- Email: [email protected]
Unusual Renal Tumors
THE JO"C"RNAL OF UROLOGT
Vol. GG, No. 4, October, JU51 Trinted in [~.S.A.
UNlJSl:"AL RENAL TUMORS THOMAS D. :'\WORE From the Department o.f Urology, John Gaston Hospital, College of ivfedicine, University of Tennessee, and the Jfoore Clinic, Baptist ~vfemorial Hospital, Memphis, Tenn.
A revieff of the literature dealing with renal tumors reveals that approximately 95 per cent are malignant and that the majority of these are carcinomas or adenocarcinomas of cortical origin of the type formerly usually designated hypernephroma. There is also evidence that renal tumors fall into t,rn rather distinct age groups: those of infancy and childhood, the majority of which are of mesoblastic origin; and those occurring after middle age, relatively few of which are of this type. The cause of tumors of the kidney in common with those in other locations has remained obscure, necessitating a classification based on the character of tissue from which they arise rather than on the impossible concept of etiology. In 1948 Culp and Hartman, in an excellent review of the literature concerning tumors of me1:,oblastic origin in the adult, \\·ere able to find 97 acceptable cases and added 4 of their mYn. They pointed out that of this group of embryonal renal tumors, there were 53 different pathologic diagnoses, most of which had been used in an attempt to describe actual or theoretical features of such tumors. They commented that the resulting conftrnion in nomenclature has no parallel in either urology or pathology. As a result of this study the1:,e authors advocated a logical and simplified classification, the general adoption of which would go a long way tmrnrd the abolishment of the existing confusion (fig. 1). If one considers especially those tumors of embryonal cortical origin, they may be grouped under the term mesoblastic nephroma y•,hich may be of epithelial, connective tissue, mixed cell types, or undifferentiated (fig. 2). In applying this classification to a group of 44 malignant renal tumors requiring surgical treatment taken from our files since 1929, there ·were 25 carcinomas or adenocarcinomas (clear or granular cell), G embryonal nephromas of which 3 were mixed tumors (Wilms' type), 2 were undifferentiated embryonal carcinomas and 1 fibrosarcoma, totalling 31 of cortical origin. The remaining 13 cases ,rnre epidermoid carcinomas of variable degrees of malignancy of intrapelvic origin. Ko instance was encountered of a tumor definitely arising from the renal capsule, although this ,ms a possibility ,Yith the one fibrosarcoma. Three of these tumors, a mixed cell mesoblastic nephroma in an adult usually referred to as a vVilms' tumor, a term discouraged by Culp and Hartman, a sarcomatous mesoblastic nephroma and an epidermoid carcinoma associated with renal tuberculosis, presented such 1musm,l features that they were considered worthy of more detailed discussion. As a rule, the diagnosis of tumor involving the kidney is not difficult. One or more of the triad of classical symptoms-pain, tumor, and hematuria-is elicited in taking the history and the subsequent urologic survey usually ,vill reveal Read at annual meeting, South Central Section, American urological Association, Tulsa, Okla. September 26, l 050
533
534
THOMAS D. MOORE
abnormal function and outline of the involved organ. Occasionally a Papanicolaou stain of the urinary sediment will be of assistance in establishing a positive diagnosis. There are certain advantages in being able to ascertain preoperatively whether a tumor is of cortical or intrapelvic origin and this has an important practical application in that those having their origin in the renal pelvisorcalyces require a complete nephro-ureterectomy. It would be highly desirable if one could determine in advance of surgery whether a tumor is benign or malignant and, if of the latter type, whether it is of renal cell adult type or mesoblastic MALIGNANT RENAL TUMORS
I l CORTEX
PELVIS
"'------"'\,, l
l
CARCIOMA from adult tubules ( clear cell) (adenocarcinoma)
EMBRYONAL from mesoblast
CAPSULE
?SARCOMA?
Frn. 1. Chief types of malignant renal tumors.
(After Culp and Hartman)
EMBRYONAL NEPHROMA
MESOBLASTIC NEPHROMA (cortex)
I l
/
,/
CARCINOMA
"'
\,,
MIXED CELL
"'
MESONEPHRIC NEPHROMA (pelvis) \,,
SARCOMA
,//
UNDIFFERENTIATED Frn. 2.
Classification of embryonal renal tumors.
(After Culp and Hartman)
embryonal origin, having a far more guarded prognosis. Unfortunately, an accurate preoperative diagnosis of the nature of tumors derived from the mesoblast in adults has not been possible. Such tumors may be suspected, however, from the comparatively brief history, rapid growth, the diminished incidence of gross hematuria and the character of tumor cells observed in stained smears of the urinary sediment. MIXED CELL MESOBLASTIC NEPHROMA IN AN ADULT
This tumor usually has been referred to as a Wilms' tumor occurring most commonly in infancy and childhood. Esersky, Saffer, Panoff and Jacobi in 1947
f
UJ'\USUAL RENAL TUMORS
535
made a careful review of the literature of this type of tumor in adults and ,vere able to find only 57 cases as of that date including three of their mrn. Since then Hill has reported l case and Silver, 2. The author's case is, therefore, the sixtyfirst in the literature. Histologically, this tumor is not to be confused with renal sarcoma, which it closely resembles in symptomatology, incidence and degree of malignancy or killing pmrnr. One should be able to find carcinoma-like cells in tubular, cylindrical, papillary or solid grouping and also sarcomatous elements which may take the form of oval, round, spindle, or giant cells. The rarity of the lesion is further illustrated by the report of Bell who did not encounter it in the study of approximately thirty thousand necropsy reports. In the group, however, were five \Vilms' tumors in children and one renal fibrosarcoma in an adult. The definite diagnosis of mixed cell mesoblastic nephroma in adults is decidedly difficult. The symptoms, signs, and findings on the urologic survey may simulate all other types of renal neoplasms. An unusually large tumor mass is common as is also elevation of temperature unaccompanied by other evidence of a urinary infection, as pointed out by Sparks. He also mentions ,rnakness of the patient out of proportion to the duration of the complaint. Apparently the true nature of the neoplasm has ne,·er been diagnosed accurately preoperatively but has been made exclusively by pathologic studies. Approximately 4 out of 5 of the cases reported have occurred in the fourth, fifth, and sixth decades. The treatment is always surgical if the lesion is operable. The prognosis, as in children, is extremely guarded although in a case reported by Loeb in 1949 the patient was living and well 7 years follmYing operation. Alcorn reported patients having lived as long as 7½ to 13 years following nephrectomy. CASE REPORT
Case Xo. 51517, Mrs. J. A. 2\1., aged 50, ,ms examined because of gross, painless hematuria which was first noticed seven months previously. This and one other episode of gross hematuria ,vere both of brief duration. Another which began 1 month before had persisted. There were no symptoms referable to the bladder but a moderate degree of discomfort had appeared in the right loin radiating downward and forward. Her general health was good although there had been recent slight loss of weight. On examination there was nothing noteworthy in the physical smTey. K either kidney was palpably enlarged and both ,Yere movable. The initial film of the region of the urinary tract was negative as was also the roentgen examination of the chest. The urine was grossly bloody and on cystoscopy the blood was seen to appear from the right ureteral orifice. Stained smears of the urinary sediment were reported negative for tumor cells. Retrograde pyelography disclosed normal outline on the left but on the right there was obliteration of the middle and upper calyces as well as their infundibula, associated with an irregular filling defect along the superior margin of the renal pelvis (fig. 3). The function of the right kidney was markedly impaired on testing with indigo carmine. A diagnosis was made of carcinoma of the right kidney which was thought to be of the intrapelvic type. Exploration was advised including a complete nephro-ureterectomy if the diagnosis were confirmed.
536
THOMAS D. MOORE
Forty-eight hours after the urologic investigation, the operation was performed with exposure of the kidney through an incision in the loin. The upper half of the organ was definitely enlarged and of dense consistency. It was possible to approach the renal pedicle with a minimum of manipulation and clamps were applied early. Mobilization was then completed including the upper ureter which was severed and the stumps immediately electrocoagulated. Removal of the organ was without incident. It was immediately sectioned, revealing an extensive neoplasm involving the upper half, whereupon the remainder of the ureter was mobilized as far as could be reached after ligating the electrocoagulated proximal end. With the patient in the recumbent posture an extraperitoneal approach was
FIG. 3. Bilateral retrograde pyelograms. Right, note irregular filling defect of pelvis and obliteration of upper calyces from tumor. Left, normal.
made through a split muscle incision to the lower third, permitting the removal of the ureter in its entirety, including a segment of the bladder. The patient made an uneventful convalescence. Both incisions healed by primary intention and she was dismissed on the eighth postoperative day. Three months later her examination, including cystoscopy and roentgen study of the chest, was negative. In answer to a recent inquiry it was reported that she considered herself in good health and doing her usual household duties fourteen months following the nephrectomy. Pathologic report. "This specimen is submitted as a right kidney and right ureter. It measures 11.5 cm. in length by 6 cm. in width and 5.5 cm. in thickness and weighs 210 gm. In one point of the kidney lies a bulbous enlargement which feels tense to the touch. On section the kidney is seen to contain some tissue
UNUSUAL RENAL TUMORS
537
which has a brawny consistency and has replaced the normal architecture in the upper two-thirds (fig. 4). The cortex in this region is thinned to 0.2 cm. at its thinnest point. The average thickness of the cortex is approximately 0.4 cm. This mass of tissue is yellowish-white in color with hemorrhage in several areas. The tumor mass in the second portion of the kidney seems to lie within the cavity lined with mucosa from the pelvis. This capsule is distended to the cortex and there is no remaining parenchyma. There is a great deal of difference in consistency in various portions of the tumor mass. That portion described as a bulbous enlargement at the upper pole is much more firm than any other tissue found in that part of the kidney. The second portion of this specimen which
FIG. 4. Mixed cell mesoblastic nephroma in an adult (Wilms' tumor)
was submitted as a ureter measures 16 cm. in length and 0.5 cm. in average diameter. On one end of the elongated structure there is a mass of tissue which appears to have been electrocoagulated. At the other end there is bulbous dilatation where the structure attains a diameter of approximately 0.9 cm. This area is soft in consistency. The lumen is patent throughout." Microscopic report. (See figure 5.) "The bulk of this lesion consists of small, spindle shaped cells with scant cytoplasm growing in intertwining bundles. Their nuclei have a fusiform shape, are hyperchromatic, and contain occasional mitotic figures. Scattered irregularly within this picture are distinct epithelial structures around which the other tumor component is concentrically arranged. This epithelial-like tissue consists of several to numerous poorly formed duct-like struc-
538
THOMAS D. MOORE
tures with lumens lined by plump, cuboidal epithelium. These duct-like structures merge with each other, and some fail to develop a lumen. Their cells have scant, pink cytoplasm and are largely occupied by a nucleus. These nuclei vary twofold in size and shape. Around the periphery of this tumor is a thick zone of fibrosis which separates it from the adjacent compressed and atrophic kidney parenchyma. Diagnosis: Mixed cell mesoblastic nephroma (Wilms')." Comment. Unlike the majority of tumors of this type, the mass was comparatively small. It will be noted that the neoplastic cells comprised a mixture both sarcomatous and carcinomatous. Occasionally one may find a distinct carcinoma
Frn. 5. Low power photomicrograph; note small spindle-shaped cells in intertwining bundles and scattered epithelial structures which did not stain well.
and sarcoma in the same organ which should be regarded as multiple tumors rather than a mixed one. The persistent gross hematuria of approximately one month's duration is unusual for this type of neoplasm. It is unfortunate that numerous stains of the urinary sediment failed to reveal characteristic tumor cells which might have permitted accurate preoperative diagnosis for the first time in this type of renal neoplasm. RENAL FIBROSARCOMA
Although the true nature of tumors derived from the mesoblast in adults has not been possible to diagnose before operation, such tumors should be suspected from the comparatively brief history, rapid growth, the diminished ini;idence of
539
UN-USUAL RE:"sAL TUMORS
gross hematuria and the character of tumor cells observed in stained smears of the urinary sediment. In 101 instances of mesoblastic nephroma collected from the literature by Culp and Hartman, including 4 of their mYn, 35 were fibrosarcomas. The rarity of such tumors is indicated by the report of eisel, Dockerty and Priestley who found that 35 had been treated surgically at the Mayo Clinic from 1904 to 1940 inclusive, an incidence of approximately only 1 in 30,000 patients examined at this institution. Microscopically, the lesions were basically composed of spindle cells resembling fibroblasts and were accordingly judged to be fibrosarcomas. All of them appeared histologically to be of a relatively high degree of malignancy; 90 per cent could be graded 3 or 4. These authors suggest the abandonment of such descriptive terms as small round-cell sarcoma, spindle cell sarcoma, and giant cell sarcoma as many of these round, spindle or giant cell forms are merely anaplastic variants of a single cellular type and do not represent individual or separate entities. The extremely poor prognosis \Yith this type of tumor is evidenced by the fact that of this group only 5 living patients could be traced, one of whom ,rns suffering from recurrence 18 months after nephrectomy and 4 had survived and were apparently well postoperatively for periods of 1, 2, 4, and 11 years. Renal sarcoma is not to be confused with mixed cell mesoblastic nephroma; the latter is a mixed neoplasm containing both connective tissue and epithelial elements of mesodermal origin. Kretschmer and Randolph state that true renal sarcoma is a disease not of children but of adults. Only at the surgical exploration may the true nature of the neoplasm be disclosed. It may comprise a yellowish solid mass which nearly ahrnys involves some portion of the renal capsule. Areas of hemorrhage are common. The microscopic pattern is Yariable and easily confused with other types, especially undifferentiated adenocarcinoma. In a case report by Henline, although an extension of the tumor ,Yas remoYed from the vena cava, the patient was alive and well 3½ years later. Such an experience serves to encourage nephrectomy whenever possible in spite of the extremely poor ultimate outlook.
,v
CASE REPORT
Case No. 46395, J. B. I., a man aged 61, was examined primarily because of symptoms of prostatism ,vhich had been slowly progressive over a period of 10 years. There was nocturia of 4 to 5 times and the symptoms had become especially troublesome during the previous month. He had experienced recently dull pain in the lmver lumbar region along ,vith some minor digestive disturbances. There had been no loss of weight, fever nor gross hematuria. The prostate gland was found to be moderately enlarged of benign type and there was 225 cc of infected residual urine. A transurethral prostatic resection ,ms performed, with the removal of 31 gm. of tissue, which was reported by the pathologist as benign prostatic hyperplasia. His immediate convalescence was satisfactory and upon removal of the inlying catheter on the third day he was able to empty the bladder completely and ,vith normal control. About this time he began to complain of an increasing degree of discomfort in the left loin associated with a mass which
540
THOMAS D. MOORE
had not been noted at his original examination and was thought to be possibly an enlarged spleen. Excretory urography, however, disclosed that the left kidney was without function and on passage of a ureteral catheter, 30 cc of thick pus was aspirated from the left renal pelvis. Retrograde pyelography failed to reveal a deformity characteristic of a renal tumor (fig. 6), but the dense mass in the region of the lower pole appeared to be connected with the kidney, which it accompanied on movement with respiration. Roentgen studies revealed no evidence of metastases. The preoperative diagnosis was left pyonephrosis associated with a left renal tumor. Three weeks following the transurethral prostatic resection, the kidney ,ms explored and presented the appearance of a typical pyonephrosis in combination with a hard nodular tumor involving the lower pole and rather loosely attached to the peritoneum over the anterior aspect. A nephrectomy was performed without incident after which the immediate con-
r
B --re•
L:" .•
Frn. 6. A, excretory urogram. Note absence of function, left. Right, normal. B, left retrograde pyelogram revealing pyonephrosis and mesial displacement of upper ureter by large mass at lower pole.
valescence was satisfactory and he was dismissed from the hospital on the nineteenth postoperative day. Although he was able to be up and about and appeared to be steadily regaining strength, his condition was progressively downhill upon his return home and his death was reported 14 days later or 32 days postoperatively. A postmortem examination was not made and the immediate cause of death was not determined. Pathologic report. "The left kidney measures 18 by 12 by 7 cm. and weighs 600 gm. There is a large deposit of fatty tissue which appears to be inflammatory. The lower pole presents a tumor mass measuring 12 cm. in diameter, and occupies more than two-thirds of the parenchyma. The cut surface is glistening, yellowish-white, and appears to be undergoing some myxoid changes. The edge of the growth which invades the parenchyma is lobulated and tends to be elevated above the cut surface. The remaining pelvis is dilated and distorted. The upper pole presents a hemorrhagic cyst 3 cm. in diameter and a number of soft yellowish-white areas which have the appearance of abscess cavities." Jl!I icroscopic report. (See figure 7.) "Sections through this lesion reveal a uniform picture of closely packed spindle-shaped cells almost completely replacing renal tissue. The limits between this tumor and the adjacent renal parenchyma are sharply defined although there is no surrounding capsule. The tumor itself is
UXUSUAL RENAL TUMORS
541
made up of slender, fusiform cells with long axes that are generally parallel to each other. The cells do not attempt to form bundles. Cell outlines are fairly distinct with their central portions containing a nucleus. These nuclei are ovoid and vary threefold in size and shape. There are occasional mitotic figures. The section taken near the renal pelvis reveals marked infiltration of neutrophils and lymphocytes. Diagnosis: Renal fibrosarcoma; pyonephrosis." Comment. This case is of unusual interest inasmuch as the patient ,rns admitted to the hospital for relief of benign prostatic obstruction and during his convalescence from a transurethral prostatic resection and ,Yhile under daily observation,
FIG. 7. Renal fibrosarcoma. A, low power photomicrograph. ;\Jote closely packed spindleshaped cells. B, high power photomicrograph. Xote slender fusiform cells with long axes and nuclei in their central portions.
the symptoms and signs of the left renal tumor rapidly appeared and were regarded as chiefly attributable to pyonephrosis. There was no gross hematuria other than that usually seen after a transurethral resection. The main evidence of renal tumor was the rapidly growing, palpable, indurated mass at the lower pole of the kidney unaccompanied by the usual pyelographic deformity of a renal neoplasm. ~ ot until the exploration was the presence of a sarcoma definitely ascertained, the true nature of which awaited the pathologist's report . It might have been suspected, however, if due attention had been paid to the typically brief history, rapid growth of the tumor, and absence of gross hematuria. The patient's early demise is characteristic of the highly malignant nature of the disease. ASSOCIATED MALIG;'\ANCY AND TUBERCULOSIS OF THE KIDNEY
The association of carcinoma or sarcoma with tuberculosis involving the same kidney is of such unusual occurrence thut it merits special consideration. :;,;reibling
542
THOMAS D. MOORE
and Walters in 1948 reviewed the literature and were able to collect 26 cases of combined renal tuberculosis and hypernephroma although in one of these reported by Keen in 1934 right renal tuberculosis was associated with a hypernephroma in the opposite kidney and perhaps should not have been included. These writers added 7 additional cases, 4 of which were considered primarily tuberculous and in 2 a renal neoplasm was diagnosed. After an analysis of 7500 autopsy reports, Pearl was of the opinion that an antagonism existed between active tuberculosis and carcinoma. Carlson and Bell, in a series of 11,195 necropsies, found active tuberculosis less common in cases of carcinoma. They did not think, however, that an antagonism actually existed. MacDonald in 1947 found two additional cases in the literature of renal sarcoma associated with tuberculosis (Pepe 1938; Suzuki 1941) and added a case of his own of hypernephroma coexisting with tuberculosis. Puigvert and Anguera in 1948 added 2 cases of papillary epithelioma and tuberculosis and also mentioned a case reported by Marion in 1912 and a case reported by Chevassu in 1934, the latter 2 cases apparently having not been mentioned in reviews of the literature by other authors. The accumulated cases referred to totaled 32 to which I would like to add a case in which not only was there an epidermoid carcinoma associated with tuberculosis but there was a stone in a tuberculous cavity of the kidney in addition to hydronephrosis and chronic pyelonephritis-a veritable pathologic museum,. CASE REPORT
Case No. 40542, Miss L. M., aged 58, was first seen May 10, 1934, because of a moderate degree of urinary frequency which had gradually progressed during the previous 2 or 3 years. Upon finding a considerable degree of pyuria, her physician had referred the patient for a urologic investigation. The initial roentgenogram disclosed an irregular shadow approximately 2 cm. in diameter in the region of the lower pole of the left kidney (fig. 8, A). Cystoscopic examination disclosed a moderate degree of chronic granular urethritis with extension of the inflammatory process onto the trigone. The bladder capacity was only 8 ounces. Phenolsulfonphthalein appeared after 3 minutes from each kidney with an excretion of 24 per cent from the right and 16 per cent from the left in 15 minutes. Urinalysis of the right renal specimen was negative, whereas the specimen from the left contained an average of 6 pus cells per high power field; a stained smear revealed no acid fast bacilli nor were other bacteria noted. Pyelography revealed a normal outline on the right but the shadow in the left renal area filled a cavitation at the lower pole which communicated with the lower calyx, suggesting the possibility of stone formation in a tuberculous cavitation (fig. 8, B). Exploration of the kidney was advised with the view of resection of the lower pole, which the patient refused. She was seen at irregular intervals because of the persistent pyuria and symptoms of a moderate degree of cystitis. She seemed content to tolerate the complaint rather than undergo surgical treatment. After a period of 14 years she began to complain of moderate discomfort in
UNUSUAL RENAL TUMORS
543
the left upper abdominal quadrant first thought to be of intestinal origin. Later the discomfort was noted more posteriorly and her physician requested another urologic study. Roentgen examination disclosed that the shadow previously mentioned was essentially unchanged in size or position. Cystoscopy revealed a fairly diffuse degree of chronic inflammation, more marked in the region of the trigone. The spurts from the right orifice were clear and were cloudy from the left. Indigo carmine returned in deep concentration after 4 minutes from the right and did not appear from the left after 10 minutes. After ureteral catheterization 22 cc of cloudy urine was aspirated from the left, containing grade 2
rr~
Fm. 8. A, initial film revealing irregular shadow in left renal area. B, excretory urogram. Note normal function and outline, right, moderately diminished function, left, with inclusion of shadow in cavitation at lower pole adjacent to lower calyx. C, left retrograde pyelogram obtained 14 years later. Note persistence of stone in cavitation at lower pole, moderate pyelectasis and striking change in outline of upper calyx from tumor.
concentration of indigo carmine and innumerable pus cells. The specimen from the right was negative. The right pyelogram was normal. The left revealed pyelectasis, grade 2, with evidence of an obstructive factor at the ureteropelvic juncture. When compared with the pyelogram made 14 years previously, changes involving the upper pole were in evidence in that it had become foreshortened and broader (fig. 8, C). Studies for acid fast bacilli were again negative. Exploration of the kidney was again advised and accepted. Under subarachnoid block the kidney was exposed through an incision in the left loin and was easily mobilized and the cortical stone at the lower pole was palpable through the thinned cortex overlying it. An indurated area about 3 0m. in diameter was noted near the upper pole on the anterior aspect, having a grayish appearance on the external surface. It appeared to be neoplastic rather
544
THOMAS D. MOORE
than inflammatory. The ureter was moderately thickened, was doubly clamped and divided about 5 cm. below its junction with the pelvis with electrocoagulation of the stumps. The kidney was removed without incident. Her convalescence was satisfactory and she was dismissed on the fourteenth postoperative day. One month following the operation she was readmitted to the hospital because of severe pain in the lower lumbar spine requiring opiates for relief. The pain gradually increased in severity and subsequent roentgen studies established the diagnosis of multiple spinal metastases. Roentgen examination of the chest at no time revealed evidence of tuberculosis or metastatic lesions from the neoplasm. The patient expired at home five months following the nephrectomy. A necropsy was not performed.
Fw. 9. A, low power photomicrograph; note area of epidermoid carcinoma and surrounding inflammatory zone of chronic pyelonephritis. B, high power; note poorly differentiated carcinoma cells. C, low power; note typical picture of tuberculosis, including giant cell and area of necrosis.
Pathologic report. "The specimen consists of a kidney which measures 13½ by 6½ by 4¼ cm. On its external surface, at the upper pole of the kidney, is an indurated area measuring 4½ by 3 by 2 cm. On section there is a firm granular, nodular tumor formation which has undergone some degree of degeneration. There is palpated in the pelvis several irregular stones. Longitudinal section through the kidney reveals the pelvis to be moderately dilated. The cortex and medulla are somewhat thinned. At the lower pole there are two stones embedded in the cortex of the kidney. The largest of these measures approximately 2 cm. in its greatest diameter. There is also noted within the pelvis of the kidney numerous small stones measuring approximately l½ to 2 mm. in size. At the site previously described on the external surface there is noticed a firm extension of tumor growth on the cut surface of the kidney." Microscopic report. (See figure 9.) "Multiple sections through this kidney reveal
UNUSUAL RENAL TUMORS
545
several distinct processes. One is an epidermoid carcinoma extending from the renal peivis rather deeply into the kidney substance. This tumor is growing as broad anastomosing sheets and cords of moderately undifferentiated keratinized epithelium. The tumor cells are relatively large with indistinct cytoplasmic outlines. The abundant cystoplasm is homogeneous and pink, consistent with keratin. Their nuclei vary fourfold in size and shape, are moderately chromatic, and shmv frequent mitotic figures, many of which are bizarre. Another distinct lesion is tuberculosis manifested by scattered, discrete, granulomatous lesions. lWost of the larger ones show principally a central caseous, amorphous mass surrounded by marked number of lymphocytes, some epitheloid cells, and occasionally considerable fibrosis. Langhans' cells are occasionally seen. In the smaller lesions there are numerous epitheloid cells arranged radially in a thick zone surrounding central necrosis. Else,vhere in the renal parenchyma is considerable diffuse, lymphocytic infiltration of the interstitial tissue, and this reaction appears removed and distinct from the tuberculous change. Glomeruli are frequently completely hyalinized, and there is much atrophy and loss of tubules. Changes in the vascular ,mils are not remarkable. Diagnosis: Epidermoid carcinoma, grade 3; renal tuberculosis; nephrolithiasis; hyclronephrosis; chronic pyelonephritis." Comment. Undoubtedly the kidney became involved -with tuberculosis in comparati,-ely early life as ,Yhcn the patient ,ms first seen 14 years previously, the cavitation at the lmrnr pole was snugly filled ,,·ith stone_ Apparently the disease remained limited to this area over the long period of years and at no time was she troubled with a severe degree of secondary tuberculous cystitis so commonly experienced in the presence of renal tuberculosis. The highly malignant epidermoid carcinoma was probably of recent origin arising in an area near the upper pole quite distant from the tuberculous process. If antagonism actually exists bet,rnen malignancy and tuberculosis as has been suggested, the latter disease failed to protect the organ from carcinoma and thus tends to refute the idea. Although the neoplasm ,ms comparatively small, the histologic picture gave definite evidence of an almost total absence of differentiation and its highly malignant nature. The period of survival, however, was much shorter than was anticipated at the time of surgery. SUMMARY
The reports of three unusual renal neoplasms are submitted in abstract. The first of these, a mixed cell mesoblastic nephroma (V\'ilms' tumor) in an adult is the sixty-first reported in the literature. The second case, a renal fibrosarcoma, apparently is the one hundred third case reported. The third case of a malignancy associated with tuberculosis involving the same kidney is the thirty-third to be recorded in the literature. I am indebted to Dr. l'vI. L. Trumbull, chief of the Department of Pathology, Baptist Memorial Hospital, for his comprehensive pathologic descriptions and valuable assistanceo 899 111adison
3, Tenn.
546
THOMAS D. MOORE
REFERENCES ALcOR"1, K. A.: Wilms' tumor in an adult. Proc. Staff ]\feet. Mayo Clinic, 12: 692-694, 1937. BELL, E. T.: A classification of renal tumors with observations on the frequency of the various types. J. Urol., 39: 238-2-J.3, 1938. CARLSON, H. A. AND BELL, E.T.: A statistical study of the occurrence of cancer and tuberculosis in 11,195 postmortem examinations. J. Cancer Res., 13: 126, 1929. CHEVAssu: (Quoted by Puigvert and Anguera). CuLP, 0. S. AND HARTMAN, F. W.: ::\fesoblastic nephroma in adults: A clinicopathologic study of Wilms' tumors and related renal neoplasms. J. Ural., 60: 552-576, 1948. EsERSKY, G. L., SAFFER, S. H., PANOFF, C. E. AND JACOBI, M.: Wilms' tumor in the adult: Review of literature and report of three additional cases. J. Urol., 58: 397-411, 1947. HENLINE, R. B.: Fibrosarcoma of a polycystic kidney with extension into vena cava: Report of case with successful surgical removal. J. Urol., 39: 238-2-!3, 1938. HILL, R. :YI.: Embryoma of the kidney in the adult. Brit. J. Ural., 18: 53-50, 1946. KEEN, M. R.: Associated renal tuberculosis and malignancy. Am. J. Surg., 25: 467-475, 193"1. KRETscmrnR, H. L. A"1D RANDOLPH, H. S.: Spindle-celled sarcoma of the kidney in adults. Ann. Surg., 88: 1033-1038, 1928. LOEB, M. J.: Report of postoperative survival of two cases of malignancy: (1) Carcinoma of the adrenal cortex; (2) Wilms' tumor in an adult. J. Urol., 61: 20-22, 1949. l\!IAcDoNALD, S. A.: Co-existing hypernephroma and tuberculosis. Can. Med. Assn. J., 57: 572-575, 1947. MARION: (Quoted by Puigvert and Anguera.) NEIBLING, H. A. A"1D WALTERS, W.: Adenocarcinoma and tuberculosis of the same kidney: Review of the literature and report of seven cases. J. Urol., 59: 1022-1035, 1948. PEARL, R.: Cancer and tuberculosis. Amer. J. Hyg., 9: 97, 1929. PEPE: (Quoted by S. A. MAcDoNALD.) PurnvERT, A. AND A"1G1:"ERA, A. 0.: Cancer and tuberculosis in the kidney. Ural. & Cutan. Rev., 52: 441-448, 1948. SILVER, H.K.: Wilms' tumor (embryoma of the kidney). J. Pediat., 31: 643-650, 1947. SPARKS, A. J.: Wilms' tumor in sixty-three year old male: Case report. J. Urol., 47: 642647, 1942. SuzuKI: (Quoted by S. A. l\fAcDoNALD.) WEISEL, W., DocKERTY, ]\f. B. AND PRIESTLEY, J. T.: Sarcoma of the kidney. J. Urol., 50: 564-573, 1943.
Vol. GG, No. 4, October, JU51 Trinted in [~.S.A.
UNlJSl:"AL RENAL TUMORS THOMAS D. :'\WORE From the Department o.f Urology, John Gaston Hospital, College of ivfedicine, University of Tennessee, and the Jfoore Clinic, Baptist ~vfemorial Hospital, Memphis, Tenn.
A revieff of the literature dealing with renal tumors reveals that approximately 95 per cent are malignant and that the majority of these are carcinomas or adenocarcinomas of cortical origin of the type formerly usually designated hypernephroma. There is also evidence that renal tumors fall into t,rn rather distinct age groups: those of infancy and childhood, the majority of which are of mesoblastic origin; and those occurring after middle age, relatively few of which are of this type. The cause of tumors of the kidney in common with those in other locations has remained obscure, necessitating a classification based on the character of tissue from which they arise rather than on the impossible concept of etiology. In 1948 Culp and Hartman, in an excellent review of the literature concerning tumors of me1:,oblastic origin in the adult, \\·ere able to find 97 acceptable cases and added 4 of their mYn. They pointed out that of this group of embryonal renal tumors, there were 53 different pathologic diagnoses, most of which had been used in an attempt to describe actual or theoretical features of such tumors. They commented that the resulting conftrnion in nomenclature has no parallel in either urology or pathology. As a result of this study the1:,e authors advocated a logical and simplified classification, the general adoption of which would go a long way tmrnrd the abolishment of the existing confusion (fig. 1). If one considers especially those tumors of embryonal cortical origin, they may be grouped under the term mesoblastic nephroma y•,hich may be of epithelial, connective tissue, mixed cell types, or undifferentiated (fig. 2). In applying this classification to a group of 44 malignant renal tumors requiring surgical treatment taken from our files since 1929, there ·were 25 carcinomas or adenocarcinomas (clear or granular cell), G embryonal nephromas of which 3 were mixed tumors (Wilms' type), 2 were undifferentiated embryonal carcinomas and 1 fibrosarcoma, totalling 31 of cortical origin. The remaining 13 cases ,rnre epidermoid carcinomas of variable degrees of malignancy of intrapelvic origin. Ko instance was encountered of a tumor definitely arising from the renal capsule, although this ,ms a possibility ,Yith the one fibrosarcoma. Three of these tumors, a mixed cell mesoblastic nephroma in an adult usually referred to as a vVilms' tumor, a term discouraged by Culp and Hartman, a sarcomatous mesoblastic nephroma and an epidermoid carcinoma associated with renal tuberculosis, presented such 1musm,l features that they were considered worthy of more detailed discussion. As a rule, the diagnosis of tumor involving the kidney is not difficult. One or more of the triad of classical symptoms-pain, tumor, and hematuria-is elicited in taking the history and the subsequent urologic survey usually ,vill reveal Read at annual meeting, South Central Section, American urological Association, Tulsa, Okla. September 26, l 050
533
534
THOMAS D. MOORE
abnormal function and outline of the involved organ. Occasionally a Papanicolaou stain of the urinary sediment will be of assistance in establishing a positive diagnosis. There are certain advantages in being able to ascertain preoperatively whether a tumor is of cortical or intrapelvic origin and this has an important practical application in that those having their origin in the renal pelvisorcalyces require a complete nephro-ureterectomy. It would be highly desirable if one could determine in advance of surgery whether a tumor is benign or malignant and, if of the latter type, whether it is of renal cell adult type or mesoblastic MALIGNANT RENAL TUMORS
I l CORTEX
PELVIS
"'------"'\,, l
l
CARCIOMA from adult tubules ( clear cell) (adenocarcinoma)
EMBRYONAL from mesoblast
CAPSULE
?SARCOMA?
Frn. 1. Chief types of malignant renal tumors.
(After Culp and Hartman)
EMBRYONAL NEPHROMA
MESOBLASTIC NEPHROMA (cortex)
I l
/
,/
CARCINOMA
"'
\,,
MIXED CELL
"'
MESONEPHRIC NEPHROMA (pelvis) \,,
SARCOMA
,//
UNDIFFERENTIATED Frn. 2.
Classification of embryonal renal tumors.
(After Culp and Hartman)
embryonal origin, having a far more guarded prognosis. Unfortunately, an accurate preoperative diagnosis of the nature of tumors derived from the mesoblast in adults has not been possible. Such tumors may be suspected, however, from the comparatively brief history, rapid growth, the diminished incidence of gross hematuria and the character of tumor cells observed in stained smears of the urinary sediment. MIXED CELL MESOBLASTIC NEPHROMA IN AN ADULT
This tumor usually has been referred to as a Wilms' tumor occurring most commonly in infancy and childhood. Esersky, Saffer, Panoff and Jacobi in 1947
f
UJ'\USUAL RENAL TUMORS
535
made a careful review of the literature of this type of tumor in adults and ,vere able to find only 57 cases as of that date including three of their mrn. Since then Hill has reported l case and Silver, 2. The author's case is, therefore, the sixtyfirst in the literature. Histologically, this tumor is not to be confused with renal sarcoma, which it closely resembles in symptomatology, incidence and degree of malignancy or killing pmrnr. One should be able to find carcinoma-like cells in tubular, cylindrical, papillary or solid grouping and also sarcomatous elements which may take the form of oval, round, spindle, or giant cells. The rarity of the lesion is further illustrated by the report of Bell who did not encounter it in the study of approximately thirty thousand necropsy reports. In the group, however, were five \Vilms' tumors in children and one renal fibrosarcoma in an adult. The definite diagnosis of mixed cell mesoblastic nephroma in adults is decidedly difficult. The symptoms, signs, and findings on the urologic survey may simulate all other types of renal neoplasms. An unusually large tumor mass is common as is also elevation of temperature unaccompanied by other evidence of a urinary infection, as pointed out by Sparks. He also mentions ,rnakness of the patient out of proportion to the duration of the complaint. Apparently the true nature of the neoplasm has ne,·er been diagnosed accurately preoperatively but has been made exclusively by pathologic studies. Approximately 4 out of 5 of the cases reported have occurred in the fourth, fifth, and sixth decades. The treatment is always surgical if the lesion is operable. The prognosis, as in children, is extremely guarded although in a case reported by Loeb in 1949 the patient was living and well 7 years follmYing operation. Alcorn reported patients having lived as long as 7½ to 13 years following nephrectomy. CASE REPORT
Case Xo. 51517, Mrs. J. A. 2\1., aged 50, ,ms examined because of gross, painless hematuria which was first noticed seven months previously. This and one other episode of gross hematuria ,vere both of brief duration. Another which began 1 month before had persisted. There were no symptoms referable to the bladder but a moderate degree of discomfort had appeared in the right loin radiating downward and forward. Her general health was good although there had been recent slight loss of weight. On examination there was nothing noteworthy in the physical smTey. K either kidney was palpably enlarged and both ,Yere movable. The initial film of the region of the urinary tract was negative as was also the roentgen examination of the chest. The urine was grossly bloody and on cystoscopy the blood was seen to appear from the right ureteral orifice. Stained smears of the urinary sediment were reported negative for tumor cells. Retrograde pyelography disclosed normal outline on the left but on the right there was obliteration of the middle and upper calyces as well as their infundibula, associated with an irregular filling defect along the superior margin of the renal pelvis (fig. 3). The function of the right kidney was markedly impaired on testing with indigo carmine. A diagnosis was made of carcinoma of the right kidney which was thought to be of the intrapelvic type. Exploration was advised including a complete nephro-ureterectomy if the diagnosis were confirmed.
536
THOMAS D. MOORE
Forty-eight hours after the urologic investigation, the operation was performed with exposure of the kidney through an incision in the loin. The upper half of the organ was definitely enlarged and of dense consistency. It was possible to approach the renal pedicle with a minimum of manipulation and clamps were applied early. Mobilization was then completed including the upper ureter which was severed and the stumps immediately electrocoagulated. Removal of the organ was without incident. It was immediately sectioned, revealing an extensive neoplasm involving the upper half, whereupon the remainder of the ureter was mobilized as far as could be reached after ligating the electrocoagulated proximal end. With the patient in the recumbent posture an extraperitoneal approach was
FIG. 3. Bilateral retrograde pyelograms. Right, note irregular filling defect of pelvis and obliteration of upper calyces from tumor. Left, normal.
made through a split muscle incision to the lower third, permitting the removal of the ureter in its entirety, including a segment of the bladder. The patient made an uneventful convalescence. Both incisions healed by primary intention and she was dismissed on the eighth postoperative day. Three months later her examination, including cystoscopy and roentgen study of the chest, was negative. In answer to a recent inquiry it was reported that she considered herself in good health and doing her usual household duties fourteen months following the nephrectomy. Pathologic report. "This specimen is submitted as a right kidney and right ureter. It measures 11.5 cm. in length by 6 cm. in width and 5.5 cm. in thickness and weighs 210 gm. In one point of the kidney lies a bulbous enlargement which feels tense to the touch. On section the kidney is seen to contain some tissue
UNUSUAL RENAL TUMORS
537
which has a brawny consistency and has replaced the normal architecture in the upper two-thirds (fig. 4). The cortex in this region is thinned to 0.2 cm. at its thinnest point. The average thickness of the cortex is approximately 0.4 cm. This mass of tissue is yellowish-white in color with hemorrhage in several areas. The tumor mass in the second portion of the kidney seems to lie within the cavity lined with mucosa from the pelvis. This capsule is distended to the cortex and there is no remaining parenchyma. There is a great deal of difference in consistency in various portions of the tumor mass. That portion described as a bulbous enlargement at the upper pole is much more firm than any other tissue found in that part of the kidney. The second portion of this specimen which
FIG. 4. Mixed cell mesoblastic nephroma in an adult (Wilms' tumor)
was submitted as a ureter measures 16 cm. in length and 0.5 cm. in average diameter. On one end of the elongated structure there is a mass of tissue which appears to have been electrocoagulated. At the other end there is bulbous dilatation where the structure attains a diameter of approximately 0.9 cm. This area is soft in consistency. The lumen is patent throughout." Microscopic report. (See figure 5.) "The bulk of this lesion consists of small, spindle shaped cells with scant cytoplasm growing in intertwining bundles. Their nuclei have a fusiform shape, are hyperchromatic, and contain occasional mitotic figures. Scattered irregularly within this picture are distinct epithelial structures around which the other tumor component is concentrically arranged. This epithelial-like tissue consists of several to numerous poorly formed duct-like struc-
538
THOMAS D. MOORE
tures with lumens lined by plump, cuboidal epithelium. These duct-like structures merge with each other, and some fail to develop a lumen. Their cells have scant, pink cytoplasm and are largely occupied by a nucleus. These nuclei vary twofold in size and shape. Around the periphery of this tumor is a thick zone of fibrosis which separates it from the adjacent compressed and atrophic kidney parenchyma. Diagnosis: Mixed cell mesoblastic nephroma (Wilms')." Comment. Unlike the majority of tumors of this type, the mass was comparatively small. It will be noted that the neoplastic cells comprised a mixture both sarcomatous and carcinomatous. Occasionally one may find a distinct carcinoma
Frn. 5. Low power photomicrograph; note small spindle-shaped cells in intertwining bundles and scattered epithelial structures which did not stain well.
and sarcoma in the same organ which should be regarded as multiple tumors rather than a mixed one. The persistent gross hematuria of approximately one month's duration is unusual for this type of neoplasm. It is unfortunate that numerous stains of the urinary sediment failed to reveal characteristic tumor cells which might have permitted accurate preoperative diagnosis for the first time in this type of renal neoplasm. RENAL FIBROSARCOMA
Although the true nature of tumors derived from the mesoblast in adults has not been possible to diagnose before operation, such tumors should be suspected from the comparatively brief history, rapid growth, the diminished ini;idence of
539
UN-USUAL RE:"sAL TUMORS
gross hematuria and the character of tumor cells observed in stained smears of the urinary sediment. In 101 instances of mesoblastic nephroma collected from the literature by Culp and Hartman, including 4 of their mYn, 35 were fibrosarcomas. The rarity of such tumors is indicated by the report of eisel, Dockerty and Priestley who found that 35 had been treated surgically at the Mayo Clinic from 1904 to 1940 inclusive, an incidence of approximately only 1 in 30,000 patients examined at this institution. Microscopically, the lesions were basically composed of spindle cells resembling fibroblasts and were accordingly judged to be fibrosarcomas. All of them appeared histologically to be of a relatively high degree of malignancy; 90 per cent could be graded 3 or 4. These authors suggest the abandonment of such descriptive terms as small round-cell sarcoma, spindle cell sarcoma, and giant cell sarcoma as many of these round, spindle or giant cell forms are merely anaplastic variants of a single cellular type and do not represent individual or separate entities. The extremely poor prognosis \Yith this type of tumor is evidenced by the fact that of this group only 5 living patients could be traced, one of whom ,rns suffering from recurrence 18 months after nephrectomy and 4 had survived and were apparently well postoperatively for periods of 1, 2, 4, and 11 years. Renal sarcoma is not to be confused with mixed cell mesoblastic nephroma; the latter is a mixed neoplasm containing both connective tissue and epithelial elements of mesodermal origin. Kretschmer and Randolph state that true renal sarcoma is a disease not of children but of adults. Only at the surgical exploration may the true nature of the neoplasm be disclosed. It may comprise a yellowish solid mass which nearly ahrnys involves some portion of the renal capsule. Areas of hemorrhage are common. The microscopic pattern is Yariable and easily confused with other types, especially undifferentiated adenocarcinoma. In a case report by Henline, although an extension of the tumor ,Yas remoYed from the vena cava, the patient was alive and well 3½ years later. Such an experience serves to encourage nephrectomy whenever possible in spite of the extremely poor ultimate outlook.
,v
CASE REPORT
Case No. 46395, J. B. I., a man aged 61, was examined primarily because of symptoms of prostatism ,vhich had been slowly progressive over a period of 10 years. There was nocturia of 4 to 5 times and the symptoms had become especially troublesome during the previous month. He had experienced recently dull pain in the lmver lumbar region along ,vith some minor digestive disturbances. There had been no loss of weight, fever nor gross hematuria. The prostate gland was found to be moderately enlarged of benign type and there was 225 cc of infected residual urine. A transurethral prostatic resection ,ms performed, with the removal of 31 gm. of tissue, which was reported by the pathologist as benign prostatic hyperplasia. His immediate convalescence was satisfactory and upon removal of the inlying catheter on the third day he was able to empty the bladder completely and ,vith normal control. About this time he began to complain of an increasing degree of discomfort in the left loin associated with a mass which
540
THOMAS D. MOORE
had not been noted at his original examination and was thought to be possibly an enlarged spleen. Excretory urography, however, disclosed that the left kidney was without function and on passage of a ureteral catheter, 30 cc of thick pus was aspirated from the left renal pelvis. Retrograde pyelography failed to reveal a deformity characteristic of a renal tumor (fig. 6), but the dense mass in the region of the lower pole appeared to be connected with the kidney, which it accompanied on movement with respiration. Roentgen studies revealed no evidence of metastases. The preoperative diagnosis was left pyonephrosis associated with a left renal tumor. Three weeks following the transurethral prostatic resection, the kidney ,ms explored and presented the appearance of a typical pyonephrosis in combination with a hard nodular tumor involving the lower pole and rather loosely attached to the peritoneum over the anterior aspect. A nephrectomy was performed without incident after which the immediate con-
r
B --re•
L:" .•
Frn. 6. A, excretory urogram. Note absence of function, left. Right, normal. B, left retrograde pyelogram revealing pyonephrosis and mesial displacement of upper ureter by large mass at lower pole.
valescence was satisfactory and he was dismissed from the hospital on the nineteenth postoperative day. Although he was able to be up and about and appeared to be steadily regaining strength, his condition was progressively downhill upon his return home and his death was reported 14 days later or 32 days postoperatively. A postmortem examination was not made and the immediate cause of death was not determined. Pathologic report. "The left kidney measures 18 by 12 by 7 cm. and weighs 600 gm. There is a large deposit of fatty tissue which appears to be inflammatory. The lower pole presents a tumor mass measuring 12 cm. in diameter, and occupies more than two-thirds of the parenchyma. The cut surface is glistening, yellowish-white, and appears to be undergoing some myxoid changes. The edge of the growth which invades the parenchyma is lobulated and tends to be elevated above the cut surface. The remaining pelvis is dilated and distorted. The upper pole presents a hemorrhagic cyst 3 cm. in diameter and a number of soft yellowish-white areas which have the appearance of abscess cavities." Jl!I icroscopic report. (See figure 7.) "Sections through this lesion reveal a uniform picture of closely packed spindle-shaped cells almost completely replacing renal tissue. The limits between this tumor and the adjacent renal parenchyma are sharply defined although there is no surrounding capsule. The tumor itself is
UXUSUAL RENAL TUMORS
541
made up of slender, fusiform cells with long axes that are generally parallel to each other. The cells do not attempt to form bundles. Cell outlines are fairly distinct with their central portions containing a nucleus. These nuclei are ovoid and vary threefold in size and shape. There are occasional mitotic figures. The section taken near the renal pelvis reveals marked infiltration of neutrophils and lymphocytes. Diagnosis: Renal fibrosarcoma; pyonephrosis." Comment. This case is of unusual interest inasmuch as the patient ,rns admitted to the hospital for relief of benign prostatic obstruction and during his convalescence from a transurethral prostatic resection and ,Yhile under daily observation,
FIG. 7. Renal fibrosarcoma. A, low power photomicrograph. ;\Jote closely packed spindleshaped cells. B, high power photomicrograph. Xote slender fusiform cells with long axes and nuclei in their central portions.
the symptoms and signs of the left renal tumor rapidly appeared and were regarded as chiefly attributable to pyonephrosis. There was no gross hematuria other than that usually seen after a transurethral resection. The main evidence of renal tumor was the rapidly growing, palpable, indurated mass at the lower pole of the kidney unaccompanied by the usual pyelographic deformity of a renal neoplasm. ~ ot until the exploration was the presence of a sarcoma definitely ascertained, the true nature of which awaited the pathologist's report . It might have been suspected, however, if due attention had been paid to the typically brief history, rapid growth of the tumor, and absence of gross hematuria. The patient's early demise is characteristic of the highly malignant nature of the disease. ASSOCIATED MALIG;'\ANCY AND TUBERCULOSIS OF THE KIDNEY
The association of carcinoma or sarcoma with tuberculosis involving the same kidney is of such unusual occurrence thut it merits special consideration. :;,;reibling
542
THOMAS D. MOORE
and Walters in 1948 reviewed the literature and were able to collect 26 cases of combined renal tuberculosis and hypernephroma although in one of these reported by Keen in 1934 right renal tuberculosis was associated with a hypernephroma in the opposite kidney and perhaps should not have been included. These writers added 7 additional cases, 4 of which were considered primarily tuberculous and in 2 a renal neoplasm was diagnosed. After an analysis of 7500 autopsy reports, Pearl was of the opinion that an antagonism existed between active tuberculosis and carcinoma. Carlson and Bell, in a series of 11,195 necropsies, found active tuberculosis less common in cases of carcinoma. They did not think, however, that an antagonism actually existed. MacDonald in 1947 found two additional cases in the literature of renal sarcoma associated with tuberculosis (Pepe 1938; Suzuki 1941) and added a case of his own of hypernephroma coexisting with tuberculosis. Puigvert and Anguera in 1948 added 2 cases of papillary epithelioma and tuberculosis and also mentioned a case reported by Marion in 1912 and a case reported by Chevassu in 1934, the latter 2 cases apparently having not been mentioned in reviews of the literature by other authors. The accumulated cases referred to totaled 32 to which I would like to add a case in which not only was there an epidermoid carcinoma associated with tuberculosis but there was a stone in a tuberculous cavity of the kidney in addition to hydronephrosis and chronic pyelonephritis-a veritable pathologic museum,. CASE REPORT
Case No. 40542, Miss L. M., aged 58, was first seen May 10, 1934, because of a moderate degree of urinary frequency which had gradually progressed during the previous 2 or 3 years. Upon finding a considerable degree of pyuria, her physician had referred the patient for a urologic investigation. The initial roentgenogram disclosed an irregular shadow approximately 2 cm. in diameter in the region of the lower pole of the left kidney (fig. 8, A). Cystoscopic examination disclosed a moderate degree of chronic granular urethritis with extension of the inflammatory process onto the trigone. The bladder capacity was only 8 ounces. Phenolsulfonphthalein appeared after 3 minutes from each kidney with an excretion of 24 per cent from the right and 16 per cent from the left in 15 minutes. Urinalysis of the right renal specimen was negative, whereas the specimen from the left contained an average of 6 pus cells per high power field; a stained smear revealed no acid fast bacilli nor were other bacteria noted. Pyelography revealed a normal outline on the right but the shadow in the left renal area filled a cavitation at the lower pole which communicated with the lower calyx, suggesting the possibility of stone formation in a tuberculous cavitation (fig. 8, B). Exploration of the kidney was advised with the view of resection of the lower pole, which the patient refused. She was seen at irregular intervals because of the persistent pyuria and symptoms of a moderate degree of cystitis. She seemed content to tolerate the complaint rather than undergo surgical treatment. After a period of 14 years she began to complain of moderate discomfort in
UNUSUAL RENAL TUMORS
543
the left upper abdominal quadrant first thought to be of intestinal origin. Later the discomfort was noted more posteriorly and her physician requested another urologic study. Roentgen examination disclosed that the shadow previously mentioned was essentially unchanged in size or position. Cystoscopy revealed a fairly diffuse degree of chronic inflammation, more marked in the region of the trigone. The spurts from the right orifice were clear and were cloudy from the left. Indigo carmine returned in deep concentration after 4 minutes from the right and did not appear from the left after 10 minutes. After ureteral catheterization 22 cc of cloudy urine was aspirated from the left, containing grade 2
rr~
Fm. 8. A, initial film revealing irregular shadow in left renal area. B, excretory urogram. Note normal function and outline, right, moderately diminished function, left, with inclusion of shadow in cavitation at lower pole adjacent to lower calyx. C, left retrograde pyelogram obtained 14 years later. Note persistence of stone in cavitation at lower pole, moderate pyelectasis and striking change in outline of upper calyx from tumor.
concentration of indigo carmine and innumerable pus cells. The specimen from the right was negative. The right pyelogram was normal. The left revealed pyelectasis, grade 2, with evidence of an obstructive factor at the ureteropelvic juncture. When compared with the pyelogram made 14 years previously, changes involving the upper pole were in evidence in that it had become foreshortened and broader (fig. 8, C). Studies for acid fast bacilli were again negative. Exploration of the kidney was again advised and accepted. Under subarachnoid block the kidney was exposed through an incision in the left loin and was easily mobilized and the cortical stone at the lower pole was palpable through the thinned cortex overlying it. An indurated area about 3 0m. in diameter was noted near the upper pole on the anterior aspect, having a grayish appearance on the external surface. It appeared to be neoplastic rather
544
THOMAS D. MOORE
than inflammatory. The ureter was moderately thickened, was doubly clamped and divided about 5 cm. below its junction with the pelvis with electrocoagulation of the stumps. The kidney was removed without incident. Her convalescence was satisfactory and she was dismissed on the fourteenth postoperative day. One month following the operation she was readmitted to the hospital because of severe pain in the lower lumbar spine requiring opiates for relief. The pain gradually increased in severity and subsequent roentgen studies established the diagnosis of multiple spinal metastases. Roentgen examination of the chest at no time revealed evidence of tuberculosis or metastatic lesions from the neoplasm. The patient expired at home five months following the nephrectomy. A necropsy was not performed.
Fw. 9. A, low power photomicrograph; note area of epidermoid carcinoma and surrounding inflammatory zone of chronic pyelonephritis. B, high power; note poorly differentiated carcinoma cells. C, low power; note typical picture of tuberculosis, including giant cell and area of necrosis.
Pathologic report. "The specimen consists of a kidney which measures 13½ by 6½ by 4¼ cm. On its external surface, at the upper pole of the kidney, is an indurated area measuring 4½ by 3 by 2 cm. On section there is a firm granular, nodular tumor formation which has undergone some degree of degeneration. There is palpated in the pelvis several irregular stones. Longitudinal section through the kidney reveals the pelvis to be moderately dilated. The cortex and medulla are somewhat thinned. At the lower pole there are two stones embedded in the cortex of the kidney. The largest of these measures approximately 2 cm. in its greatest diameter. There is also noted within the pelvis of the kidney numerous small stones measuring approximately l½ to 2 mm. in size. At the site previously described on the external surface there is noticed a firm extension of tumor growth on the cut surface of the kidney." Microscopic report. (See figure 9.) "Multiple sections through this kidney reveal
UNUSUAL RENAL TUMORS
545
several distinct processes. One is an epidermoid carcinoma extending from the renal peivis rather deeply into the kidney substance. This tumor is growing as broad anastomosing sheets and cords of moderately undifferentiated keratinized epithelium. The tumor cells are relatively large with indistinct cytoplasmic outlines. The abundant cystoplasm is homogeneous and pink, consistent with keratin. Their nuclei vary fourfold in size and shape, are moderately chromatic, and shmv frequent mitotic figures, many of which are bizarre. Another distinct lesion is tuberculosis manifested by scattered, discrete, granulomatous lesions. lWost of the larger ones show principally a central caseous, amorphous mass surrounded by marked number of lymphocytes, some epitheloid cells, and occasionally considerable fibrosis. Langhans' cells are occasionally seen. In the smaller lesions there are numerous epitheloid cells arranged radially in a thick zone surrounding central necrosis. Else,vhere in the renal parenchyma is considerable diffuse, lymphocytic infiltration of the interstitial tissue, and this reaction appears removed and distinct from the tuberculous change. Glomeruli are frequently completely hyalinized, and there is much atrophy and loss of tubules. Changes in the vascular ,mils are not remarkable. Diagnosis: Epidermoid carcinoma, grade 3; renal tuberculosis; nephrolithiasis; hyclronephrosis; chronic pyelonephritis." Comment. Undoubtedly the kidney became involved -with tuberculosis in comparati,-ely early life as ,Yhcn the patient ,ms first seen 14 years previously, the cavitation at the lmrnr pole was snugly filled ,,·ith stone_ Apparently the disease remained limited to this area over the long period of years and at no time was she troubled with a severe degree of secondary tuberculous cystitis so commonly experienced in the presence of renal tuberculosis. The highly malignant epidermoid carcinoma was probably of recent origin arising in an area near the upper pole quite distant from the tuberculous process. If antagonism actually exists bet,rnen malignancy and tuberculosis as has been suggested, the latter disease failed to protect the organ from carcinoma and thus tends to refute the idea. Although the neoplasm ,ms comparatively small, the histologic picture gave definite evidence of an almost total absence of differentiation and its highly malignant nature. The period of survival, however, was much shorter than was anticipated at the time of surgery. SUMMARY
The reports of three unusual renal neoplasms are submitted in abstract. The first of these, a mixed cell mesoblastic nephroma (V\'ilms' tumor) in an adult is the sixty-first reported in the literature. The second case, a renal fibrosarcoma, apparently is the one hundred third case reported. The third case of a malignancy associated with tuberculosis involving the same kidney is the thirty-third to be recorded in the literature. I am indebted to Dr. l'vI. L. Trumbull, chief of the Department of Pathology, Baptist Memorial Hospital, for his comprehensive pathologic descriptions and valuable assistanceo 899 111adison
3, Tenn.
546
THOMAS D. MOORE
REFERENCES ALcOR"1, K. A.: Wilms' tumor in an adult. Proc. Staff ]\feet. Mayo Clinic, 12: 692-694, 1937. BELL, E. T.: A classification of renal tumors with observations on the frequency of the various types. J. Urol., 39: 238-2-J.3, 1938. CARLSON, H. A. AND BELL, E.T.: A statistical study of the occurrence of cancer and tuberculosis in 11,195 postmortem examinations. J. Cancer Res., 13: 126, 1929. CHEVAssu: (Quoted by Puigvert and Anguera). CuLP, 0. S. AND HARTMAN, F. W.: ::\fesoblastic nephroma in adults: A clinicopathologic study of Wilms' tumors and related renal neoplasms. J. Ural., 60: 552-576, 1948. EsERSKY, G. L., SAFFER, S. H., PANOFF, C. E. AND JACOBI, M.: Wilms' tumor in the adult: Review of literature and report of three additional cases. J. Urol., 58: 397-411, 1947. HENLINE, R. B.: Fibrosarcoma of a polycystic kidney with extension into vena cava: Report of case with successful surgical removal. J. Urol., 39: 238-2-!3, 1938. HILL, R. :YI.: Embryoma of the kidney in the adult. Brit. J. Ural., 18: 53-50, 1946. KEEN, M. R.: Associated renal tuberculosis and malignancy. Am. J. Surg., 25: 467-475, 193"1. KRETscmrnR, H. L. A"1D RANDOLPH, H. S.: Spindle-celled sarcoma of the kidney in adults. Ann. Surg., 88: 1033-1038, 1928. LOEB, M. J.: Report of postoperative survival of two cases of malignancy: (1) Carcinoma of the adrenal cortex; (2) Wilms' tumor in an adult. J. Urol., 61: 20-22, 1949. l\!IAcDoNALD, S. A.: Co-existing hypernephroma and tuberculosis. Can. Med. Assn. J., 57: 572-575, 1947. MARION: (Quoted by Puigvert and Anguera.) NEIBLING, H. A. A"1D WALTERS, W.: Adenocarcinoma and tuberculosis of the same kidney: Review of the literature and report of seven cases. J. Urol., 59: 1022-1035, 1948. PEARL, R.: Cancer and tuberculosis. Amer. J. Hyg., 9: 97, 1929. PEPE: (Quoted by S. A. MAcDoNALD.) PurnvERT, A. AND A"1G1:"ERA, A. 0.: Cancer and tuberculosis in the kidney. Ural. & Cutan. Rev., 52: 441-448, 1948. SILVER, H.K.: Wilms' tumor (embryoma of the kidney). J. Pediat., 31: 643-650, 1947. SPARKS, A. J.: Wilms' tumor in sixty-three year old male: Case report. J. Urol., 47: 642647, 1942. SuzuKI: (Quoted by S. A. l\fAcDoNALD.) WEISEL, W., DocKERTY, ]\f. B. AND PRIESTLEY, J. T.: Sarcoma of the kidney. J. Urol., 50: 564-573, 1943.