Talk:Paper - Cysts of the Wolffian body (1924)

CYSTS OF THE WOLFFIAN BODY

By Frank Hinman, M.D., Tuomas E. Grsson, M.D. AND Apvoutew A. Kutzmann, M.D. or San Francisco, Cau.

FROM THE DEPARTMENT OF UROLOGY. UNIVERSITY OF CALIFORNIA MEDICAL SCHOOL, SAN FRANCISCO

THE extreme rarity of occurrence of cysts of the Wolffian body (mesonephros) and the paucity of reports in the literature warrants the consideration of such cases when they are encountered. For a thorough knowledge of these cysts as regards their character and location, one must bear in mind the retroperitoneal development of the Wolffian body in the lumbar region; its complex metamorphosis, the remarkable migration of its component parts and their ultimate fate, the atrophic changes which they undergo and, lastly, the vestigial remnants as they exist after complete development has occurred. The surgeon is not fully efficient who does not bear in mind the pathological potentialities of the Wolffian body in the exploration of the abdomen. These facts, together with the confusion extant in the literature, the failure of textbooks to consider the Wolffian body in differential diagnosis, renders important the consideration anew. The subject was brought to our attention by the case reported below.

The present tendency is to credit nearly all cystic retroperitoneal tumors as having their origin from fcetal renal elements, unless they are definitely proven to be associated with other organs. Lobstein, in 1829, and later Witzel (Bauer), were the first to describe retroperitoneal tumors; they did not differentiate between the cystic and solid tumors and gave but little information as to origin. There appears to be some confusion as to who first pointed out the relationship between these cystic tumors and the Wolffian body. Kroenig credits Roth as having been the first to point out their true etiology in 1881 (Maury), while Bauer says that Przewoski in 1886 was the initial investigator to call attention to their connection with the Wolffian and Mullerian bodies. These findings were later confirmed by Obalinsky, Niosi and von Hippel. Since then but few cases are to be found in the literature which show any true morphological relationship. All retroperitoneal serous cysts, retroperitoneal cystadenomata, etc., unless definitely associated with an organ, are put in the same group by most authors.

Case.*—L. D., male, white, age twenty-seven months, was seen because of a swelling in the lower left flank. The family history was negative except that the father

had been treated for lues. The past history showed the child to have had a normal feeding and developmental course. There was no history of any disease.

• This case is reported by courtesy of Dr. Richard F. Tomlinson, San Francisco.

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The present illness began about six months prior to operation when the mother noticed a small swelling in the region of the left flank. This gradually increased in size until at the time of operation it was the size of a small orange. The mother states that at no time were there any gastro-intestinal or urinary signs and symptoms. No hematuria nor other urinary signs were noticed. The child appeared happy at all times and never seemed in distress. There was no loss of weight nor signs of emaciation, except that the mother thought the child to be a little pallid.

The physical examination showed the child to be well developed and nourished. The abdomen was flat, soft and tympanitic throughout. No areas of tenderness were made out. Palpation of the left abdomen easily revealed a painless tumor about the size of an orange which was freely movable, ballotable and seemed semifluctuant in consistency. The left kidney was not felt. The remainder of the examination was negative.

R6ntgen-ray examination of the abdomen showed some vaguely outlined mass to the left of the spine, opposite the third and fourth lumbar vertebre. The urine examination was negative.

The case was seen in consultation with an_ urologist and it was decided that the tumor mass was in no way related to the urinary tract. A diagnosis of chylous or dermoid cyst was made and operation advised. Fic. 1.—Photograph showing external aspect of Wolffian body

Operation was performed cyst. The cavities of the larger and smaller cysts communicate.

The larger cyst measures 614% cm. in diameter, the smaller 2 cm. under gas and ether anesthesia. A midline incision 12 cm. in-length was made. The peritoneal contents were found to be normal. The tumor mass. was easily felt and found to be situated retroperitoneally just caudad and somewhat anterior to the left kidney. No relationship to the left kidney could be made out. The posterior peritoneum was then penetrated and the tumor found to be a cyst about 6 cm. in diameter. No pedicle was found but the attachment was by means of a broad base retroperitoneally. Adjoining this cyst was found a smaller cyst about I to 2 cm. in diameter. The eystic mass was easily shelled out and closure made in the usual manner. The convalescence was uneventful, the child leaving the hospital on the eighth day post-operatively.

Pathological Report. Gross——Specimen (Fig. 1) consists of a tough, thin-walled sac in two parts which communicate with each other. The larger measuring approximately 6%4 cm. and the smaller 2 cm. The thickness of the wall varies considerably, but for the most part is of paper thickness and at no point is more than 5 mm. The lining of the two parts is smooth and shiny, the contents a thin straw-colored fluid.

Microscopic.—Sections of the wall at the thickest point to show the lining of both cysts, reveals a rather dense fibrous wall and a very dense hyalinized connective-tissue

763 HINMAN, GIBSON AND KUTZMANN

lining. Throughout the connective tissue of the wall are a multitude of small or large alveolar structures lined by a cuboidal or low columnar epithelium, numerous structures resembling quite closely the glomeruli of the kidney and a multitude of large, quite thickwalled vessels. (Figs. 2, 3, 4.) An occasional large microscopic cavity which probably represents a small cyst, shows an epithelial lining varying from a single to several layers and taking mostly the low cuboidal form. The structures described in the wall represent primitive glomeruli of the Wolffian body and primitive tubules. The picture is typical of that of a retroperitoneal cyst arising from the anlage of the Wolffian body. Diagnosis: Retroperitoneal cyst of Wolffian body origin. Pathogenesis.—Intraabdominal cysts of various types form the subject of a voluminous literature, chiefly Euro| pean. They are reported as having origin in nearly every structure within the limits of the abdomen, intra-, extra- or retro-peritoneal. Thus there have been described pancreatic, ovarian, parovarian, renal, perirenal, pararenal, mesenteric,

. iS 2.—-Microphotograph (low power) showing thickest portion peritoneal, omental, of cyst wall. A dense hyalinized layer of connective tissue lines j the cyst cavity. Note primitive glomerular and tubular structures 0 MD) halo-mesenteric,

scattered throughout cyst wall. retroperitoneal, Wolffian body (mesonephric), lymphatic, dermoid, hepatic, ecchinococcus, chylous, serous, uni- and multilocular cysts, benign and malignant cystadenomata, as well as cysts arising from remnants of the Mullerian ducts.

Due to the remoteness in point of time and the obscurity which veils the origin of many of these cysts, great confusion has arisen as to their true nature and pathogenesis. It has, therefore, been impossible to review the literature with the hope of selecting those cysts of definite mesonephric origin. This difficulty arises not alone from the variety of locations these cysts may occupy, but from their variegated histo-pathological structure as well. A cyst may be situated in the fused layers of the mesocolon and delude the surgeon into believing them intraperitoneal; or may so closely approximate the gut as to suggest an enteric origin. Moreover, the histological structure may present a variety of pictures quite contrary to that of the normal mesonephric elements.

There exists one definite criterion which denotes a given cyst as being of mesonephric origin and that is the presence of primitive glomeruli or

764 CYSTS OF THE WOLFFIAN BODY

renal tubules in the cyst wall (the cyst, of course, being independent of the kidney). This type of cyst is of the greatest rarity, only four cases having been reported on the American continent (Maury). The literature, however, abounds with reports of cysts which present none of these structures and yet are considered as being of Wolffian origin, either on mere supposition or reasoning by a process of elimination. It is highly probable, and more evidence is gradually being collected to support the view, that a great variety of cystic and solid neoplasms of the retroperitoneal space in relation to the adrenal, the kidney, the broad ligament, spermatic cord, epididymis and possibly the testicle, have their origin in Wolffian body remnants. In the last analysis the etiology of testicular tumors remains unknown and the theory is tenable that such tumors may arise from remnants of the Wolffian body. Bland-Sutton has suggested the paradidymis as the origin of certain testicular neoplasms. In a previous report + the literature on dermoid

Fic. 3.—Microphotograph (low power) of area from cyst wall

cysts of the spermatic showing well formed glomeruli A, and tubules B, resembling closely

thi t .

cord has been reviewed ‘"¢ *d¥lt structure

and their origin considered. Staehlin emphasizes the fact that the pronephric portion of the Wolffian duct arises in part from primitive ectoderm and sug gests this as an explanation of various dermoids seen within the abdomen and along the spermatic cord. In the complex development of the urogenital tract there undoubtedly exists abundant opportunities for maldevelopments, while the numerous fcetal remnants left over in the process of development afford an attractive explanation for these cysts. Definite evidence in support of this hypothesis is furnished by the fact that mesonephric cysts are of much greater frequency in the female sex, a fact explained by Maury as being due to the relatively greater amount of Wolffian body remnants existing in the female as compared to the male. The latter utilizes most of his primitive urinary excretory apparatus in the formation of the vas deferens and epididymis, whereas in the female the major portion of the Wolffian body remains vestigial.

+ Hinman and Gibson: Tumors of the Spermatic Cord, Epididymis, etc. Arch. Surg., January, 1924, p. 100.

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It was stated above that the mesonephros definitely gives rise to cyst formation, as shown by the finding of retroperitoneal cysts with primitive glomeruli and tubules in their walls. These constitute an extremely small group and to these must be added a relatively larger group of retroperitoneal cysts which do not exhibit such structures but exhibit a more or less fibrous wall with an inner epithelial lining, varying from a simple single layer to cyst adenomatous structures. Reasoning by a process of exclusion, Jacquot and Fairise, having made a careful study, have shown that these also must be considered as_ being derived of the Wolffian body. Maury has stated that one is forced to the conclusion that all retroperitoneal cysts not arising from retroperitoneal organs ‘are genetically Wolffian. Jacquot and Fairise have reported such a case in an eighteen-year-old female, a large cyst containing 4 to 5 litres of fluid, situated between the layers of the descending —_mesocolon. They supply a very comprehensive bibliography

Fic. 4.—Microphotograph (high power) of area from cyst wall ‘ : showing more or less differentiated glomeruli in a-matrix of connect- and review the literature,

ive tissue. collecting with their own,

fourteen cases which they consider definite Wolffian cysts.

Clinical—Eliminating the solid retroperitoneal tumors, there remains but a very small group of cystic tumors. If we further eliminate such cysts whose structure morphologically give us no clue as to their origin and confine ourselves to such as do show definite embryologic structures in their walls, it is at once apparent that such tumors are exceedingly rare. Due to the confusion in the literature and the entire absence of clinical statistics relative to incidence, no definite statements can be made. Suffice it to say, cystic tumors similar to the one reported, are most rare.

As far as we can determine they occur almost always in the female. Nor are they confined to any particular period of life as indicated by Maury’s case and ours. The former occurred in a woman of twenty-eight years, while the latter was found in a male child of twenty-seven months. The youngest case in the literature is one of ten months, reported by Albarran, while Kast

766 CYSTS OF THE WOLFFIAN BODY

has reported one in a woman of sixty-five years. For the most part it can be said that they occur chiefly during adult life.

The symptomatology is practically limited to physical signs, especially while the tumor is still small. Due to the diversified locations, the symptomatology may be variable. Their beginning is slow and progressive and may therefore pass unnoticed at first. The occurrence of a blow or fall may quite accidentally call attention to such an abdominal tumor. In children, asymmetrical prominence of the abdomen may cause the parents to examine it. Only when a large size has been attained do we begin to get subjective symptoms. A sensation of fulness with some dull painful radiations in the lumbar region may attract attention to the tumor. Attacks of renal colic have been noted in some cases probably due to ureteral compression. As the tumor increases in size the appearance of pressure signs may become apparent. Again, the location of the tumor is the main factor;

tumors in the renal or

sy: Fic. 5.—Microphotograph (low power) of area from kidney of

perirenal position may 20 mm. human embryo showing resemblance to structure in wall of

give a neuralgia of the Wolfen cyst. twelfth dorsal nerve; then again the situation may be in such relation to the ureter as to produce symptoms of hydronephrosis. Compression of the left renal or spermatic. vein may lead to varicocele formation. Large tumors situated in the pelvis may lead to cedema and varices of the lower limbs. Rarely do ascites, compression of the portal vein or intestinal obstruction occur. If the latter is present the symptoms will be those of a chronic obstruction. Attention has also been called to the fact that urinary frequency may be caused by pressure on the bladder. From the signs noted, it is apparent that exclusive of the size of the tumor itself, the subjective and objective signs and symptoms are essentially those of pressure. That they may attain a huge size is noted by the fact that some cases have reached the capacity .of ten litres.

The diagnosis is essentially one of exclusion. The making of an exact diagnosis is therefore a difficult one. Cases as a rule are only correctly diagnosed at operation or later by the pathologist. If an urological investi 767 HINMAN, GIBSON AND KUTZMANN

gation excludes the tumor from the urinary tract the differential diagnosis still rests on a large number of possibilities such as movable spleen, Riedel’s lobe of the liver, adrenal tumor, solitary cyst of the kidney, benign and malignant peri- and para-renal tumors of a solid nature, lipoma, ovarian cyst, pancreatic cyst, lymphatic cyst, enteric cyst, omental cyst, etc. The history of a long duration is a factor against malignancy, yet certain of these cysts of an adenomatous type may become malignant, resembling ovarian cysts in this respect. These cysts when small are generally freely movable. Likewise they

a at eee are generally smooth in outline, a point against solid malignant tumors. In view of the extreme difficulty of clinical diagnosis, the surgeon can, as a rule, only keep in mind the possibilities. If on surgical exploration a cystic structure is found independent of the postperitoneal organs, it may be considered of Wolffian origin. The contents of the cyst vary with the

Fic. 6.—Microphotograph (high power) of area from kidney of : 20 mm. human embryo. Note the close resemblance to the primitive type, probably depending

renal structures in the wall of the Wolffian cyst. on the portion of the

mesonephros from which it originated. The fluid may be thin, serous, chocolate-brown or pseudomucinous in type. Whether those cysts whose walls show tubules and glomeruli contain urinary elements has not been stated. Treatment consists in early and complete removal, not only because of the inherent tendency to continued increase in size, but also because there is a tendency to malignant degeneration in certain types. The prognosis is an excellent one, excepting in those cases of malignant change and huge size.

SUMMARY

1. Cysts of the Wolffian body are of such rarity as to warrant reporting.

2. The case reported is a true Wolffian body cyst, as evidenced by the finding of primitive renal structures in its wall.

3. Pathologically great confusion exists in the literature, as shown by the large variety of cysts which have been attributed to Wolffian origin. In the absence of any definite relationship to retroperitoneal organs, the presence of all such cysts in that region or between the layers of the mesocolon are probably of Wolffian origin, even though recognizable primitive renal elements are not to be found in their walls.

4. Wolffian body cysts have generally been found in the female. This is probably due to the fact that a greater portion of the Wolffian body and duct

768 CYSTS OF THE WOLFFIAN BODY

become vestigial remnants in the female, whereas in the male it is almost entirely utilized in the formation of the genital tract.

5. Cysts of the Wolffian body may occur at all ages, but especially during adult life.

6. The symptomatology consists chiefly of secondary compression phenomena, varying with the size and location of the tumor. These cysts may grow to a volume of ten litres.

7. Diagnosis is chiefly one of exclusion and is usually made at operation or on later pathological study.

8. Treatment consists of early and complete removal because of the tendency toward malignant degeneration in certain types.

BIBLIOGRAPHY

Albarran: Bull. Soc. de Chir., 1903, pp. 117-119.

Ashhurst and McGuire: J. A. M. A., 1920, vol. Ixxv, p. 1494.

Bauer : Beit. klin. Chir., 1910, vol. Ixx, p. 829.

Dubs: Arch. f. klin. Chir., 1918-19, vol. cxi, p. 860.

Elder: Canad. M. A. J., 1920, vol. x, p. 272. /

von Hippel: Langenbeck’s Arch., vol. Ixxxviii, p. 1008.

Jacquot and Fairise: Rev. de Gyn., 1913, vol. xx, p. 551.

Kast: Rev. de Gyn. et d’Obs., 1904.

Kroenig: Betr. z. Geburtsh. u. Gyn., 1901, vol. iv, p. 61.

Lapointe: Bull. et Mem. Soc. de Chir. de Paris, 1919, vol. xlv, p. 728. Lobstein: Lehrbuch der pathologischen Anatomie, Bd. i, ii, Stuttgart, 1835. Maury: Surg., Gyn. and Obs., 1918, vol. xxvi, p. 663. °

Niosi: Clinica Mod., 1907, pp. 20-24.

Niosi: Virch. Arch., 1907, p. 190.

Obalinski: Wien, klin. Wochschr., 1891, vol. xxxix, p. 719. Przewoski: Gazeta Lekarska, vol. xxiv, p. 820.

Roth: Virch. Arch., 1881, vol. Ixxxvi, p. 371.

Simpson: South. M. J., 1923, vol. xvi, p. 121.

Staehlin: ANNALS or SURGERY, 1915, vol. Ixi, p. 312.

Vignard: Annal. des malad. des org. gen. urin., 1901, vol. xix, p. 1372.

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