CARCI~OMA I~ CROSSED RENAL ECTOPIA HOWARD T. LANGWORTHY
AND
LEO S. DREXLER
From the Urological Service, C1imberland Hospital, Brooklyn, N. Y.
Crossed renal ectopia must be differentiated from unilateral fused or unilateral double kidney. In the former, 2 kidneys are found on the same side of the body, the ureters, however, empty into their normal positions in the bladder, while in the latter condition the ureteral openings are always adjacent to each other with no opening on the opposite side of the trigone. In crossed renal ectopia the kidney may be fused or unfused. The fused type is more common. A search of the literature reveals 20 cases which in all probability are true cases of the unfused type of crossed renal ectopia. This anomal~r predominates in the male, and the left side is more frequently involved than the right. Single congenital ectopia occurs during the development of the metanephros and is due either to the lack of growth of the premature ureter or the persistence of the early mesonephros. The kidney migrates upward, following the line of the degenerating mesonephros. With this, deviation of the kidney occurs. The axial rotation is completed at the end of the second month. The network of the rudimentary mesonephric arteries has been described as a ladder upon which the kidney makes its ascent so that as the upward rise of the kidney is arrested, the arteries that supply the kidney at this point will remain as the permanent blood supply. Because of this an anomalous source of supply is exceedingly common. The vessels are usually multiple and, when the kidney is in the pelvic position, arise from the iliac, sacral or inferior mcsenteric arteries. How these kidneys are drawn to the opposite side of the body has never been satisfactorily explained. Mayer suggests that rotation of the intestine plays an important role in this migration. The arrangement of the kidneys is usually longitudinal, more rarely transverse. The ectopic kidney may be normal or hypoplastic in appearance. Its position is usually below that of the normal kidney and as Beer stated, "seems to have slipped across the spine to its new position." The ectopic kidney, whether cro;3sed or uncrossed, becomes diseased in about 30 per cent of the cases. Poor drainage is associated either with congenital malposition or constriction accompanied by abnormalities in its blood supply, so that chronic nephritis, hydronephrosis, calculi, pyelonephritis, pyonephrosis and tuberculosis have been reported as occurring in this type of kidney. Carcinoma of the kidney, to our knowledge, has never been reported in crossed renal ectopy, either in the fused or the unfused types. vVe are herewith reporting 2 cases of crossed renal ectopia associated with carcinoma. CASE REPORTS
Case 1. A. Y., male, aged 47, married, was admitted to the Cumberland Hospital March 18, 1940, complaining of severe pain in the lumbar region. This pain had been present for 2 months as a dull ache in the left renal area, radiating 776
CARCINOMA IN CROSSED RENAL ECTOPIA
777
to the groin, scrotum and left thigh, finally becoming colicky in character. The patient complained of urinary frequency, but there was no dysuria or hematuria. The past personal history was essentially negative. The patient had never had any serious illnesses or surgical operations. No loss of weight had been noticed. However, there were vague digestive symptoms for a period of 6 months. Physical examination revealed a well-developed and well-nourished man. His temperature on admission was 102.8°F., pulse 100, respirations 28. Physical examination was essentially negative except for definite tenderness in the left lower quadrant of the abdomen. No masses were palpable abdominally. The urine on admission was turbid. Microscopic examination disclosed the presence of numerous pus and red blood cells with many Gram-positive bacteria. Laboratory Data: Hemoglobin, 74 per cent; red blood cells, 4,500,000; white blood cells, 6,100; polymorphonuclears, 82 per cent; small lymphocytes, 4 per cent; large lymphocytes, 14 per cent; blood Wassermann, negative. Blood chemistry: Sugar, 95.2 mg., urea nitrogen, 12.8 mg. The diagnosis on admission was left renal calculus and pyelonephritis. The patient was placed on sulfapyridine, 15 grains every 4 hours. The temperature dropped to normal in 48 hours and the urine became clear. On March 20 an x-ray and intravenous pyelogram were done. X-ray revealed the presence of a large shadow in the right lumbar area which was thought to be a mesenteric lymph node. An intravenous urogram disclosed the presence of a normal kidney on the left side. No evidence of dye was noted on the right side. On March 26 a cystoscopy was done. No pathologic process was noted in the bladder. Both ureteral orifices appeared in their normal location. The left ureter was catheterized to the renal pelvis without difficulty and clear urine obtained. The right ureter was catheterized for about one-third its distance. Drainage from this side was poor. Indigo carmine injected intravenously returned in good concentration on the left side. No return was noted on the right side at the end of 15 minutes. X-ray showed the left catheter to be in normal position. The right catheter was observed to be pulled to the left side and obstructed at the level of the sacrum. A soft shadow was seen at this point on the left side. A bilateral pyelo-ureterogram disclosed a normal pelvis, calyces and ureter on the left side. The right kidney was seen lying transversely and extreme deformity was noted in the pyelogram. The diagnosis at this time was crossed renal ectopia with an associated hypoplasia and infection of the ectopic kidney. On March 29, 1940, a midline abdominal incision was made through skin, fascia and muscles. The peritoneum was opened and the intestines were walled off. The pelvic kidney was easily palpable and was found to be deep to the left iliac fossa between the rectum and the bladder. The posterior peritoneum was opened at this point. The kidney was found adherent and was freed up from the surrounding peritoneal adhesions. The ureter ran anteriorly and could be traced from the renal pelvis across the sacrum to the right side. The left ureter could be palpated laterally. The ureter leading to the ectopic kidney was
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HOWARD T. LANGWORTHY AND LEO S. DREXLER
clamped, cut and doubly ligated. The vessels were multiple and seemed to arise from the external iliac on the left side. The vessels were clamped, doubly ligated and cut. The parietal peritoneum was closed and the wound then closed without drainage.
Fm. 1
Frn. 2
Frn. 1. X-ray taken with opaque catheter in place. Showing left ureteral catheter going to left kidney in its normal position. Right catheter crosses the sacrum. Calcified shadow in right Ium bar area is of an extrarenal character. Fm. 2. Bilateral pyelogram revealing moderate dilatation of left renal pelvis. Bizarre filling defect of ectopic kidney is noted.
Frn. 3. Pyelogram taken after ectopic kidney had been removed, showing; filling defect in renal pelvis.
The specimen was a kidney measuring 10 by 7 .5 by 6 cm. Its external surface was pink in color. The capsule was thickened and stripped with difficulty. The organ was firmer than normal and most of it was of a very tough consistency. On section the lower two-thirds of the kidney were found to be replaced by a yellow-gray mass which was neoplastic and presented a granular cut surface.
CARCINOMA IN CROSSED RENAL EC'l'OPIA
779
Several papillae of normal kidney tissue were discerned in the upper pole. The renal artery and veins were normal. Microscopic study of sections of the tissue removed showed the kidney to be the seat of a malignant neoplasm. The renal capsule was normal and the remaining cortex showed recognizable normal glomcruli and tubules. The normal areas, however, merged with groups of large, irregular cells containing atypical hyperchromatic nuclei, many of which showed mitotic figures. The8e ce!l8 8howed definite tendency toward alveolar formation resembling tubules. In some areas cords of large, pale vacuolated cells rescmbliag a hypcrnephroma, were present, but the:c;e were not common. The tram,itional epithelium of the kidney pelvis was hyperplastic and in :c;ome areas showed papillary outgrowth:s. There was extensiYc necrosis and moderate hemorrhage within the tumor tissue. Diagnosis: Adenocarcinoma of the kidney.
Frn. 4. Photograph of kidney, showing capsular (A) and cut (B) surfaces. Arrow points to ill-defined margin between normal upper pole and tumor mass which occupies major portion of organ.
The question aro8e as to whether we were dealing with a carcinoma of an ectopic kidney lying in the transverse position. The x-ray, however, shcnved the right ureter to be displaced to the left. On exploration of the kidney the calyees seemed to point to the left side. This conformed to the distinction described Lowslcy and Kirwin that the ealyces almost always point toward the side where the kidney should properly lie. The arterial supply also was seen to come from the left side. The patient had no reaction from the operation and went on to an uneventful rccover)r. The wound healed by primary intention. In spite of this, however, pain in the back and left leg persisted. X-ray of the long bones and lungs was entirely negative except for evidences of metastases. The pain in the back and leg persisted and the patient was transferred to the Kings County Hospital for post-operative x-ray therapy. The patient was admitted to the Kings County Hospital April 29, 1940, and a
780
HOWARD T. LANGWORTHY AND LEO S. DREXLER
course of deep x-ray therapy was given. At the completion of this course of therapy he still complained of severe pain and eventually x-ray examination revealed osteolytic metastatic processes in the left inferior ramus of the pelvis and ischium. At a later time a pathologic fracture of the left femur was found with bony metastasis to the right and left inferior rami of the pelvis. The patient expired September 21, 1940. Case 2. E. C., a white male, aged 75, single, was admitted to the Cumberland Hospital April 9, 1941, with a chief complaint of hematuria of 5 days' duration. The patient complained of feeling very tired and had little or no desire for food. He had lost 4 pounds during the preceding month. This was patient's second admission to the hospital. At the time of his first admission (June 6, 1937) he complained of pain in the right lower quadrant of the
FIG. 5. Photomicrograph of renal cortex, showing glomeruli at junction with tumor mass. Note tendency of neoplasm to form tubular structures, as indicated by arrows. (- 200).
abdomen. On examination extreme spasticity was noted in this region. The temperature was somewhat elevated and there was an associated leukocytosis. A diagnosis of acute appendicitis was made and a laparotomy was performed on the day that the patient entered the hospital. Description of the operative procedure and findings disclosed follow: "A 4½-inch right rectus incision was made to the outer side of the rectus, through the skin and fascia. The muscle was pulled medially and the peritoneum incised in the line of the skin incision. The appendix was found to be about an inch and a half in length and was normal in appearance. Immediately behind the cecum, retroperitoneally, there was a hard multilocular mass which was movable upward and downward. This mass presented several white ish areas on its surface, alternating with bluish discolored areas. The upper polof the mass felt like kidney, but the kidney pedicle could not be palpated. The
78] gall bladder was normal to palpation. No other ma:,,scs were noted. Tbe mescntcr~' of the small inte:,,tine was nodular in appearance. The peritoneum on the lateral side of the cecum was incised and the cecum was cli:-,Hected off the pociterior parietal peritoneum. The mass was dissected free. Because of the absence of a kidney functional test on the left side, a biopsy ,Yas taken frmn the :mrface of the mass, and the nnY area closed over with an atraumatic suture. The appendix was not removed." Pathological report of the biopsy: Specimen consi:-,ts of 2 irregular pieces of grn~' ti,ssue, the of which measures 1.2 em. in its greatest dimension. Rec-
FIG. 6
Fru. 7
FIG. G. X-ray with opaque cathetern in place. Left ureter crosses to right ectopic kidney. Right catheter leads to upper kidney. FIG. 7. Bilateral pyclogrmn, showing marked clogation of renal pelvic in crossed ectopic kidney. Section taken from kidney reveals presence of a.denocarcinoma.
tion shows the tissue to be made up of nodules of firm granular tissue with golden streaking of the framework. ":VIicroscopic: The tissue is made up of glandular structure which is invasive in areas. 2\Iost of the glands are lined by a single layer of cuboidal epithelium which resembles renal tubules. The lumina of the:-;e contain fresh blood. In some areas there is variation in the lining from cuboidal to pesudo-stratificd columnar epithelium with a tendency to papillary projections into the lumen. The density of the neoplasm varies, some: arras showing compact.masses of tubularstructures, while others lieinalooscand edematous connc:ctive fo;sue framework which is rich in capillaries and in which there is recent and old hemorrhagic extraYasation. Some parts of the neoplastic mass are entirely containing ·within them old remaining island:-; as cvi-
782
HOWARD T. LANGWORTHY AND LEO S. DREXLER
denced by blood pigment deposition. The tubular character of the neoplasm, the resemblance of the cells to those of renal tubules, and the occasional transformation of the simple cuboidal epithelium into pseudo-stratified columnar, all suggest the renal origin of this neoplasm. Diagnosis: Adenocarcinoma of the kidney." On the sixth postoperative day the patient eviscerated and required re-suture of the wound. Following this he made an uneventful recovery. Transfer to the Brooklyn Cancer Institute for further study and therapy was advised. However, the patient stated that he felt perfectly well, refused further treatment, signed a release and left the hospital. He was apparently symptom-free for a period of approximately 4 years until the time of his re-admission to the hospital
Fm. 8
Fm. 9
Fm. 8. Photomicrograph of representative portion of neoplasm, showing tendency to (A.) hemorrhage with necrosis, and (b) tubular character of structure. (X 75). Fm. 9. Higher magnification of figure 8, showing pseudo-stratified columnar cells (arrow) lining some of tubules constituting neoplasm. (X 180).
on April 9, 1941, when he had his first attack of hematuria. Cystoscopic examination April 10 revealed the presence of several small blood clots in the bladder. No calculus or neoplasm was noted. Both ureteral orifices were situated in their normal position on the inter-ureteric ridge. Both ureters were catheterized without difficulty. The urine from the right side was sanguineous. The urine from the left side was clear. Indigo carmine injected intravenously returned in 4 minutes on the left side in good concentration and in 10 minutes on the right side in poor concentration. Retrograde pyelography showed the right catheter to be in normal position. The left ureter was seen to cross the spine and was noted at the level of the fifth lumbar vertebra on the right side. Bilateral pyeloureterogram showed some downward displacement of the upper pelvis. The left pelvis was elongated and lay below and mesial to the right kidney.
CARClNOiVIA IN CROSSED RENAL ECTOPIA
An x-ray examination of the chest failed to reveal any evidenre of pulmonary metastases. X-ray of the spine and long bones ,ms negative for :-,kcletal meta,;tases. Laboratory Data. Blood ·wassermann, negative; hemoglobin, 90 per cent, white blood cells, ; polymorphonuclears, 68 per cent; lymphocyte;-;, 24 per cent; monocytes, Gper cent. Blood chemistry: Sugar, 228.4 mg.; urea nitrogen, 15.7 mgm;-;. Urinalysis: Color, red; specific gravity, 1.022; reaction, acid; albumin, 3 plur-;, sugar, negative; massive groups of red blood cells per high power field, 5-6 \Yhite blood cells per high power field. Frine recovered from the bladder at cystoscopy was bloody on gros;-; examination; micrm,copic examination disclosed massive groups of red blood cells. Frinc from the left kidney contained an occasional white blood cell per high pmver field; from the right kidney, massive groups of red blood cells per high pmver field. Specimen from the right kidne:v was gros1-1ly bloody. The diagnosi;-; wafi croRRed renal ectopy with carcinoma in the ectopic kidney. An exploratory operation was advised. However, the bleeding had the urine became clear, and the patient had no complaints. He rehrned any further t,reatment. SUMMARY
Renal ectopia is itself an unusual condition, although Thompson and Pa('e state that its incidence at the :'.\Iayo Clinic is about 1 in every 1,200 necropsies. In their series of 97 cases 30 per cent ·were found to have some pathological changes, but carcinoma was not noted in this group. Crossed ectopy is a far rarer clinical entity. The kidneys may be fused or unfused. One hundred and fifty-nine cases are reported in detail by Townsend and Frumkin. No evidence of neoplasm was noted in this series. Twenty cases of the fused type of croR;-;ed renal ectopy are by J\fayer and here again, no case of carcinoma was found. 337 Washington Ave., Brooklyn, N. Y. 125 Eastern Parkway, Brooklyn, N. Y. REFERENCES BEER, EDWARD AND FERBER, WILLIAM, L. S.: Crossed renal ectopia. .J. Urol., 38: 5+1, 19;37_ CAMPBELL, MEREDITH F.: Renal ectopy. J. Urol., 24: 187, 1930. CARLETON, ALICE: Crossed ectopia of kidney and its possible cause. J. Anat., 71: 292, 1D37. FRAZ1rn, E. B.: Congenital renal dystrophy. Urol. and Cut. Rev., 39: 381, 1935. LowsLEY AND Krn-ivrN: Clinical Urology. Baltimore: Williams & Wilkins Co., 1940, vol. 2, p. 1300. MAYER, MARTIN, l\T.: Crossed renal ectopia. J. Urol., 36: 111, 1936. STITES, JAMES R. AND BmVKK, .J. ANDREW: Crossed renal extopia of the kidney. J Urol., 42: 9, 1939. THOMPSON, GirnsHOM J. AND PACE, JonN: Ectopic kidney. Surg., Gynec. and Obst., 64: 935, 1937. TowNc,END, TERRY JvI. .AND FRl'.MKIN, JACOB: Unilateral fused kidney. Urol. and Cut. Rev., 41: 324, 1937. YmrNG, HuGH H. AND DAvrn, D. ;\I.· Practice of Urology. Philadelphia itnd LondonW. B. Saunders Co., 1926, vol. 2, p. 12.