Wilms’ Tumor in Crossed Fused Renal Ectopia . By John F. Redman and D. 1. Berry
l The
occurrence
in a crossed ported patient
only with
ectopic one
of a
previous
these
nephroblastoma
kidney findings
has
time.’
been
A second
is the
of this report.
re-
subject
CASE
INDEX renal
WORDS:
Wilms’
tumor;
crossed
ectopia.
REPORT
J.J. (#40-57-27), a 3 yr old white male, was first seen in July, 1974, following the discovery on physical examination of a large right-sided abdominal mass. Excretory urography demonstrated crossed renal ectopia (Fig. I). Aortography confirmed the presence of a tumor involving the right side of the abdomen and extending to the left side. The patient was explored through a transverse abdominal incision and was found to have a very large retroperitoneal tumor which
From the Departments of Urology and Pediatrics. Universit.v of’ Arkansas College of Medicine, Little Rock, Arkansas. Address reprint requests to: John F. Redman. M.D., Associate Proyessor and Chairman, Dept. Urology, University of Arkansas College of Medicine, 4301 West Markham St., Little Rock, Ark. 72201. 0 1977 bv Grune & Stratton. Inc. .hmal
of Pediatric Surgery, Vol. 12, No. 4 (August), 1977
601
CASE
602
Fig. bladder.
2.
Large The renal
mass
as
bodies
exposed are hidden
through
transverse
abdominal
incision.
Arrow
REPORTS
identifies
gall
by the mass.
arose from the upper pole of the superior renal body (Fig. 2). The mass encompassed the great vessels in the midline and involved the mesentery of the descending colon. Following biopsy and marking of the renal mass with silver clips, the procedure was terminated. The pathologic diagnosis was nephroblastoma. The patient was treated with Vincristine and Actinomycin-D until September, 1975. He received a total of 3000 rad external irradiation to the tumor mass. Twenty-three months postoperatively he has no palpable mass or other evidence of disease. His family history is of interest in that a paternal aunt had a documented crossed renal ectopia.
DISCUSSION Renal cell carcinoma in crossed renal ectopia has been described on two occasions.2.3 The occurrence of nephroblastoma and renal cell carcinoma in a horseshoe kidney has been reported.4*s The occurrence of Wilms’ tumor with other congenital anomalies, particularly aniridia and hemihypertrophy, has been noted.6 Congenital anomalies have been reported more commonly with bilaterally occurring nephroblastoma.7 The significance of the findings of congenital anomalies in association with nephroblastoma is controversial.8.9 Shashikumar et al.4 suggested a “predisposition of horseshoe kidneys to develop Wilms’ tumor” and recommended frequent periodic abdominal palpation for all children with confirmed horseshoe kidneys. Reported series of crossed renal ectopia and horseshoe kidneys do not strongly indicate an increased incidence of tumor occurrence. IOII In our patient the occurrence of a large nephric tumor in a fused renal body made the treatment similar to either a large localized but unresectable nephroblastoma in a single kidney or a bilateral nephroblastoma. The treatment of these lesions has been the subject of several publications which show the value of aggressive combined therapy.‘2-‘4 Radiation therapy which involves the remaining renal tissue is fraught with hazards but currently is superior to the alternative of radical surgery and renal transplantation.‘3
CASE
603
REPORTS
REFERENCES I. Berant M, Jacob ET, Pevzner S: Wilms’ tumor in the crossed ectopic kidney. J Pediatr Surg 10:555-556. 1975 2. Langworthy HT, Drexler LS: Carcinoma in crossed renal ectopia. J Ural 47:776. 1942 3. Lee HP: Crossed unfused with tumor. J Ural 61:333, 1949
renal
ectopia
4. Shashikumar VL, Somers LA, Pilling GP IV. et al: Wilms’ tumor in the horseshoe kidney. J Pediatr Surg 9: 185, 1974 5. Redman JF, Mobley JE: Renal cell carcinoma in a horseshoe kidney. South Med J 66:963, 1973 6. Miller RW, Fraumeni JF Jr, Manning MD: Association of Wilms’ tumor with aniridia. hemihypertrophy, and other congenital malformations. N Engl J Med 270:922, 1964 7. Bond JV: Bilateral Wilms’ tumour: age at diagnosis, associated congenital anomalies. and possible pattern of inheritance. Lancet 2:482. 1975
8. Miller RW: Relation between cancer and congenital defects in man. N Engl J Med 275:87, 1966 9. Berry, CL, Keeling J. Hilton, C: Coincidence of congenital malformation and embryonic tumours of childhood. Arch Dis Child 45:229, 1970 10. Boatman DL, Culp DA Jr, Culp DA et al: Crossed renal ectopia. J Ural 108:30, 1972 I I. Segura JW, Kelalis PP, Burke EC: Horseshoe kidney in children. J Ural 108:333. 1972 12. Garrett RA, Battersky JS: Bilateral Wilms tumor: 4 year survival. J Urol 106:942. 1971 13. Ragab AH, Wietti TJ, Crist W et al: Bilateral Wilms’ tumor: a review. Cancer 30~983, 1972 14. Kumar APM, Wrenn EL Jr, Fleming ID et al: Preoperative therapy for unresectable malignant tumors in children. J Pediatr Surg 10:657-670. 1975
l The
occurrence
in a crossed ported patient
only with
ectopic one
of a
previous
these
nephroblastoma
kidney findings
has
time.’
been
A second
is the
of this report.
re-
subject
CASE
INDEX renal
WORDS:
Wilms’
tumor;
crossed
ectopia.
REPORT
J.J. (#40-57-27), a 3 yr old white male, was first seen in July, 1974, following the discovery on physical examination of a large right-sided abdominal mass. Excretory urography demonstrated crossed renal ectopia (Fig. I). Aortography confirmed the presence of a tumor involving the right side of the abdomen and extending to the left side. The patient was explored through a transverse abdominal incision and was found to have a very large retroperitoneal tumor which
From the Departments of Urology and Pediatrics. Universit.v of’ Arkansas College of Medicine, Little Rock, Arkansas. Address reprint requests to: John F. Redman. M.D., Associate Proyessor and Chairman, Dept. Urology, University of Arkansas College of Medicine, 4301 West Markham St., Little Rock, Ark. 72201. 0 1977 bv Grune & Stratton. Inc. .hmal
of Pediatric Surgery, Vol. 12, No. 4 (August), 1977
601
CASE
602
Fig. bladder.
2.
Large The renal
mass
as
bodies
exposed are hidden
through
transverse
abdominal
incision.
Arrow
REPORTS
identifies
gall
by the mass.
arose from the upper pole of the superior renal body (Fig. 2). The mass encompassed the great vessels in the midline and involved the mesentery of the descending colon. Following biopsy and marking of the renal mass with silver clips, the procedure was terminated. The pathologic diagnosis was nephroblastoma. The patient was treated with Vincristine and Actinomycin-D until September, 1975. He received a total of 3000 rad external irradiation to the tumor mass. Twenty-three months postoperatively he has no palpable mass or other evidence of disease. His family history is of interest in that a paternal aunt had a documented crossed renal ectopia.
DISCUSSION Renal cell carcinoma in crossed renal ectopia has been described on two occasions.2.3 The occurrence of nephroblastoma and renal cell carcinoma in a horseshoe kidney has been reported.4*s The occurrence of Wilms’ tumor with other congenital anomalies, particularly aniridia and hemihypertrophy, has been noted.6 Congenital anomalies have been reported more commonly with bilaterally occurring nephroblastoma.7 The significance of the findings of congenital anomalies in association with nephroblastoma is controversial.8.9 Shashikumar et al.4 suggested a “predisposition of horseshoe kidneys to develop Wilms’ tumor” and recommended frequent periodic abdominal palpation for all children with confirmed horseshoe kidneys. Reported series of crossed renal ectopia and horseshoe kidneys do not strongly indicate an increased incidence of tumor occurrence. IOII In our patient the occurrence of a large nephric tumor in a fused renal body made the treatment similar to either a large localized but unresectable nephroblastoma in a single kidney or a bilateral nephroblastoma. The treatment of these lesions has been the subject of several publications which show the value of aggressive combined therapy.‘2-‘4 Radiation therapy which involves the remaining renal tissue is fraught with hazards but currently is superior to the alternative of radical surgery and renal transplantation.‘3
CASE
603
REPORTS
REFERENCES I. Berant M, Jacob ET, Pevzner S: Wilms’ tumor in the crossed ectopic kidney. J Pediatr Surg 10:555-556. 1975 2. Langworthy HT, Drexler LS: Carcinoma in crossed renal ectopia. J Ural 47:776. 1942 3. Lee HP: Crossed unfused with tumor. J Ural 61:333, 1949
renal
ectopia
4. Shashikumar VL, Somers LA, Pilling GP IV. et al: Wilms’ tumor in the horseshoe kidney. J Pediatr Surg 9: 185, 1974 5. Redman JF, Mobley JE: Renal cell carcinoma in a horseshoe kidney. South Med J 66:963, 1973 6. Miller RW, Fraumeni JF Jr, Manning MD: Association of Wilms’ tumor with aniridia. hemihypertrophy, and other congenital malformations. N Engl J Med 270:922, 1964 7. Bond JV: Bilateral Wilms’ tumour: age at diagnosis, associated congenital anomalies. and possible pattern of inheritance. Lancet 2:482. 1975
8. Miller RW: Relation between cancer and congenital defects in man. N Engl J Med 275:87, 1966 9. Berry, CL, Keeling J. Hilton, C: Coincidence of congenital malformation and embryonic tumours of childhood. Arch Dis Child 45:229, 1970 10. Boatman DL, Culp DA Jr, Culp DA et al: Crossed renal ectopia. J Ural 108:30, 1972 I I. Segura JW, Kelalis PP, Burke EC: Horseshoe kidney in children. J Ural 108:333. 1972 12. Garrett RA, Battersky JS: Bilateral Wilms tumor: 4 year survival. J Urol 106:942. 1971 13. Ragab AH, Wietti TJ, Crist W et al: Bilateral Wilms’ tumor: a review. Cancer 30~983, 1972 14. Kumar APM, Wrenn EL Jr, Fleming ID et al: Preoperative therapy for unresectable malignant tumors in children. J Pediatr Surg 10:657-670. 1975
