Solitary multilocular cyst of the kidney

Solitary Multilocular

Cyst of the Kidney

By K. Aterman, P. Boustani, and D. A. Gillis

I

N 1892 Edmunds’ reported what has been considered to be the first case of a solitary, but multilocular, cyst of the kidney in an 18-yr-old girl under the title of “cyst-adenoma of the kidney” (Fig. 1A). Since then at least 40 cases of this lesion have been described, but it is still considered “very rare,“2 and cannot be viewed as well known. Farman for instance, states that the disease is “most frequently found in adults,” when in fact 18 of 40 cases have been reported in children. The existing uncertainty is illustrated from a different angle by Williams,4 who briefly mentions the occurrence of “multilocular cysts,” but goes on to describe three “lymphangiomas” that may well have been instances of true multilocular cysts of the kidney. The recognition of this lesion is, however, of considerable clinical importance since it must be distinguished, on the one hand, from cystic changes of the “polycystic” or “multicystic kidney” type and, on the other, from Wilms’ or other malignant kidney tumors. It is, therefore, considered of interest to present an additional case of multilocular cyst, and to review the reported instances of this disorder. CASE HISTORY A 2 -yr-old boy developed sudden painless hematuria. An abdominal mass was noted 5 days prior to admission. Four weeks before the onset of hematuria, he had had an upper respiratory infection with bronchitis, treated with penicillin. The past history was otherwise not remarkable. NO history of renal disease in any member was given. The patient was a well-developed, slightly pale, afebrile, white boy with a pulse rate of I IO/min and a blood pressure varying around 15O/lOOmm Hg. The only significant finding was the presence of a rather firm and irregular mass in the left side of the upper abdomen extending 5-6 cm below the left costal margin. The rest of the physical examination did not show any abnormality.

Laboratory Findings Blood. Hg, 13.1 g/l00 ml; hematocrit, 38.5%; WBC, 13,000, with normal differential count; sedimentation rate, I9 (normal: l-9). Urine. Specific gravity, 1.024; pH, 6; no sugar or ketone bodies found; proteins, 30 mg/lOO ml; occult blood, small amount. Microscopic examination: WBC, IO-20/highpower field; RBC, O-30/ high-/power field. No casts or crystals present, but a few epithelial cells were noted in the urine. X-rays. Intravenous pyelogram showed good function of the right kidney, but on the left side the collecting system could not be visualized and a large mass was seen in the region of the lower pole of

From Departments of Pathology and Surgery, Dalhousie University and the lzaak Walton Killam Hospital for Children, Haltfax, Nova Scotia, Canada. K. Aterman, M.D., D.Sc., F.R.C.Path. (London): Professor, Department of Pathology, Dalhousie University. and the Izaak Walton Killam Hospital for Children, Halifax, N.S., Canada. P. Boustani, M.D.: Resident, Department of Pathology, Dalhousie University, and the Izaak Walton Killam Hospital for Children, Halt&, Nova Scotia. Canada. D. A. Gills, M.D., F.R.C.S. (Canada): Associate Professor of Surgery, Daihousie University, and Surgeon-in-Chief, the Izaak Walton Kitlam Hospital for Children. Halifax. Nova Scotia. Canada.

Addressfor reprint requests: Pro/: Kurt A terman, The Izaak Walton Killam Hospital/or University A venue, Haltfax, Nova Scotia, Canada. 8~8 1973 b.v Grune & Stratton, Inc. Journal of Pediatric Surgery, Vol. 8. No. 4 (August). 1973

Children,

505

506

ATERMAN. BOUSTANI. AND GILLIS

Fig. 1. (A) A reproduction of Edmunda” caaa of a “cy$tadanoma” of the kidnay. genomlly accaptad as the first rapottad inatanca of a “multilocular cyst” of the kidnay. (B) The cut saction of the kidnay mass of the prasant caaa shows the typi-I appaaranca of a multilocular cyst of the kidney. Note (1) the similarity of the gross appaaranca to that shown in (A); (2) tha hamiatad daughtar cyst protruding imo the palvis; (3) the hydronephrotic thinning of @a kidney cortex with pyalonaphrftfc, but no other changes.

the left kidney, whose radiolucency gave the impression that it might be cystic. The outline of the up per pole of the left kidney appeared normal. Gross hematuria, hypertension, and failure of excretion on intravenous pyelography are all unusual, although not unknown, in cases of Wilms’ tumor. However, it was felt that this diagnosis had to be excluded by laparotomy, when the left kidney showed a large, seemingly cystic, lesion involving the middle and lower portions of the organ. One anomalous artery supplying the upper pole of the kidney was seen, but the blood supply otherwise appeared normal, as did the right kidney. The whole left kidney with the left ureter was removed. Convalescence was uncomplicated and the boy was normotensive at the time of discharge.

Pathologic Findings Gross examination. The specimen weighed 200 g, and measured 9 by 6 by 6 cm. It showed a kidney distorted by a bulging “knobbly” mass, which, on palpation, felt “cystic.” The bisected specimen (Fig. IB) presented a well-encapsulated mass, about 6 cm in diameter, with numerous cysts of variable size up to about 2 cm in diameter. Most cysts contained clear, colorless fluid, but some appeared bluish. The septa separating the cysts from each other were rather thin. No recognizable kidney tissue was found, but at both poles of the multilocular cyst compressed remnants of kidney tissue were present, which showed hydronephrotic dilatation of the pelvis, thinning of the cortex, and changes indicative of pyelonephritis. Apart from these secondary changes, the kidney tissue itself did not show any cystic malformation. The mass seemed to be encapsulated by fibrous tissue and compressed kidney parenchyma. The pelvis of the kidney was found to be quite separate from the cyst, but in one section a polypoid segment of the cyst had prolapsed into the pelvis and seemed to contribute to the hydronephrotic obstruction and, possibly, to the bematuria (Fig. IB). The ap-

SOLITARY

MULTILOCUIAR

CYST OF KIDNEY

507

pearance was similar to that described by Gibson5 The ureter was patent and no communication with the cyst could be demonstrated. Microscopic examination. The kidney tissue showed typical hydronephrotic and pyelonephritic changes but it must be emphasized again that neither cystic malformation nor heterotopic elements were seen in the kidney tissue itself. Sections of the cystic mass showed multiple cysts of variable size (Fig. 2A) containing eosinophilic debris and also some desquamated cells, and separated from each other by connective tissue septa of variable thickness. The lining consisted of one layer of flat or low cuboidal cells (Fig. 28) on a basement membrane that was clearly brought out in periodic acid-Schiff and methenamine silver stains. The lining cells were clearly different from the flat, spindle-shaped fibroblastic cells seen in close association with the basement membrane. The septaof the cysts consisted of rather homogeneous-appearing connective tissue (Fig. 2C) containing fairly plump fibroblasts and inflammatory cells, predominantly lymphocytes with some plasma cells. Smooth muscle could not be convincingly demonstrated,2 but occasional elastic fibers could be seen. On no occasion were glomerular elements found in any of the septa, but several tubular formations were identified. They consisted of rounded or oblong slits lined by cuboidal cells and contained variable amounts of eosinophilic debris in their lumen. Occasionally an aggregate of such tubules could be found (Fig. ZD), not only in the border between the compressed kidney and the cyst, but also at some distance from the border in the septa themselves. They were not, however, found in sections of the central portions of the cyst. It was not possible to distinguish the tubules from similar tubules in the adjacent compressed kidney tissue, and their presence here should not be taken to indicate the existence of “primitive” elements in the septa described by some workers. The possibility cannot be excluded that we are dealing with surviving remnants of replaced kidney parenchyma. Occasionally an impression was gained that the inflammatory cell infiltrate mentioned was associated with residual atrophic or distended tubules, indicating perhaps a continuing process of tubular obliteration. No heterotopic elements, such as striated muscle, bone, or cartilage, were found in the cyst. DISCUSSION

Powell et aL6 have suggested eight criteria that they consider characteristic features of the “solitary multilocular cyst” of the kidney: (1) The cyst should be unilateral. (2) It should be solitary. (3) It has to be multilocular. (4) The loculi should not communicate with one another. (5) The loculi should have an epithelial lining. (6) The cyst should not communicate with the renal pelvis. (7) The remaining kidney should be normal. (8) There should be no renal elements within the main cyst. The last point has been qualified by Boggs and Kimmelstiel,’ who state that no fully developed nephrons or portions of nephrons should be present in the septa of the cystic lesion. The presence of embryonic elements, however, has been reported by several workers,5*7-g and has been used as evidence in favor of the view that the multilocular cyst is a true tumor5 rather than a hamartoma.‘O More recently, Fowler” even suggested that these formations were indicative of the maturation of the nephrogenic blastema to the benign, tumorous, multilocular cyst. It is widely assumed that Edmunds’ reported the first instance of multilocular cyst of the kidney. However, apparently earlier workers described what they considered to be “lymphangiomas” of the kidney. Perlman,12 for instance, has described such a “lymphangioma,” which fulfills the criteria of a multilocular cyst. Today there are no more reliable distinguishing criteria available that would enable a reviewer at this stage to separate these two entities with certainty, than there were when Fahr and Lubarsch13 discussed this difficulty, and one may well raise the question as to whether such a distinction can and should be made at all.

ATERMAN,

BOUSTANI,

AND

GILLIS

Fig. 2. (A) Low-power microphotograph to show the multilocular nature of the cyst and the variable thlchneu of tha dividing aapta. 1096 buffared formalin, 78, hematoxyfin and aosin, x 20. 18) Microphotograph of the lining callr of the cyst. Note the ffattening of the cells toward the right upper comer. Many cysts am lined by completely Sat cells, so that the appoaranca of the lining crlfs cannot always be used as a reliable distinguishing future; 10?4 formalin, 7~. hematoxylin and eosln. x 510. (C; opposite Yipage) crophotogmph of septum dividing two adjacent cysts. Note the loose connective tissue between the two layers of denser, eoslnophilic tlssuo immadiately below the lining of the cysts; 1096 formalin, 7A, hematoxylin and eosin. x 190. (D; opposite page) Microphotogmph to show an aggmgatq of tubules in one of the septa dividing the cysts. Note atrophic appaarance of the tubules and the scattered inflammatory round cells. A few thin-walled vessels are alao seen; 10% formalin, 71, periodic-acid-Schiff, x i

90.

SOLITARY

MULTILOCULAR

CYST OF KIDNEY

509

See legend on facing page.

“Needless to say, great caution is needed before diagnosing as lymphangiomatous a cystic growth in an epithelial organ.“14*15Be that as it may, following the description by Edmunds’ 17 instances of multilocular cyst of the kidney have been reported in the pediatric age group, and 22 instances in adult patients.‘~5~6~‘2~*6--26 The pediatric cases are summarized in Table 1. It is very probable that the incidence of this disorder is greater than the number of published cases would lead one to believe. Lattimer,27 for instance, mentions that in a series of 650 children with abdominal masses he found six instances of

1951

1951

1956

1956

1957

1960

Frariers

Frazier%

Boggs and Kimmelstiel’

Boggs and Kimmelstiel’

Moore4’

Uson. del Rosario and

5.

6.

7.

8.

8.

1964

1971

1972

Williams4

Chri#

Faur4”

Fowler”

Present case

14.

15.

16.

17.

18.

1967

1967

1964

Williams*

13.

1963

Uson and Melicow”

Economou ’

Dainko. Dammers and

12.

11.

1963

1949

Carveras

4.

Melicow3’

1945

Chauvin ”

3.

10.

1942

right kidney

and abdominal

2 l/2

yr

2yr 13mo

10mo

Not stated

Not stated

9mo

22 mo

ture quoted without references.

Left

M

and abdominal mass

Hematuria.

hypertension.

Radiotherapy inel mass

Constipation and abdomLeft

petvis. Hypertension

Hemiation of a daughter cyst into the renal

10 days before nephrectomy

nephroblastoma”

“polycystic Illustration shows typical multilocular cyst.

Microscopic: Not stated

before operation. Right

given

(probably multilocular cyst)

“Lymphangiomas”

Actinomycin 0 and radiation treatment

(probably multilocular cyst)

“Lymphangiomas”

mild hydronephrosis. 5-yr follow up

Daughter cyst herniated into the renal pelvis;

locular cyst. Follow-up period: 6 yr

Pathological diagnosis: Wilms’ tumor in multi-

surgery

Presented with intussusception. died after

pattern

Cellular connective tissue with organoid

Enuresis since birth

medulla

small cysts at the junction of cortex and

A collection of multilocular cysts. Several

M M

Renal mass

Renal mass

Abdominal mass

Abdominal mass

before surgery Several additional cases in the French litera-

Abdominal mass

Laft

Right

Clinical impression: Renal

embryoma. received deep x-ray therapy

130/80

Sustained hypertension. Blood pressure.

Remarks

Right

F

M

F

Abdominal mass

Left

F

9mo

Abdominal mass

Len

F

19 mo

Abdominal mass

Left

Abdominal mass

mass

Hematuda

Abdominal mass

enlarged

Hematuria,

mass

Hematuria and abdominal

Abdominal mass

Hematuria and fever

Presenting Signs or Symptoms

M

Left

left

M M

Left

M

Right

Right

Left Left

Side Involved

11 mo

5mo

6mo

yr

F

12 mo

3 l/2

F

M

M

Sex

yr

4 l/2

3 yr 19mo

1940

Burreli’+’

Toulson and Wagner4’

Ags

Date

1.

Reference

2.

No.

SOLITARY

MULTILOCUIAR

CYST OF KIDNEY

511

solitary multilocular cysts. De Weerdz8 briefly states that he encountered two cases of multilocular cyst formation in the kidney. Uson and Melicowz5 report that they have seen the files of ten cases of multilocular cyst, of which five were children. These cases have not been included in the present survey, since the pertinent details are lacking. Williams4 described three instances of what he considered to be “lymphangiomas” of the kidney in children. His brief description strongly suggests that he may well have been dealing with multilocular cysts, one of which in addition, as was the case in the instance presented here, showed prolapse of a cyst into the renal pelvis. Since Williams,4 however, states that one of these children also had a similar “lymphangiomatous mass” in the lung, this case was excluded from the present survey, and only the other two “lymphangiomas” were considered as possible instances of multilocular cyst, keeping in mind the difficulty of distinguishing between “lymphangiomas” and “multilocular cysts of the kidney.” Cases of multilocular cysts referred to in the Japanese literature were not accessible to us, and we have not reviewed the eight cases quoted by Chauvin, 2Qalthough, judging by the description of his own case, he clearly recognized the entity. One major difficulty that reviewers of this disorder encounter is the fact that authors frequently will speak of “multilocuiar cysts” without applying the strict criteria laid down by Powell et al.s A “congenital multilocular renal cyst in a newborn infant,” for instance, has been described30 that cannot be accepted here, because renal tissue was not identified. The different names applied to this or similar cystic conditions-multicystic kidney, multilocular cysts, cystadenoma, lymphangioma of kidney, partial polycystic kidney, focal polycystic kidney-make it unwise to include some instances of allegedly multilocular cysts in this review. A “multicystic” kidney, for instance,5*Q*32which, by definition, is unilateral, would not come under the heading of “multilocular solitary cyst,” since additional criteria necessary for this diagnosis are missing. Some of the other published descriptions probably indicate polycystic kidneys. Even the case described by Haslinger, wrongly quoted and interpreted by Boggs and Kimmelstiel’ and by Fowler,” cannot be included here despite the presence of seven of the eight criteria listed; the cyst described by him appears to be a pararenal cyst. The “lymphangioma” of the kidney reported briefly by Willis14 in the second, but omitted in the fourth, edition of his bookI may well have been an instance of a multilocular cyst but, like the two instances of “lymphangioma of the kidney” briefly mentioned by Luck6 and Schlumberger, 34 has not been included here. The cases reviewed are those in which we are more or less satisfied, on the basis of either the authors’ descriptions or their illustrations, that we are dealing with true instances of solitary multilocular cyst of the kidney; no claim for completeness of this survey, however, can be made. If all the instances reviewed are graded according to age an interesting feature emerges. Multilocular cysts can occur at widely separated ages, the youngest patient being 5 mo old and the oldest 70 yr old. The oldest case in the pediatric age group is a 5-yr-old female, reported in the Japanese literature, and the youngest case in the adult group appears to have been 18 yr old. The significance of this gap in age is not clear. Perhaps instances in the intermediate age group have not been reported. On the other hand, the possibility cannot be ruled out that this

512

ATERMAN.

BOUSTANI.

AND

GILLIS

difference may not be simply a chance finding, but may have a real significance, since it has been claimed9 that some instances of multilocular cyst may have been acquired rather than congenital. Another feature of interest is that the adult group involves more than twice as many females as males. In the pediatric age group, on the other hand, nine children were males as compared with six females. However, the significance of the greater incidence of multilocular cysts in females of the adult group is uncertain. PATHOGENESIS

A review of some of the older theories of the pathogenesis of this disorder can be found in the papers by Boggs and Kimmelstiel’ and Dainko et aLs The views of Boggs and Kimmelstiel’ are of particular interest not only because they critically examine, and dismiss, some of the suggestions advanced, but also because they themselves have put forward the view that the multilocular cyst should be considered “a tumorous multilocular cystic nephroma.” This suggestion, which apparently had been considered already by Jenckel,r6 has recently been reiterated by Fowler, ‘r who, like Boggs and Kimmelstiel,’ points to a possible relation of the multilocular cyst containing embryonic tubular elements to the fully developed nephroblastoma known as “Wilms’ tumor.” According to Fowler,” there exist degrees of differentiation in some of the nephroblastomas reported and hence, conceivably, the multilocular cyst represents one stage of this process and the fully developed nephroblastoma another. WilliP has also emphasized the possibility of maturation of a nephroblastoma, which led Fowler” to paraphrase Wilis’15 view of ganglioneuromas by stating that “every nephroma must once have been a nephroblastoma.” The relationship of the multilocular cyst to the “congenital mesoblastic nephroma of infancy” described by Bolande et a1.3ein this scheme is not clear or, for that matter, necessarily relevant. What does matter is the view that the multilocular cyst is a matured nephroblastoma or its analog. This view is based on stated or implied assumptions that have by no means been proved. There is, for instance, no real evidence to show that the multilocular cyst always is a congenital lesion.37 So far, not a single instance has been reported of a multilocular cyst in a premature infant or a fetus, and only one case of what may be a multilocular cyst in a newborn infant has been described.38 Unfortunately, further details would be needed to assess this case, which therefore has not been included in Table 1. On the other hand, several cases of multilocular cyst have been reported in people past the age of 60 yr. Moreover, Uson and Melicowz6 have reported the case of a patient returning with a multilocular cyst 4 yr after a urologic investigation, during which he was thought to have “morpholocially normal kidneys.” Boggs and Kimmelstiel’ have pointed out that the metanephric blastema may remain dormant for a considerable period before assuming its “nephromatous” potential. This interpretation could explain the fact that multilocular cysts, like nephroblastomas,3s can occur in persons past childhood, but it does not explain the striking gap in the age incidence of reported cases referred to earlier; nor can it explain why, with one exception quoted by Fowler,” none of the cases in the older group have shown the obvious embryonic remnants that one would expect to find in at least some instances, if “maturation” from a

SOLITARY

MULTILOCUIAR

CYST OF KIDNEY

513

more primitive stage was a factor in the development of these lesions. It is precisely the presence of “embryonic” or “primitive” tubules in the walls of some cysts that has led Boggs and Kimmelstiel’ and Fowler” to postulate a relationship between the multilocular cyst and the true nephroblastoma. It may, therefore, be pertinent to point out that even when tubules can be found in a multilocular cyst, it is not certain that they are indeed “primitive.” Interestingly enough, Bolande also had some misgivings about the “embryonic” nature of these structuresin the tumors reported by him and his associates.36 Also disturbing is the fact that, in contrast to the frequent presence of “heterotopic” cartilage or striated muscle in Wilms’ tumor, no such elements have been described in the multilocular cyst, if the casual and apparently unsubstantiated statement of Dainko et al.,s that cartilage occasionally is found, is excepted, although KissaneZ states that he has seen in one instance “dysontogenetic cartilage” in the capsule of a multilocular cyst. The smooth muscle that has been mentioned in several reports can hardly be considered as heterotopic, since smooth muscle fibers have been found in the subcapsular regions and around the renal veins of normal kidneys.17.3s Finally, it is difficult to see why the process of maturation of the nephroblastomatous tissue should lead to the invariable development of multiple cysts whose structure would be difficult to distinguish histologically from the cysts in some of the other variants of “cystic kidneys, ” where “maturation” has not been invoked. There are, on the other hand, reports in the literature in which the histologic examination of what grossly appeared to be a typical multilocular cyst of the kidney led to a firm diagnosis of Wilms’ tumor.27*40One such instance has recently been seen by one of us (Aterman) at a meeting of the Canadian Association of Pathology. Uson et al. 37 have given a detailed account of one case, and state that in a series of 60 cases of renal tumors in children they found only one Wilms’ tumor associated with a multilocular cyst. The views of Boggs and Kimmelstie17 and of Fowler” have already been mentioned. It must be conceded, therefore, that, in at least some instances, a multilocular cyst can be associated with Wilms’ tumor. It is only the nature of this association that needs further clarification. Christ40 considered, but rejected, the possibility that the association between Wilms’ tumor and multilocular cysts may be merely coincidental, and concluded that the two lesions may be developmentally related, with tumors having features of both these conditions representing borderline lesions. Uson et aL3’ however, thought that pure Wilms’ tumors and cystic lesions of the kidney were the result of two separate and different developmental errors, which, in combined lesions, simply coincided. The well-known association of Wilms’ tumor with other congenital anomalies of the kidney3s,40 or elsewhere is in keeping with the view of Uson et a1.37The possibility that Wilms’ tumor develops in a kidney with a multilocular cyst precisely because such an anomaly, like other congenital anomalies, predisposes to the development of Wilms’ tumor, can by no means be dismissed. Conversely, the association of these two features cannot be taken as necessarily denoting a causal connection. In the adult a somewhat similar problem has been considered by Pearlman4* who discussed the simultaneous occurrence of renal cysts and renal carcinoma. Interestingly enough, one of his cases was that of a woman, aged 50 yr, who was shown to have a renal

514

ATERMAN.

BOUSTANI,

AND

GILLIS

adenocarcinoma and a multilocular cyst. Chauvin2g mentions another such case. Farman states that according to Weingarten “about 7% of all cases of renal cystic disease are malignant.” Until such time that more conclusive evidence can be adduced in favor of the view of Boggs and Kimmelstiel? and of Fowler” that the multilocular cyst is a stage in the maturation of nephroblastomas, this interpretation should be viewed at most as an interesting hypothesis. From the practical point of view, however, the possibility of an underlying malignant condition must always be kept in mind. Brief mention should be made of some of the clinical features (Table 1). The most common presenting feature is an abdominal mass that is found on routine examination or when the patient is referred to a physician for some other reason.7 In four of the cases in the pediatric age group reviewed here, hematuria was the first symptom, although on examination these cases also showed an abdominal mass. That finding led to the pediatric cases most frequently being misdiagnosed as Wilms’ tumor, although hematuria occurs only comparatively rarely in Wilms’ tumors. This diagnosis has even led to radiotherapy with or without Actinomycin In most instances a definite D treatment being given prior to operation. 11*40*42 diagnosis will be made only at or after operation, although appropriate investigations may help to narrow down differential diagnostic choices. A plain film of the abdomen may occasionally suggest the presence of cystic spaces in the tumor mass. In the case presented here, a suspicion of cyst formation in the kidney arose on x-ray examination of the abdomen. Occasionally, prolapse of part of the cyst into the renal pelvis or the ureter may lead to the creation of a filling defect on retrograde pyelography,5 which should arouse suspicion. Fine28 described two such instances, and reviewed several other published reports of this occurrence. Surgical exploration, however, will still be indicated in most instances because of the uncertainty in diagnosis, and because of the higher probability of an abnormal kidney developing a neoplasm. 8*25*43 A simple excision of the cyst may be undertaken in cases of small multilocular cysts or if there is reason to believe that the other kidney may not be able to compensate, although in most instances a nephrectomy will be done because of the danger of a coexistent malignancy. If such a malignancy is found, treatment should be directed toward the potentially malignant tumor rather than the cystic lesion whose outlook so far has always been good. It is in this context that the concept of maturation may induce a false sense of security, as has been pointed out in the somewhat analogous case of neuroblastomas.44 A Wilms’ tumor in a multilocular cyst should therefore still be considered malignant, although metastatic lesions have so far not been reported in the few recorded instances in which this unusual association has been found. SUM MARY

Solitary multilocular cysts of the kidney are considered to be rare lesions. An instance of this unusual entity occurring in a child of 2 yr of age has been described, and compared with other reported instances. Eighteen such cases in the pediatric age group have been reviewed and compared with 22 similar cases in adults. The pathogenesis of this lesion is discussed with particular reference to the hypothesis currently proposed by other workers, that there exists a distinct developmental association between Wilms’ tumor and multilocular cysts of the kidney.

SOLITARY

MULTILOCULAR

CYST OF KIDNEY

515

ACKNOWLEDGMENT The illustrations were prepared by the Audio-Visual Departments of the Izaak Walton Killam Hospital for Children and of Dalhousie University, to whom thanks are due. The secretarial assistance of Miss Helen Butt is gratefully acknowledged.

REFERENCES I. Edmunds W: Cystadenoma of the kidney. Trans Path Sot London 43:89, 1892 2. Kissane J: Congenital malformations, in Heptinstall R (ed): Pathology of the Kidney. Boston Little, Brown 1966, p 63 3. Farman F: Anomalies of the kidney, in Alken CE et al (eds): Malformations, vol VII/pt I, Handbuch der Urologie. Berlin, Springer, 1968,pSl 4. Williams D: Nephroblastoma. Clinical picture and diagnosis, in Smithers DW , Rich E (eds): Tumors of the Kidney and Ureter, Edinburgh, & London, Livingstone, 1964, p 235 5. Gibson T: Multilocular cyst of the kidney. Case report. Trans Am Assoc Genitourin Surg 53:53, 1961 6. Powell T, Shackman R, Johnson H: Multilocular cysts of the kidney. Br J Ural 23: 142, 1951 7. Boggs K, Kimmelstiel P: Benign multilocular cystic nephroma: Report of two cases of so-called multilocular cyst of the kidney. J Urol 76530, 1956 8. Frazier T: Multilocular cysts of the kidney. J Urol65:351, 1951 9. Dainko E, Dammers W, and Economou S: Multilocular cysts of the kidney in children. Report of a case and review of the literature. J Pediat 63:249, 1963 10. Arey JB: Cystic lesions of the kidney in infants and children. J Pediat 54:529, 1959 I 1. Fowler M: Differentiated nephroblastoma. Solid, cystic or mixed. J Path 105:215, 1971 12. Perlmann S: Uber einen Fall von Lymphangioma cysticum der Niere. Virchows Arch 268:524, 1928 13. Fahr T, Lubarsch 0: Die gewebsgleichen (homologen), fur gewiihulich nicht destruierend wachsenden Gewachse van ausgereiftem Typus. in Henke F, Lubarsch 0 (ed) Handbuch der spcziellen pathologischen Anatomie und Histologie. Vl VI/Pt I: Niere. Berlin, Springer, p 589, 1925 14. Willis R: Pathology of Tumors. (ed I). London, Buttersworth, 1948 15. Pathology ofTumors. (ed4). London, Buttersworth, 1967, p 877 16. Jenckel A: Beitrag zur Kenntniss der so-

genannten emhryonalen Driisengeschwtilste der Niere. Dtsch Ztschr Chir 60:$00, 1901 17. Reinecke R: Solitare vielkammerige Cyste der Niere. Virchows Arch 254:425, 1925 18. Ravich A: Multilocular tuberculous cyst of kidney. Resection and recovery. J Urol 25:223, 1931 19. Fawcett A: Case of congenital cyst adenoma of kidney. Br J Surg 20:678, 1933 20. Meland E, Braasch W: Multilocular cysts of kidney. J Urol29:505, 1933 2 I Lieberthal F: Multilocular solitary cyst of renal hilus. J Uro142:321, 1939 22. Heckel N, Gould H: Report of papillary cystadenoma of kidney. J Urol44:200, 1940 23. Wynn-Williams D, A: Morgan Lymphangioma of the kidney. Br J Surg 37:346, 1949-50 24. Gibson T: Multilocular cyst of the kidney. Case report. J Ural 87:297, 1962 25. Uson A, Melicow M: Multilocular cysts of the kidney with intrapelvic herniation of a “daughter” cyst. Report of 4 cases, J Ural 89:341, 1963 26. Fine M: Intrapelvic presentation of renal cysts. Report of two cases and discussion of the literature. J Ural 91:325, 1964 27. Lattimer J: In discussion of T. Gibson. Multilocular cyst of the kidney. Case report. Trans Am Assoc Genitourin Surg 53:53, 1961 28. De Weerd J: Percutaneous aspiration of selected expanding renal lesions. J Ural 87:303, 1962 29. Chauvin M: Un cas de rein polykystique partiel. J d’Urol52:210, 1945 30. Case report No. 105: Congenital multilocular renal cyst in a newborn infant. Clin Proc Child Hosp (Wash, DC) 3:248, 1947 31. Spence R: Congenital unilateral multicystic kidney. An entity to be distinguished from polycystic kidney disease and other cystic disorders. J Ural 74:693, 1955 32. Muecke E, Marshall V: Multicystic kidney. Case 3. Cystic Diseases of the Kidney. Conferences in Pediatric Urology. Baltimore, Williams & Wilkins 1969, p 40 33. Haslinger J: Eine multilokullre Nierenzyste. Wien Klin Wochenschr 19:534, 1926 34. Luckt B, Schlumberger HG: Tumors of

516

the kidney, renal pelvis and ureter. Atlas of Tumor Pathology, Section VIII, fascicle 30, Armed Forces Institute of Pathology, Washington, D.C., 1957 35. Willis R: The Pathology of the Tumors of Children. Edinburgh, London, 1962, ~0 51-57 36. Bolande R, Brough A, Izant R: Congenital mesoblastic nephroma of infancy. A report of eight cases and the relationship to Wilms’ tumor. Pediatrics 40272, 1967 37. Uson A, de Rosario C, Melicow M: Wilms’ tumor in association with cystic renal disease: Report of two cases. J Ural 83:262, 1960 38. Osathanondh U, Potter E: Pathogenesis of polycystic kidneys. Arch Path 77:474, 1964 39. Zollinger HU: Niere und ableitende Harnwege, in Doerr W, Uehlinger E feds): Spezielle Pathologische Anatomie. Berlin, Springer, 1966, p 3 40. Christ M: Polycystic nephroblastoma. J Urol98570, 1967

ATERMAN.

BOUSTANI.

AND

GILLIS

41. Pearlman C: Coexisting renal carcinoma and cyst. J Int Surg41:620, 1964 42. Toulson W, Wagner J: Congenital encapsulated multilocular serous cyst of kidney associated with hypertension. Occurrence in a nineteen month old infant. Bull School Med., Univ. Md. 26: 177, 1952 43. Ackerman LV: Surgical Pathology (ed 2). St. Louis, Mosby, 1959 44. Aterman K, Schueller E: Maturation of neuroblastoma to ganglioneuroma. Am J Dis Child 120~217,1970 45. Burrell N: Multilocular cyst of the kidney. Report of a case. J Urol43:656, 1940 46. Carver J: Cystic disease of the right kidney in an infant. Br J Urol21:229, 1949 47. Moore T: Unilateral cystic kidney. Br J Urol29:3, 1957 48. FaurC C: Les maladies kystiques des reins chez l’enfant. J Can Assoc Radio1 18:356, 1967