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Wilms Tumor in a Horseshoe Kidney: A Surviving Case
THE JOUBK~\.L OF' lTROLOGY
\'ol. XS, No. I
Copyright CD
,July 1\162 \Yilliams & \Vilkins Cn. [i.S.A.
\VILJ\1S TUl\IOR I:\: A HORSESHOE KIDNEY: A SFRVI\T[\G CASE ERNEST LATHEM
AND
KITT H. SMITH
F tom the Depart'IJlenl of Urology, Bowman Gray School of Medicine of the Wake For est College.
Winst01hc;azem, N C. ancl Greenvi,'lle Geneml
\Yilms tumor in a horseshoe kidne:-· is rare. Twdve cases were found on a revie1Y of the literatun, to date (table 1). 1 • 12 The case presented here is the only one in \Yhich 1ve have been able to c:onfirm long term survin1l, :1lthough it will be noted tlrnt six :1re unaccounted for.
pulse 100, and repirations 18. Blood pressnre 1rns not recorded. Examination was within nornmi limits except for a firm, nontcndcr m:1ss wb iel1 was easily palpable in the right npper of the abdomen. The mass was sligbtly movnlllc and measured 10 cm. in greatest diameter. Crinalysis: l plus alburnin, 15-20 white blood cells, 13-14 red bloocl cells, s11ecifiG gravity 1 hemoglobin 13.7 gm. per cent, white blood cells 15,400. An excretory urogrnm suggested a spac:eO<'Cupying lesion of the right kiclne>y (fig. i). Sedimentation rate was not done . On January 15, with a of YVilms tumor, exploration was carried out. through a right flank incision. The presence of renal tumor \\'as confirmed. Hmrnver, the low,,r pole of the right kidney was found to be attad1ed to the left kidney by :1 parenchyrna.J isthmus. The tumor was confined to the right upper and middle portions of the kidney, and accordingly thr, isthmus was divided and :1 rigl1t tomy performed. The postoperative course was mwventfnl. Tt,e patient received immediate irradiation to the anterior (1200 r) and posterior ( I800 r; abdomen, Gross e:,;amination of the, specimen rcvcnL-:cl a 10 br 9 by 6 cm. oval, crstic tumor, On tlte lcrn '" edge was attached an elong3,kd portion of re;rnl tissue (fig. 2). lVIicroscopic cxnmin:ition rcvcaied numerous irregular - ......,,..... , .... ,, cells with rom1lled nuclei. The r,ytoplasrn of sorrn, cells was striakt! suggesting muscle, A few focal c:ondcnsation:, oi cells gnve an appearance and tubules, and there were some s1x1cc·8 (fig. 3) The microscopic pattern was considered compatible with an adenosarcoma tumor1 of thn kidney. Eight years following treatment, the is lO years old, weighs 71 pounds and is 5/'i in elms tall. Both the weight am! height un' in the 50Ul He is symptompercentile for 10-year-olcl
CASE R.J<:PORT
A 2½-:rc'ar-old white boy was admitted to the Grne1willc, General Hospital, ,Jarnrnry 13, 1954 with :1 liistory of abdominal :,;welling of 6 months' duration following a fall from a During the month before ad mission abdominal discomfort was noted. At times the chilcl 1rnuld cry because of pain when voiding. The rcm:1incler of the history was non-c,ontributory Examination revealed temperature of 99.6F, AccepLed for publication December 22, 1961 1
Hildebrant, 0.: Sarkom der
hufeisennier.
Deutsche Zeit, Chirurg., 40: 90-149, 1895. 2 Konigs and Pels-Leusclen: Die tuberculose der niere. Deutsche Zeit, Chirurg., 55: 1~19, 1900. 3 Gibbon, J. H .. Sarcoma of kidney. Ann. Surg., 49: 428--430, 1909 4 Eliasou, E. L. and W.· Wilrns' tumor in a hol'Seshoe kidnev, Surg., 119: 788-790, 1944. . 'Rose, D. K. and Wilms' tumor in isthmus of Urol. Cutan. Rev., 49: 365-3(i7, 1945. c McGiun, E J. and Wickham, J. M.: Wilrns' tumor in a horseshoe kidney. J. Urol., 56: 520-524,
A. N.: Maligrrnnt tumol'S of kidney, 29: 332-3:34, 19°10. J.. Gloirn1rnlonephritis, Wilms' tumor and horseshoe kidney in an iufant. AMA Arch. Path., 42: 5'19-554, Hl4(i. 9 M .. Ernbrvorna in horseshoe kidne,· in 15 rnontb girl; transperitoneal herninephreclorny. Rev. Argent. Urol., 24: 365-370, 1955. 10 LL1cke, B, and Schumberger, H. C.: N0phro-
blastoma in a horseshoe In: Atlas of Tumor Pathology, Sec. VII1 Fas. Armed Forces Institute Pathology, Washington 25, D. C. 1957, p. 106. ll
111
Greenville, S. C.
Beck, W. C. and Hlivko, Ji.. E. Wilms' tumor
the isthmus of a horseshoe kidney AMA Arch.
81: 803-806, 19Gl. " vV. C.: Personal communication. u Lawlor, J. B., Lattimer, J. K. and Wolff, J. A .. '\Vil ms' tumor in a hmseshoe kidney. Pedi· atric~ . 23: :35+-358, 1959
13 Silver, H.K., Kempe, C.H. arid Handbook of Pediatrics. Los Alto. Lange Pub., 1957, p. 55.
25
26
J. E. LATHEM AND K. H. SMITH
FIG. I. Preoperative excretory urogram revealing mass in area of right kidney. In retrospect horseshoe kidney should have been suspected.
free and the physical examination is normal. Radiological examination in 1960 (six and onehalf years postoperatively) showed the chest to be within normal limits and there were no masses noted in the abdomen. DISCUSSION
Horseshoe kidney is a form of congenital fused kidney, the connection (isthmus) being more commonly at the lower poles. 14 The frequency of the anomaly has been estimated as 1: 1000 in the general population.14 - 16 This condition in itself does not necessarily indicate poor prognosis for 14 Allen, A. C.: The Kidney: Medical and Surgical Diseases. New York: Grune and Stratton Co., 1951, p. 94-96. 15 Campbell, M. F.: Embryology and anomalies of the urogenital tract. In: Urology, ed. by M. F. Campbell. Philadelphia: W. B. Saunders Co., 1954, vol. I, pp. 266-272. 16 Dees, J.E.: Clinical importance of congenital anomalies of the upper urinary tract. J. Uro!., 46: 659-666, 1941.
Frn. 2. Cut surface of right half of horseshoe kidney. Tumor replaces almost all of kidney. Divided isthmus can be seen.
WILMS TUMOR IN A HORSESHOE KIDNEY
Fm. 3. Histologic section of tumor. Region shows attempted tubule formation in area surrounded by smooth muscle and connective tissue. X50.
the patient.17 However, associated positional anomalies of the drainage systems and blood supplies may interfere with emptying and therefore such kidneys are more susceptible to infections and stone formation. Horseshoe kidney requires no treatment unless symptoms or evidence of a secondary pathological process is present. Surgical intervention is required in 25 per cent of patients.17 Following the classic description of Wilms (1899), certain malignant embryonal tumors of the kidney of mesodermal origin have generally been referred to as Wilms tumors. Culp and Hartman18 state that more than 50 different pathological diagnoses have been ascribed to these lesions. Any combination of mesoclermal elements may be present. \Vilms tumor is the most common abdominal neoplasm in children, and comprises 20 per cent of all tumors in this 17 Glenn, J. F.: Analysis of 51 patients with horseshoe kidney. New Eng. J. Med., 261:
684-687, 1959. 18 Culp, 0. S. and Hartman, F. W.: Mesoblastic
nephroma in adults: a clinico-pathologic study of Wilms' tumors and related renal neoplasms . .J. Urol., 60: 552-57(i, Hl48.
27
age group. 19 It occurs once per one million population annually. 20 The therapy of Wilms tumor consists of surgery, irradiation, chemotherapy, and combinations of these methods. Surgery is regarded as the best single treatment, and most observers recommend immediate postoperative irracliation.21-23 Some suggest additional preoperative irradiation. 24 • 25 Survival rates have gradually improved in recent years and Gross 23 feels this is due to the addition of x-ray therapy postoperatively. Irradiation alone is usually recommended only for bilateral or inoperable lesions. Chemotherapy is still under evaluation with actinomycin D showing the most promise. Owens 26 reports that survival parallels age at the time of therapy. In his series, survival rates were 100 per cent in the under 18-months age group, whereas it was only 11 per cent in the same number of patients over 18 months of age. Gross 27 reported survival in Wilms tumor was twice as high in patients under 12 months of age compared with those over 12 months. Collins28 reasoned that a tumor can be only as old as a child plus 9 months for the period of gestation. Based on this he postulated that, assuming that tumor and metastases grow at a constant rate, any recurrences should be evident at the encl of a period equal to the chronological age plus 9 months from time of definitive therapy. The Collins rule has been almost 100 per cent 19 Porter, L. and Carter, W. E.: Observations on tumors of kidney region in children. Am. J. Dis. Child., 20: 323-330, 1920. 20 Glenn, J. F. and Rhame, R. C.: Wilms' tumor: epidemiological experience. J. Urol., 85: 911-918, 1961.
21 Hinman, F. and Kutzmann, A. A.: Malignant tumors of the kidney in children. Ann. Surg., 80:
569-590, 1924.
22 Cresson, L. and Pilling, G. P., IV: Renal tumors. Pediat. Clin. N. Amer., 6: 473-490, 1959. 23 Gross, R. E. and Neuhauser, E. B. D.: Treatment of mixed tumors of the kidney in childhood. Pediatrics, 6: 843-852, 1950. 24 Harvey, R. M.: Wilms' tumors: evaluation of treatment methods. Radiology, 54: 689-696, 1950. 26 Ng, E. and Low-Beer, B. V. A.: Treatment of Wilms' tumor. J. Pediat., 48: 763-769, 1956. 26 Owings, R. S. and Radakoirch, M.: Wilms' tumor: an evaluation of prognosis and treatment. Surgery, 46: 864-869, 1959. 27 Gross, R. E.: Surgery of Infancy and Childhood. Philadelphia: W. B. Saunders Co., 1953, p. 558.
28 Collins, V. P.: Wilms' tumor, its behavior and prognosis. J. Louisiana M. Soc., 107: 474-480, Ul55.
28
J. E. LA'l'IIEM AND K. H. SMITH
accurate in several series of known metastases of \Vilms tumors. 2s- 3 o In the present case, therefore, the prospect of future recurrence would appear to be remote. SUMMARY
Eight year survival following removal of a Wilms tumor in a horseshoe kidney has been presented. A review of the literature fails to 29 Kiesewetter, W. B. and Mason, E. J.: Malignant tumors in childhood. J.A.M.A., 172: 1117-
1121, 1960.
30 Sutow, W.W.: Prognosis in neuroblastoma in childhood. AMA J. Dis. Child., 96: 299-305, 1958.
reveal another such case with documented long term survival. Si;;: results arc unknown and 5 patients are known to have died. The treatment of \Vilms tumor in the horseshoe kidney does not differ in principle from that of \Vilms tumor occurring in the normally separated kidneys. Provided that the lesion is resectable by heminephrectomy, prognosis is not adversely affected by the presence of the underlying anomaly of fusion and the treatment does not differ.
Box 241, N. C. Baptist Hospital, Winston-Salem, N. C. (J. E. L.)
\'ol. XS, No. I
Copyright CD
,July 1\162 \Yilliams & \Vilkins Cn. [i.S.A.
\VILJ\1S TUl\IOR I:\: A HORSESHOE KIDNEY: A SFRVI\T[\G CASE ERNEST LATHEM
AND
KITT H. SMITH
F tom the Depart'IJlenl of Urology, Bowman Gray School of Medicine of the Wake For est College.
Winst01hc;azem, N C. ancl Greenvi,'lle Geneml
\Yilms tumor in a horseshoe kidne:-· is rare. Twdve cases were found on a revie1Y of the literatun, to date (table 1). 1 • 12 The case presented here is the only one in \Yhich 1ve have been able to c:onfirm long term survin1l, :1lthough it will be noted tlrnt six :1re unaccounted for.
pulse 100, and repirations 18. Blood pressnre 1rns not recorded. Examination was within nornmi limits except for a firm, nontcndcr m:1ss wb iel1 was easily palpable in the right npper of the abdomen. The mass was sligbtly movnlllc and measured 10 cm. in greatest diameter. Crinalysis: l plus alburnin, 15-20 white blood cells, 13-14 red bloocl cells, s11ecifiG gravity 1 hemoglobin 13.7 gm. per cent, white blood cells 15,400. An excretory urogrnm suggested a spac:eO<'Cupying lesion of the right kiclne>y (fig. i). Sedimentation rate was not done . On January 15, with a of YVilms tumor, exploration was carried out. through a right flank incision. The presence of renal tumor \\'as confirmed. Hmrnver, the low,,r pole of the right kidney was found to be attad1ed to the left kidney by :1 parenchyrna.J isthmus. The tumor was confined to the right upper and middle portions of the kidney, and accordingly thr, isthmus was divided and :1 rigl1t tomy performed. The postoperative course was mwventfnl. Tt,e patient received immediate irradiation to the anterior (1200 r) and posterior ( I800 r; abdomen, Gross e:,;amination of the, specimen rcvcnL-:cl a 10 br 9 by 6 cm. oval, crstic tumor, On tlte lcrn '" edge was attached an elong3,kd portion of re;rnl tissue (fig. 2). lVIicroscopic cxnmin:ition rcvcaied numerous irregular - ......,,..... , .... ,, cells with rom1lled nuclei. The r,ytoplasrn of sorrn, cells was striakt! suggesting muscle, A few focal c:ondcnsation:, oi cells gnve an appearance and tubules, and there were some s1x1cc·8 (fig. 3) The microscopic pattern was considered compatible with an adenosarcoma tumor1 of thn kidney. Eight years following treatment, the is lO years old, weighs 71 pounds and is 5/'i in elms tall. Both the weight am! height un' in the 50Ul He is symptompercentile for 10-year-olcl
CASE R.J<:PORT
A 2½-:rc'ar-old white boy was admitted to the Grne1willc, General Hospital, ,Jarnrnry 13, 1954 with :1 liistory of abdominal :,;welling of 6 months' duration following a fall from a During the month before ad mission abdominal discomfort was noted. At times the chilcl 1rnuld cry because of pain when voiding. The rcm:1incler of the history was non-c,ontributory Examination revealed temperature of 99.6F, AccepLed for publication December 22, 1961 1
Hildebrant, 0.: Sarkom der
hufeisennier.
Deutsche Zeit, Chirurg., 40: 90-149, 1895. 2 Konigs and Pels-Leusclen: Die tuberculose der niere. Deutsche Zeit, Chirurg., 55: 1~19, 1900. 3 Gibbon, J. H .. Sarcoma of kidney. Ann. Surg., 49: 428--430, 1909 4 Eliasou, E. L. and W.· Wilrns' tumor in a hol'Seshoe kidnev, Surg., 119: 788-790, 1944. . 'Rose, D. K. and Wilms' tumor in isthmus of Urol. Cutan. Rev., 49: 365-3(i7, 1945. c McGiun, E J. and Wickham, J. M.: Wilrns' tumor in a horseshoe kidney. J. Urol., 56: 520-524,
A. N.: Maligrrnnt tumol'S of kidney, 29: 332-3:34, 19°10. J.. Gloirn1rnlonephritis, Wilms' tumor and horseshoe kidney in an iufant. AMA Arch. Path., 42: 5'19-554, Hl4(i. 9 M .. Ernbrvorna in horseshoe kidne,· in 15 rnontb girl; transperitoneal herninephreclorny. Rev. Argent. Urol., 24: 365-370, 1955. 10 LL1cke, B, and Schumberger, H. C.: N0phro-
blastoma in a horseshoe In: Atlas of Tumor Pathology, Sec. VII1 Fas. Armed Forces Institute Pathology, Washington 25, D. C. 1957, p. 106. ll
111
Greenville, S. C.
Beck, W. C. and Hlivko, Ji.. E. Wilms' tumor
the isthmus of a horseshoe kidney AMA Arch.
81: 803-806, 19Gl. " vV. C.: Personal communication. u Lawlor, J. B., Lattimer, J. K. and Wolff, J. A .. '\Vil ms' tumor in a hmseshoe kidney. Pedi· atric~ . 23: :35+-358, 1959
13 Silver, H.K., Kempe, C.H. arid Handbook of Pediatrics. Los Alto. Lange Pub., 1957, p. 55.
25
26
J. E. LATHEM AND K. H. SMITH
FIG. I. Preoperative excretory urogram revealing mass in area of right kidney. In retrospect horseshoe kidney should have been suspected.
free and the physical examination is normal. Radiological examination in 1960 (six and onehalf years postoperatively) showed the chest to be within normal limits and there were no masses noted in the abdomen. DISCUSSION
Horseshoe kidney is a form of congenital fused kidney, the connection (isthmus) being more commonly at the lower poles. 14 The frequency of the anomaly has been estimated as 1: 1000 in the general population.14 - 16 This condition in itself does not necessarily indicate poor prognosis for 14 Allen, A. C.: The Kidney: Medical and Surgical Diseases. New York: Grune and Stratton Co., 1951, p. 94-96. 15 Campbell, M. F.: Embryology and anomalies of the urogenital tract. In: Urology, ed. by M. F. Campbell. Philadelphia: W. B. Saunders Co., 1954, vol. I, pp. 266-272. 16 Dees, J.E.: Clinical importance of congenital anomalies of the upper urinary tract. J. Uro!., 46: 659-666, 1941.
Frn. 2. Cut surface of right half of horseshoe kidney. Tumor replaces almost all of kidney. Divided isthmus can be seen.
WILMS TUMOR IN A HORSESHOE KIDNEY
Fm. 3. Histologic section of tumor. Region shows attempted tubule formation in area surrounded by smooth muscle and connective tissue. X50.
the patient.17 However, associated positional anomalies of the drainage systems and blood supplies may interfere with emptying and therefore such kidneys are more susceptible to infections and stone formation. Horseshoe kidney requires no treatment unless symptoms or evidence of a secondary pathological process is present. Surgical intervention is required in 25 per cent of patients.17 Following the classic description of Wilms (1899), certain malignant embryonal tumors of the kidney of mesodermal origin have generally been referred to as Wilms tumors. Culp and Hartman18 state that more than 50 different pathological diagnoses have been ascribed to these lesions. Any combination of mesoclermal elements may be present. \Vilms tumor is the most common abdominal neoplasm in children, and comprises 20 per cent of all tumors in this 17 Glenn, J. F.: Analysis of 51 patients with horseshoe kidney. New Eng. J. Med., 261:
684-687, 1959. 18 Culp, 0. S. and Hartman, F. W.: Mesoblastic
nephroma in adults: a clinico-pathologic study of Wilms' tumors and related renal neoplasms . .J. Urol., 60: 552-57(i, Hl48.
27
age group. 19 It occurs once per one million population annually. 20 The therapy of Wilms tumor consists of surgery, irradiation, chemotherapy, and combinations of these methods. Surgery is regarded as the best single treatment, and most observers recommend immediate postoperative irracliation.21-23 Some suggest additional preoperative irradiation. 24 • 25 Survival rates have gradually improved in recent years and Gross 23 feels this is due to the addition of x-ray therapy postoperatively. Irradiation alone is usually recommended only for bilateral or inoperable lesions. Chemotherapy is still under evaluation with actinomycin D showing the most promise. Owens 26 reports that survival parallels age at the time of therapy. In his series, survival rates were 100 per cent in the under 18-months age group, whereas it was only 11 per cent in the same number of patients over 18 months of age. Gross 27 reported survival in Wilms tumor was twice as high in patients under 12 months of age compared with those over 12 months. Collins28 reasoned that a tumor can be only as old as a child plus 9 months for the period of gestation. Based on this he postulated that, assuming that tumor and metastases grow at a constant rate, any recurrences should be evident at the encl of a period equal to the chronological age plus 9 months from time of definitive therapy. The Collins rule has been almost 100 per cent 19 Porter, L. and Carter, W. E.: Observations on tumors of kidney region in children. Am. J. Dis. Child., 20: 323-330, 1920. 20 Glenn, J. F. and Rhame, R. C.: Wilms' tumor: epidemiological experience. J. Urol., 85: 911-918, 1961.
21 Hinman, F. and Kutzmann, A. A.: Malignant tumors of the kidney in children. Ann. Surg., 80:
569-590, 1924.
22 Cresson, L. and Pilling, G. P., IV: Renal tumors. Pediat. Clin. N. Amer., 6: 473-490, 1959. 23 Gross, R. E. and Neuhauser, E. B. D.: Treatment of mixed tumors of the kidney in childhood. Pediatrics, 6: 843-852, 1950. 24 Harvey, R. M.: Wilms' tumors: evaluation of treatment methods. Radiology, 54: 689-696, 1950. 26 Ng, E. and Low-Beer, B. V. A.: Treatment of Wilms' tumor. J. Pediat., 48: 763-769, 1956. 26 Owings, R. S. and Radakoirch, M.: Wilms' tumor: an evaluation of prognosis and treatment. Surgery, 46: 864-869, 1959. 27 Gross, R. E.: Surgery of Infancy and Childhood. Philadelphia: W. B. Saunders Co., 1953, p. 558.
28 Collins, V. P.: Wilms' tumor, its behavior and prognosis. J. Louisiana M. Soc., 107: 474-480, Ul55.
28
J. E. LA'l'IIEM AND K. H. SMITH
accurate in several series of known metastases of \Vilms tumors. 2s- 3 o In the present case, therefore, the prospect of future recurrence would appear to be remote. SUMMARY
Eight year survival following removal of a Wilms tumor in a horseshoe kidney has been presented. A review of the literature fails to 29 Kiesewetter, W. B. and Mason, E. J.: Malignant tumors in childhood. J.A.M.A., 172: 1117-
1121, 1960.
30 Sutow, W.W.: Prognosis in neuroblastoma in childhood. AMA J. Dis. Child., 96: 299-305, 1958.
reveal another such case with documented long term survival. Si;;: results arc unknown and 5 patients are known to have died. The treatment of \Vilms tumor in the horseshoe kidney does not differ in principle from that of \Vilms tumor occurring in the normally separated kidneys. Provided that the lesion is resectable by heminephrectomy, prognosis is not adversely affected by the presence of the underlying anomaly of fusion and the treatment does not differ.
Box 241, N. C. Baptist Hospital, Winston-Salem, N. C. (J. E. L.)