vesicle. Only 1 case of bilateral cyst formation was documented;7 the rest were distributed approximately equally on both sides. The majority of the patients presented with urinary and perineal symptoms. Some patients were asymptomatic while others were investigated for infertility, hematuria, and epididymitis. Our patient, however, did not present with urinary symptoms. He came to the hospital primarily for problems of defecation. Though barium enema did not demonstrate a
XXIV. NUMBER 4
373
structural extrinsic deformity of the rectum, at operation the mass was seen to compress the rectum. Failure to demonstrate any structural change in the rectum at barium enema would be due to the fact that the mass was cystic, multisaccular, and might have been subject to periodic change in volume. Occasionally, especially in acquired types, the cyst could be infected, and the patient presents with fever and a prostatic mass.6 Usually seminal vesicle cysts are diagnosed by a combination of history, physical examination (including palpation of a mass per rectum), and intravenous pyelography. Other investigations would include cystoscopy and angiography, a vasoseminal vesiculogram, and renal scan studies. Unfortunately, our patient did not have some of these investigations before surgery, largely because his symptoms were of rectal rather than urinary tract origin. The association of congenital cysts of seminal vesicle and ipsilateral renal agenesis is not unusual since both organs are related developmentally.* During embyrogenesis the seminal vesicle develops as an outpouch from the mesonephric duct in close relationship to the ureteral bud which had developed from the mesonephric duct at approximately six weeks of gestation. Maldevelopment of the mesonephric duct in and around the origin of the ureteral bud will lead to anomalous growth of the ureteral bud and seminal vesicles. In turn, the full development of the metanephric blastema is dependent on normality of and induction by ureteral bud. Therefore, anomaly of the ureteral bud will lead to renal agenesis on the affected side. The majority of cases of cyst of the seminal vesicle have been treated by open excision. Other surgical approaches used in management of these cases include transperineal and transrectal aspiration or transurethral unroofing of the cyst. Differential diagnosis of this lesion may also present a problem. Cysts of the seminal vesicle have to be distinguished from
374
abscesses walled off in the cul-de-sac, leiomyoma of the urinary bladder wall, cyst of the miillerian and wolfian duct remnants, cyst of the utricle, and malignant lesions of the rectum. Other lesions which may be confused with cyst of the seminal vesicles are the presacral tumors such as chordomas, teratomas, dermoid neurofibromas, and cysts, ependymomas, neurilemomas. These presacral tumors, however, besides compressing the rectum and urinary bladder will usually present with some neurologic features which are absent in cysts of the seminal vesicle. Cyst of the seminal vesicle, though rare, has to be included in the differential diagnosis of a mass around the prostate and urinary bladder in a male in his height of productive activity, especially if the mass can be palpated per rectum. PO. Box 2425 Enugu, Anambra State, Nigeria (DR. E JECKHAM) ACKNOWLEDGMENT. To the secretaries of the Department of Pathology, the librarian, and staff of the Grace Hospital for their assistance. References 1. Zinner A: Ein Fall van intravesikaler Samenblasenzyste, Wien Med Wochenschr 64: 606 (1914). 2. Fuselier HA Jr, and Peters DH: Cyst of seminal vesicle with ipsilateral renal agenesis and ectopic ureter, J Urol 116: 833 (1976). 3. Case Records of the Massachusetts General Hospital. Weekly clinicopathologic exercises, N Engl J Med 302: 1246 (1980). 4. Beckley RF, and Bleich HL: Paper chase. A computer based reprint storage and retrieval system, Comput Biomed Res 10: 423 (1977). 5. Knudsen JB, Brun B, and Emus HC: Familial renal agenesis and urogenital malformations: seminal vesicle cyst and vaginal cyst with bicornuate uterus in siblings, Stand J Urol Nephrol 13: 109 (1979). 6. Rajfer J, Eggleston JC, Sanders RC, and Walsh PC: Clinicopathological Conference: fever and prostatic mass in a young man, J Urol 119: 555 (1978). 7. Sharma TC, Dorman PS, and Dorman PI-I: Bilateral seminal vesicle cyst, ibid 102: 741 (1969). 8. Arty LB: Developmental Anatomy, 7th ed. Philadelphia, WB Saunders Co, 1965.
UROLOGY
i
OCTOBER
1984
/
VOLUME
XXIV, NUMBER
4
A. S. LEWIS,
WITH
RENAL AGENESIS
C. EJECKAM,
S. GOVATSOS,
ASSOCIATED
M.D.
M.D. M.D.
From the Departments of Pathology, Laboratories and Surgery, S.A. Grace General Hospital, St. John’s, Newfoundland, Canada
Medicine,
ABSTRACT-A case of cyst of seminal vesicle associated with ipsilateral renal agenesis in a twentythree-year-old man presenting primarily with rectal symptoms is reported. The usual symptoms had been urinary bladder irritation and pain on ejaculation. The embryologic development of this rare entity is discussed.
Cysts of seminal vesicle with or without ipsilatera1 renal agenesis are rare. Zinner’ reported the first case in 1914. In a review of literature in 1976, Fuselier and Peters2 found only 20 cases including their own. However, Klein, in his discussion in the clinicopathologic exercises of Massachusetts General Hospital3 quoting Bleckley and Bleich4 claimed that a search of the literature revealed that the association of seminal vesicle cysts and renal agenesis has been reported in about 100 patients. The clinical presentation of these cysts is not always diagnostic, and their existence may not be a common knowledge. We report an additional case of cyst of seminal vesicle associated with ipsilateral renal agenesis encountered in our hospital. Case Report A twenty-three-year-old man was admitted to this hospital on January 16, 1980, with complaints of difficulty with defecation for about a month and a half. He would have to strain excessively in order to evacuate, and even after that he always had a feeling of fullness in the rectum. He took laxatives without relief. Later he saw his family physician who felt a mass in
372
the rectum and referred the patient to a surgeon. Past medical history and family history showed he was one of a set of triplets and was small at birth but otherwise well. One of his sister triplets had a blockage in the coronary artery and underwent surgery at age sixteen years. Examination at the hospital revealed a physically normal male except for the rectal examination which revealed a mass which appeared to be just above the prostate gland. Further investigations were done including ultrasound which gave a suggestion of solid mass deep to the bladder, a barium enema which was noncontributory, and a chest x-ray film which was normal. Other investigations included total white cell count 8.7 x 103, hemoglobin 15.7 Gm/lOO ml, hematocrit 48, mean corpuscular volume 87.8, and platelets 275 x 103. His blood chemistry and urinalysis were normal. A diagnosis of a pelvic mass was made, and on January 18, 1980 he was operated on. Operative findings At laparotomy, a large retroperitoneal mass was found below the urinary bladder and in
UROLOGY
/ OCTOBER
1984
/ VOLUME
XXIV, NUMBER 4
FIGURE 1. (A) Section of cyst lined by columnar epithelium thrown into papillary folds. (B) Another area of cyst showing flattened epithelial lining; no inflammatory cell infiltrate. (Hematoxylin and eosin. original magnification x 60 and x 40, respectively.)
front of the rectum. On opening of the peritoneum and mobilization of the mass it was seen that the mass was lobulated and cystic. Because the impression created was of an ectopic right hydronephrotic kidney the right renal fossa was examined and revealed no kidney. The left kidney was present but was slightly enlarged. There was a vascular pedicle to the mass as well as a short tubular structure. These were clamped, ligated, and divided, and the mass was removed. The patient made an uneventful recovery. An intravenous pyelogram (IVP) was performed after full recovery, and this confirmed the absence of the right kidney. Pathology Grossly the specimen consisted of a cystic bosselated mass and measured 7 by 4 by 3 cm. There was a dilated saccular and tubular structure leading to the main mass; this measured 3.5 cm in length. On section this cyst was unilocular, and the mucosal lining in areas appeared granular while in other areas fine ridges were present. The wall was thickened and measured 0.2 cm. Microscopically, the section consisted of cystic spaces lined by columnar epithelium which had been thrown into primary and secondary papillary folds (Fig. 1A) and joined by epithelial anastomosis in areas. A few rounded basal cells were present. In other areas the lining of the cyst was flattened and consisted of flattened to low cuboidal epithelium (Fig. 1B). Secondary and tertiary cysts formed in the muscle coat due to invagination of the lining, were present. No renal, ureteric tissue, or epithelium were
UROLOGY
I
OCTOBER
1984
/ VOLUME
found. A pathologic diagnosis of cyst of seminal vesicle was made. Comment Cysts of the seminal vesicle are exceptionally rare. They are either acquired following blockage consequent to inflammation, or congenital in which case they can be accompanied by ipsilateral renal agenesis. The age range of the previously documented cases was between eighteen and forty-one years, with a peak between twenty and twenty-eight. The late age of onset in the congenital cases, however, can be attributed to the fact that though the blockage of the duct exists at birth, the production of seminal fluid and its accumulation require long periods of time to enable the cyst to enlarge enough to cause symptoms.5 Only 1 case reported by Rajfer et aLe occurred in a black male; all others were encountered in the white race. The significance of this racial difference is yet to be understood fully. However, it may reflect the readiness of one racial group as against the other to seek medical care. There is no predilection for left or right side of a seminal vesicle. Only 1 case of bilateral cyst formation was documented;7 the rest were distributed approximately equally on both sides. The majority of the patients presented with urinary and perineal symptoms. Some patients were asymptomatic while others were investigated for infertility, hematuria, and epididymitis. Our patient, however, did not present with urinary symptoms. He came to the hospital primarily for problems of defecation. Though barium enema did not demonstrate a
XXIV. NUMBER 4
373
structural extrinsic deformity of the rectum, at operation the mass was seen to compress the rectum. Failure to demonstrate any structural change in the rectum at barium enema would be due to the fact that the mass was cystic, multisaccular, and might have been subject to periodic change in volume. Occasionally, especially in acquired types, the cyst could be infected, and the patient presents with fever and a prostatic mass.6 Usually seminal vesicle cysts are diagnosed by a combination of history, physical examination (including palpation of a mass per rectum), and intravenous pyelography. Other investigations would include cystoscopy and angiography, a vasoseminal vesiculogram, and renal scan studies. Unfortunately, our patient did not have some of these investigations before surgery, largely because his symptoms were of rectal rather than urinary tract origin. The association of congenital cysts of seminal vesicle and ipsilateral renal agenesis is not unusual since both organs are related developmentally.* During embyrogenesis the seminal vesicle develops as an outpouch from the mesonephric duct in close relationship to the ureteral bud which had developed from the mesonephric duct at approximately six weeks of gestation. Maldevelopment of the mesonephric duct in and around the origin of the ureteral bud will lead to anomalous growth of the ureteral bud and seminal vesicles. In turn, the full development of the metanephric blastema is dependent on normality of and induction by ureteral bud. Therefore, anomaly of the ureteral bud will lead to renal agenesis on the affected side. The majority of cases of cyst of the seminal vesicle have been treated by open excision. Other surgical approaches used in management of these cases include transperineal and transrectal aspiration or transurethral unroofing of the cyst. Differential diagnosis of this lesion may also present a problem. Cysts of the seminal vesicle have to be distinguished from
374
abscesses walled off in the cul-de-sac, leiomyoma of the urinary bladder wall, cyst of the miillerian and wolfian duct remnants, cyst of the utricle, and malignant lesions of the rectum. Other lesions which may be confused with cyst of the seminal vesicles are the presacral tumors such as chordomas, teratomas, dermoid neurofibromas, and cysts, ependymomas, neurilemomas. These presacral tumors, however, besides compressing the rectum and urinary bladder will usually present with some neurologic features which are absent in cysts of the seminal vesicle. Cyst of the seminal vesicle, though rare, has to be included in the differential diagnosis of a mass around the prostate and urinary bladder in a male in his height of productive activity, especially if the mass can be palpated per rectum. PO. Box 2425 Enugu, Anambra State, Nigeria (DR. E JECKHAM) ACKNOWLEDGMENT. To the secretaries of the Department of Pathology, the librarian, and staff of the Grace Hospital for their assistance. References 1. Zinner A: Ein Fall van intravesikaler Samenblasenzyste, Wien Med Wochenschr 64: 606 (1914). 2. Fuselier HA Jr, and Peters DH: Cyst of seminal vesicle with ipsilateral renal agenesis and ectopic ureter, J Urol 116: 833 (1976). 3. Case Records of the Massachusetts General Hospital. Weekly clinicopathologic exercises, N Engl J Med 302: 1246 (1980). 4. Beckley RF, and Bleich HL: Paper chase. A computer based reprint storage and retrieval system, Comput Biomed Res 10: 423 (1977). 5. Knudsen JB, Brun B, and Emus HC: Familial renal agenesis and urogenital malformations: seminal vesicle cyst and vaginal cyst with bicornuate uterus in siblings, Stand J Urol Nephrol 13: 109 (1979). 6. Rajfer J, Eggleston JC, Sanders RC, and Walsh PC: Clinicopathological Conference: fever and prostatic mass in a young man, J Urol 119: 555 (1978). 7. Sharma TC, Dorman PS, and Dorman PI-I: Bilateral seminal vesicle cyst, ibid 102: 741 (1969). 8. Arty LB: Developmental Anatomy, 7th ed. Philadelphia, WB Saunders Co, 1965.
UROLOGY
i
OCTOBER
1984
/
VOLUME
XXIV, NUMBER
4