Müllerian Duct Cysts

Müllerian Duct Cysts

MULLERIAN DUCT CYSTS REPORT OF A CASE 1 RUSSELL A. HENNESSEY It is our purpose in this presentation to call attention to certain affections developi...

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MULLERIAN DUCT CYSTS REPORT OF A CASE 1

RUSSELL A. HENNESSEY

It is our purpose in this presentation to call attention to certain affections developing from remnants of Muller's ducts, about which but little can now be found in the literature. Symmetrical midline tumors of the lower abdomen arising from the region of the recto-vesical septum in the male which simulate a distended urinary bladder and produce urinary symptoms are rarely encountered. Because of their inaccessibility, such lesions require thorough clinical study to accurately determine their point of origin. Case report. A white male, aged 36 years, entered the hospital, complaining of an enlarging mass in the lower abdomen, first noticed about one month before admission. The act of micturition had become slow, frequent and difficult. Ten years before he had passed an ureteral calculus spontaneously, following an attack of left renal colic. In all other particulars, his family and past history were unimportant. The patient, of medium stature, weighed 215 pounds, and presented a rather prominent abdomen. Physical examination was negative except for a symmetrical ovoid mass palpable in the lower abdomen extending upward to the umbilicus. On deep palpation it was possible to move the mass laterally, but no vertical movement was possible. The mass was firm but elastic, and simulated a markedly distended bladder. Little discomfort was elicited on palpation. Rectal examination revealed the prostate to be normal in size and consistency. As the examining finger reached the upper prostate region, a firm cystic mass was found fixed deeply in the pelvis and bulging into the upper rectum. Moderate resiliency of the mass could be elicited on bimanual examination. The blood and urine examinations were negative. A blood wassermann was also found to be negative. X-rays of the chest were negative. An ovoid homogeneous shadow could be seen extending upward from the pelvis in a plain x-ray of the abdomen (fig. 1). Two ounces of residual urine was recovered from the bladder, with no alteration in the size nor change in the contour of the abdominal tumor. Cystoscopy was attempted, but was unsuccessful because of an unyielding obstruction in the posterior urethra, resulting from distortion. 1 Read at annual meeting, American Urological Association, White Sulphur Springs, W. Va., June 1, 1939.

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In order to determine the origin of the mass as accurately as possible and to determine its relationship to adjacent structures, a cystogram, intravenous

FrG. 1. Flat x-ray of abdomen, showing homogeneous cystic mass in lower abdomen

FIG. 2. Cystogram showing distortion of hladder from tumor pressure

urograms and barium enema x-rays were made. Marked elliptical compression and distortion of the bladder could be demonstrated (fig. 2). No dilatation of the kidney pelves or ureters was evident in the intravenous urograms

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(fig. 3). Marked extrapelvic displacement and compression of the sigmoid and colon was found in the barium enema x-ray (fig. 4).

FIG. 3. Intravenous urograms show no changes in upper urinary tract

FIG. 4. Barium enema, demonstrating extrapelvic displacement of the colon and compression of the signoid.

On the third day after admission, abdominal exploration of the tumor was done through a right rectus incision. Upon opening the peritoneum, the

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large, ovid, tense, cystic mass was found to arise from the deep pelvis. No adhesions to the dome or posterior wall of the mass were found, but the deep anterior cyst wall was found inseparably attached to the bladder (fig. 5). It was dark gray in color, and of rubbery consistency. After careful preparation, the cyst was punctured and aspirated. Three thousand cubic centimeters of viscid chocolate-colored fluid was removed. The cyst wall was found to be about 2 to 3 mm. in thickness. Upon examination of the interior of the cyst, a solid tumor mass could be felt deep in the pelvis and attached firmly at a point behind the bladder and apparently at the apex of the prostate. To identify the attachment of the tumor more accurately, a finger was inserted

FIG. 5. Graphic illustration of cystic mass

into the rectum. The cyst and tumor mass could be felt to be intimately attached to the upper pole of the right lobe of the prostate and posterior wall of the bladder. The broad pedicle of this mass was clamped and the tumor removed. Because of the inaccessibility of the pedicle, ligation was not attempted and the pedicle clamp was left in situ. A greater part of the cyst wall was removed, and the remaining portion wrapped about the pedicle clamp. Drainage of the pelvis was provided by one rubber tube drain, and the incision closed. The clamp was removed in 72 hours. The postoperative course was uneventful, and the patient was discharged on the fourteenth day. Pathological Report (Dr. T. C. Moss and Dr. W. W. Robinson): The cyst fluid was rich in cholesterin crystals and had a specific gravity of 1.030. Spec-

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imen consisted of large cyst, which appeared to be about 30 cm. in diameter. The cyst wall, which was 2 to 3 mm. in thickness, was grayish brown in color, of rubberty consistency and in portions was hyalinized. Contained in the cyst and continuous with the cyst wall was a mass the size of an orange, which on section was found to be composed of multiple cyst cavities measuring 2 cm. in diameter. The lumen of these cysts was filled with a clear gelatinous fluid. Microscopically, the wall of the cyst cavities was composed of fibrous tissues in which smooth muscle fibers were abundant. The cyst cavities contained cuboidal epithelium in some instances of more than 1 cell layer in depth. The gelatinous substance in the lumen of the cysts was homogenous

FIG. 6. Photomicrograph of solid tumor tissue removed from posterior wall of bladder

and pink staining. No cells could be found in this substance (fig. 6). Diagnosis: Multilocular cystadenoma of Mi.illerian duct origin (benign in character). Subsequent rectal examinations revealed a soft cystic mass extending upward from the upper pole of the right lobe of the prostate. Therefore, 6 months later, the patient was readmitted to the hospital, and through a perineal exposure this mass was removed. The specimen measured 4 x 3 x 2 cm. and was described pathologically as identical with the previously described specimen. Subsequent examinations of this patient have revealed no further masses or other trouble.

Embryology: The development of the reproductive and urinary systems

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are closely related. Both originate from the mesoderm and are first distinguishable as a common urogenital fold. With later development, nephric and genital ridges appear. Development of each system is complex with some primitive parts appearing only to disappear after a transitory existence (fig. 7). Mature organs result from the union of structures that originally were remotely separated. These developmental phenomena explain the frequency of anomalies of the urinary tract. Embryos of 6 weeks, or about 12 mm., are characterized by the possession of indifferent or both male and female ducts. Evidence of testicular development begins in embryos of 13 mm. When the sex is established, the ducts of the

Mesonephros

Cloaca INDIFFERENT STAGE

FIG.

7. From Arey.

Development of Mtillerian ducts

opposite sex disappear. In the female embryo, the Mullerian duct develops into the Fallopian tube, uterus and vagina. In the male embryo, the duct on each side disappears and the only remnants of it found in the adult are the appendix testis formed from the proximal end, and the prostatic utricle formed from the distal end. If this structure persisted in the adult, it would extend from the appendix testis in a groove between the testicle and epididymis, up the spermatic cord, through the abdominal ring and finally come to lie between the vas deferens and the bladder, where it would join with the duct from the opposite side. They would be incorporated in the bladder wall, passing through the prostate to end in the utricle. Its inclusion in the musculature of the bladder explains why it has been impossible in the op-

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erated cases of Mi.illerian duct cysts to separate them readily from the posterior wall of the bladder (fig. 8). Origin of pelvic tumors in the male. Instances of pelvic tumors in the male, which have attained sufficient size to disturb bladder function or produce urinary symptoms, are rarely encountered. We have, however, observed two such cases, the first a Mi.illerian duct cyst, the subject of this report, and a second which was a solid tumor, a fibro-sarcoma showing myxomatous degeneration, occurring in a child which came to autopsy. The origin of pelvic tumors reported in the literature is in most instances very vague. Few cases were subjected to open surgery, and but few came to autopsy. Epidid.Jmis

MALE

FIG. 8. From Arey.

Normal male sex development and obliteration of Miiller's ducts

In the majority of reported cases, their origin was cited as cysts of the prostate, cysts of the ejaculatory duct, cysts of the seminal vesicles or cysts of the bladder wall. Wesson, in 1925, reviewed 29 cysts of the prostate and cited 4 cases of cystic tumors which undoubtedly arose from remnants of the Wolffian body or Mi.illerian duct. Voelker stated that cysts of the seminal vesicle develop usually from obstruction of the ejaculatory duct and, as a rule, do not attain a size larger than a hazel nut. He was also of the opinion that most of the cases reported as cysts of the seminal vesical in reality arose from remains of the Wolffian ducts or Mi.illerian tubules. Furthermore, as a rule, spermatozoa should be demonstrable in the contents of seminal vesical cysts. In providing an explanation for the site of origin of retrovesical tumors in the male, Begg stated that when complete obliteration of the central part of the ducts does not take place, the residual epithelium may act

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in a similar manner to that of the urachus. It would seem very likely in this respect that the additional case which we have observed could be accounted for in this manner. Begg also stated that to qualify as a cystic tumor arising from Mullerian remnants it should (1) show evidence of a cysto-adenoma, (2) the acini should be lined with cuboidal epithelium, (3) intimate relationship between the cyst and the posterior wall of the bladder should be demonstrable, and (4) a wedge of tumor tissue should pass through the prostate to the region of the verumontanum without affecting the adjacent prostatic tissue. Literature. R. Campbell Begg, reporting a case of Mtillerian duct cyst in 1936, stated that he had been unable to find a parallel case in the literature of the previous 20 years. Wesson, in 1925, pointed out that certain previously reported cases of retrovesical cysts, notably those of Smith, Rolfe and Spence undoubtedly arose from remnants of the Mullerian ducts. R. Guiteras in 1894, reported an instance of an enormous retrovesical cyst which, because of details contained in the report, was doubtless an example of a Mullerian duct cyst. Von Gaza, in 1923, cited an instance of a retrovesical cyst which he believed developed from Muller's duct. Coppridge recently presented an excellent article in which he reviewed a number of reported cases, discussed the development of Mullerian duct cysts, and reported a new case. Culver and Baker (1928) reported 2 instances of perivesical tumors creating urinary symptoms. The first was reported as an enchondroma and a second case showed myxomatous tissue most probably arising from embryonal mucous membrane. This fact suggested the possibility again of the origin of certain pelvic tumors in the male from unobliterated Mullerian tissues. Diagnosis. The development of a retrovesical cystic mass which attains sufficient size to interfere with normal bladder function should suggest the presence of a cyst-adenoma or other tumor arising from the non-obliterated remnants of Muller's ducts. The mass frequently becomes sufficiently large to strikingly resemble a distended bladder and become palpable in the lower abdomen. Palpation of the upper prostatic area invariably reveals a mass encroaching upon the rectum and extending upward from the region of the prostate and seminal vesicles. Because of compression or distortion of the posterior urethra and internal vesical orifice, instrumental examination may not at times be successfully done. It then becomes necessary to depend upon excretory urography, cystography, gastrointestinal x-rays, barium enem-

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ata and exploratory surgery to determine the ongm and character of the mass. Treatment. Relatively few instances of complete surgical removal of Mtillerian duct cysts are found in the literature. In Begg's case, surgical drainage only could be done. In 2 instances, aspiration by the introduction of a trocar through the rectum was the method of treatment employed. N. R. Smith reported the removal of 5 quarts of fluid in this manner. Coppridge removed all possible cystic tissue suprapubically that could be dissected from the bladder and promoted further destruction by coagulation and packing. Von Gaza's surgical approach was through the perineum. In the masses of considerable size, the abdominal approach would seem to be the method of choice. However, to successfully remove all tumor tissue, perineal section may also be necessary. CONCLUSIONS

Symmetrical midline tumors of the lower abdomen in the male simulating a distended bladder and producing symptoms of bladder dysfunction should suggest the existence of a Mtillerian duct cyst-adenoma. Treatment consists of surgical removal which may be accomplished by abdominal or perineal approach, or both. Remnants of the Mtillerian ducts quite likely provide the point of origin for retrovesical tumors more often than commonly thought. One additional case of cyst-adenoma of the Mtillerian duct is reported.

Exchange Bldg., Memphis, Tenn. REFERENCES AREY: Developmental Anatomy. Saunders Co., Philadelphia, Pa. BEGG, R. CAMPBELL: Massive cystadenoma of Muller's Duct. Brit. J. Urol., 8: 1936. COPPRIDGE, W. M.: Mtillerian duct cysts. South. M. J., 32: 248-251, 1939. CULVER, H., AND BAKER, W. J.: Exvesical tumors of the male causing interference with urination. Trans. Amer. Asso. of Genito-Urinary Surg., 21: 285-297 and 327-335, 1928. DEMING, C. L.: Complete urinary obstruction due to hydatid cyst. J. Urol., 10: 1-43, 1923. F1sK, A. L.: A cyst of the right vesicula seminalis; aspiration by rectum. Ann. Surg., 28: 652-654, 1898. GAZA, W. VON: Uber chronische Spermatocystitis und tiber eine Cyste des persistierenden Mtillershen Ganges. Arch. f. klin. Chir., 126: 502-509, 1923. GurTERAS, R.: A case of sero-purulent cyst, probably of right seminal vesicle. Lancet, Lond. 2: 74, 1894. ROLFE: Cystic tumor of the left seminal vesicle; undescended left testicle. Lancet, Long., 2: 782, 1876. SMITH, N. R.: Hydrocele of seminal vesicle. Lancet, Lond., 2: 558, 1872. VOELCKER, F.: Chirurgie der Samenblassen. Neue Deutsche Chirurgie, 2: 195-197, WESSON, M. B.: Cysts of the prostate and urethra. J. Urol., 13: 605-632, 1925. ZINNER, A.: Ein Fall von intravesikaler Samenblasencyste. Verhandl. d. deutsch. Gesellsch. f. Urol., 4: 384-9, 1914; also: Wien. med. Wchnschr., 64: 605-609, 1914.