Adult Wilms' Tumor: Report of a Case and Review o f the Literature Arvind KUMAR,*Brajesh LAL,* Manoj SINGH** and Brij KAPUR* ABSTRACT: A 48 year old male presented with a o n e and a half year history o f a progressively increasing, asymptomadc lump in the left hypochondrium and no history o f hematuria. His general physical examination was unremarkable, and an abdominal examination revealed a large, firm, irregular and tender mass in the left hypochondrium extending into the lumbar region. Chest X-ray was normal. An intravenous urogram revealed a normally functioning right kidney with non-visualization o f the left kidney. CT-scan o f the abdomen revealed a large, mixed attenuating mass replacing the left kidney. At laparotomy, a large, fleshy, wellencapsulated tumor was found in the left k i d n e y with no surrounding infiltration and a left radical nephrectomy was performed. Microscopic examination revealed a poorly differentiated tumor comprised o f small r o u n d cells with focal areas o f abortive embryonal tubular and glomerular differentiation suggestive o f Wilms' tumor. The patient was advised chemotherapy and radiotherapy but he absconded and was lost to follow-up. KEY WORDS:
Wilms' tumor, nephroblastoma
INTRODUCTION
W
i
l
m
s
'
tumor is the most c o m m o n renal tumor in children and accounts for 20 per cent o f all childhood neoplasms? T h e introduction o f multimodal therapy, however, combining surgery, radiation therapy and chemotherapy has improved the survival o f these children to up to 90 per c e n C In adults, the true incidence o f this tumor is difficult to ascertain, but it is certainly very rare. T h e variation in the n u m b e r o f cases reported reflects the confusion in histologic features and nomenclature existing in the past? -7 In comparison to children, however,
The Departments of Surgery* and Pathology, ** All India Institute of Medical Sciences, New Delhi, India Reprint requests to: Brij Kapur, MD, The Department of Surgery, All India Institute of Medical Sciences, New Delhi, India
Wilms' tumor in adults is diagnosed at a more advanced clinical stage and the prognosis is much worse. 2 We report herein, a well documented case o f Wilms' tumor in an adult. We also present an exhaustive updated review o f the world medical literature and give guidelines for establishing the diagnosis and managing this rare disease. CASE REPORT A 48 year old male was admitted to our hospital in July, 1988 with a one and a half year history o f a progressively increasing, asymptomatic lump in the left hypochondrium without hematuria. Examination revealed a well patient with mild pallor, no lymphadenopathy, a pulse rate o f 8 2 / m and a blood pressure o f 115/90 mmHg. Abdominal examination revealed a
JAPANESEJOURNALOFSURGERY,VOL.20, No. 5 pp. 585-589, 1990
586
Kumar et at.
Fig: 1. Photomicrograph of the tumor showing a stroma of poorly differentiated cells. (HE • large, firm, irregular and tender mass in the left hypochondrium extending into the lumbar region. T h e mass was bimanually palpable and the left renal angle was dull on percussion. His chest X-ray was normal. An intravenous u,rogram revealed a normally placed and functioning right kidney, but the left kidney could not be visualized. CT scan of the abdomen revealed a large, mixed attenuating mass replacing the left kidney which itself was not seen separately. T h e r e was no extension into the renal vein. A preliminary clinical diagnosis of left renal cell carcinoma was made and the patient scheduled for surgery. Laparotomy subsequently revealed the left kidney to be replaced by a large, fleshy, well encapsulated tumor without any infiltration into the surrounding structures. T h e tumor was not extending into the renal vein. A left radical nephrectomy was done and the patient had an uneventful postoperative recovery. After the histological diagnosis had b e e n established, the patient was advised to undergo chemotherapy and local radiotherapy but he absconded and was lost to follow up. O n pathological examination, the kidney measured 30 X 20 X 8 cm and weighed 4700 g. T h e external surface was irregular and the capsule infiltrated at the upper pole. O n sectioning, the tumor involved the entire
Jpn. J. Surg. September 1990
Fig. 2. Photomicrograph of another area of the same tumor showing abortive tubular differentiation. (HE X240) kidney with areas o f hemorrhage, necrosis and cystic degeneration. T h e pelvicalyceal system was infiltrated with the tumor, though blood vessels at the hilum were free. O n microscopy, the tumor was poorly differentiated, comprising mostly of small r o u n d cells with hyperchromatic nuclei (Fig. 1). Focal areas o f abortive embryonal tubular as well as glomerular differentiation were seen (Fig. 2). These features were compatible with Wilms' tumor. DISCUSSION T h e first case o f Wilms' tumor in an adult was reported by Kocker and Langhans in 18788 and a review o f literature up to 1980 by Babaian et al. yielded a total of only 167 cases. 9 However, in the world literature, spanning i10 years from 1878 to 1988, we have recorded a total o f 211 casesY - ~ Culp and Hartman noted fifty three synonyms for Wilms' tumor, 3 yet considering the confusion in terminology and the propensity of renal cell carcinomas which mimic its histologic features, some of these may not represent a true Wilms' tumor. 9,a4 Controversy regarding the terminology of this tumor abated after the introduction of the term "nephroblastoma". This term reflects the accepted theory of the tumor's origin from metanephrogenic blastoma, the precursor of both the nephrons and intersti-
Volume 20 Number 5
Adult Wilms' tumor
tium o f the kidney? 5,~6 Presently, only two interchangeable terms remain-nephroblastoma, and Wilms' tumor; we prefer to use the latter. The diagnostic pathological features of Wilms' t u m o r in both adults and children are identical, Qthe most important feature being the p r e s e n c e o f abortive e m b r y o n a l g! omeruli in an immature spindle cell stroma? 6 Renal cell c a r c i n o m a s h a v i n g g l a n d u l a r snaactures along with sarcomatous or undifferentiated cells are difficult to distinguish from it, but Wilms' tumors do not contain the mature glandular structures seen in renal cell carcinoma? ~ Because the t u m o r is rare in adults, i t is difficult to d i a g n o s e p r e o p e r a t i v e l y . I n Eserskey's series o f 56 cases, n o n e were diagnosed correctly before surgerya as there is no test to differentiate it f r o m renal cell carcinoma. About two thirds o f these patients present with pain, while an abdominal mass a n d / o r hematuria is seen in three quarters.14 A plain abdominal X-ray may reveal fine calcification in 10-30 p e r cent? 7 Intravenous urograms show a space occupying lesion or n o n visualization o f the affected kidney TM and angiography may reveal varying degrees o f vascularity including a "creeping vine" or "spaghetti" pattern in some. 19CT scan usually shows a n o n h o m o g e n o u s intrarenal mass and is also useful in evaluating the presence of multiple masses, the extent o f the tumor, major vessel i n v o l v e m e n t a n d s e c o n d a r y spread. 2~ T h o u g h well established in children, the p r o g n o s t i c f a c t o r s r e m a i n u n c e r t a i n in adults. In chgildren, large tumor size, lymphnodal involvement and distant metastasis suggest p o o r prognosis21 T h e first National Wilms' T u m o r Study (NWTS) identified a small subset (11 p e r cent) of patients with anaplastic, r h a b d o i d or sarcomatous features, to have p o o r prognosis. T h e s e features are t e r m e d as " u n f a v o u r a b l e histology", while the remaining m o n o m o r p h o u s epithelial tumors are referred to as having a
587
"favourable histology"22 All authors 7,15,~a,24 agree that the adult subset o f Wilms' t u m o r patients b e h a v e "differently" both in terms o f natural history and response to treatment. However, after an extensive review of available literature, we have not f o u n d any consensus regarding the nature of this "difference". Altug et al. 7 stated that the adult Wilms' t u m o r was o f a m o r e malignant pathology t h a n that s e e n in children, but this decision was b a s e d on his 3 case reports o f which one had metastasis to the lungs and a n o t h e r to the brain. Conversely, Jagasia and T h u r m a n ~4 concluded o n the basis o f 12 cases, t h a t the Wilms' tumor in adults may behave in a less malign a n t m a n n e r than that seen in c h i l d r e n . Their series showed survival after the initial diagnosis o f Wilms' t u m o r to range from 3 m o n t h s to 15 years: Owing to the rarity o f this disease, no individual has b e e n able to collect a n d analyse any significantly large series with regard to the types o f histology in adults. Conclusions that can be confidently drawn from the literature, however, are that: in adults, 1. the pattern of lymph node involvement is the same as in childrenY 2. the locations of metastatic deposits are the same as in children ~with lungs being the most c o m m o n site o f metastasis, others b e i n g the liver, bone, skin, orbit, urinary bladder, colon, brain a n d the opposite kidney. 3. the histologic picture is the sante as in children. 9 4. the natural history is different from that in children. 7,a5,~,2~ 5. the stage at diagnosis is more advanced than in children. ~ 6. the response to treatment is p o o r e r than in children. 2 O n the o t h e r hand, the questions which remain unanswered are regarding: 1. the frequency of cases with " p o o r histology" in adults c o m p a r e d to the 11.5 per cent seen in children and,
Jpn. J. Surg. September 1990
Kumar et al.
588
Table 1. National Wilms' Tumor Study Grouping System2~ Group Group Group Group Group
I -II - III-IV-V --
Tumor limited to within the kidney and completely excised. Tumor extending beyond the kidney but completely excised. Residual non haematogenous tumor confined to the abdomen. Haematogenous metastases to the lung, liver, bone or brain. Bilateral renal involvement.
Table 2. Statistics Showing the Survival of Adults Compared to Children with Wilms' Tumoa~,1~ Survival Over-aU Survival 2 years 3 years 3 Year Survival Early stage tumor Stage IV tumor
2.
Adults (%) Children (%) 54 24
75 74
48 11
87 53
the natural history of adult tumors compared to tumors o f the same stage in children. T h e NWTS evolved a clinico-pathological grouping system for childhood Wilms' tumor (Table 1) by which Wilms' tumor in aduks should also be staged. 9,~* No definite treatment schedule for adult Wilms' tumor has b e e n firmly accepted, mainly because of the paucity of cases on which to base inferences. Surgery was the first therapeutic modality to be used and the surgical aspects were reviewed by Leappe 25 who r e c o m m e n d e d a transabdominal approach to allow inspection o f the extent of disease and biopsy o f suspicious lymph nodes. T h o u g h no longer the sole modality of treatment, surgery is an important part o f a n y regimen, serving the purposes of both therapy and diagnosis. 26 Along with surgery, radiation therapy aimed at giving up to 4000 rads to the operation site, and combination chemotherapy using Actinomycin D (AMD), Vincristine (VCR) and Doxorubicin (DRB) are now universally accepted as essential for the treatment of adult Wilms' tumors. 26 Nevertheless, specific indications for the use o f this multi-
modal therapy remain controversial. Kilt0n et aL suggested the same multimodal therapy for all stages of the disease 14 and based on the adult subset of the NWTS, D'Angio p r o p o s e d surgery, radiation and aggressive three drug chemotherapy for all stages of this diseaseY Graded treatment, based on the clinicopathological staging o f the tumor has also b e e n proposed, which is probably the best option available. 9 T h e following therapeutic guidelines are therefore proposed: Group I--surgery with chemotherapy. Group II--surgery with chemotherapy followed by radiation to the operation site. Group I I I - - s u r g e r y with chemotherapy, followed by radiation to the entire abdomen with shielding of the opposite kidney. Group I V - - t h e same treatm e n t as for Group III, with the addition of radiation to secondary deposits. T h o u g h multimodal therapy in children has seen cure rates approach 90 per cent, adults with Wilms' tumor have not fared as well, with cure rates o f only about 25 per cent ~ having b e e n reported (Table 2). Nevertheless, multimodal therapy is also the best available treatment for adult Wilms' tumor. However, a concerted and cooperative effort is required to determine the exact prognostic factors and improve the therapeutic alternatives so as to improve survival in this rare disease. (Received for publication on Aug. 1, 1989) REFERENCES 1. Klapproth HJ. Wilms tumor: A report of 45 cases and an analysis o f 1351 cases reported in the world literature from 1940 to 1958. J Urol 1959; 81:
Volume 20 Number 5
Adult Wilms' tumor
633-648. 2. Byrd RL, Evans AE, D'Angio GJ. Adult Wilms' tumor: Effect of combined therapy on survival. J Urol 1982; 127: 648-651. 3. Culp OS, Hartman FW. Mesoblastic nephroma in adults: a clinico-pathologic study of Wilms' tumors and related renal neoplasms. J Urol 1948; 60: 552-576. 4. Esersky GL, Saffer SH, Panoff LE, Jacobi M. Wilms' tumor in the adult. Review of the literature and report of 3 additional cases. J Urol 1947; 58: 397-411. 5. Smith KH. Wilms' tumor in the adult. BrJ Surg 1960; 47: 397-400. 6. Newman D, Vellios F. Adult carcinosarcoma (Adult Wilms' tumor of the kidney. A m J Clin Path 1964; 42: 45-54. 7. Altug M, Carmichael FA, Henry CL, Stockton RW. Wilms' tumor in an adult: Long term survival with palliative resection of lung and brain metastases. J Urol 1964; 91: 212-219. 8. Kocker T, Langhans T. Eine nephrotomie wegen nievensarcom. Deutsche Ztschr F Chir 1878; 9: 312. 9. Babaian RJ, Skinner DG, Wiasman J: Wilms' tumor in the adult patient. Diagnosis, management and review of world medical literature. Cancer 1980; 45: 1713-1719. 10. Chung TS, Reyes CV, Stefani SS. Wilms' tumor in adult. Urology 1984; 24: 275-277. 11. Lurie M, Sova I, Mecz Y, Lurie A. Adult nephroblastoma. Cancer 1988; 61: 2342-2347. 12. Roth DR, Wright J, Cawood CD, Pranke DW. Nephroblastoma in adults. J Urol 1984; 132: 108-110. 13. Koretz MJ. Extrarenal adult Wilms' tumor. Cancer 1987; 60: 2484-2488. 14. Kilton L, Matthews MJ, Cohen MH. Adult Wilms' tumor: a report of prolonged survival and review of literature. J Urol 1980; 124: 1-5. 15. Olson BF, Bischoff AJ. Wilms' tumor in an adult. Cancer 1970; 25: 21-25.
589
16. Bodian M, Rigby CC. The pathology of nephroblastoma. In: Tumors of the kidney and ureter. Riches, E. (Ed). Baltimore, Williams and Wilkins 1964, pp 219-234. 17. Cope JR, RoylanceJ, Gordon 1RS. The radiological features of Wilms' tumor. Clin Rad 1972; 23: 331-339. 18. Kumar R. Adult Wilms tumor: Clinical and radiographic features. Urol Radiol 1984; 6: 164-169. 19. Chien-Hsing M, Elkin M. Angiography manifestations of Wilms' tumor. AmJ Roentgenol 1969; t05: 95-104. 20. Fishman EK, Hartman DS, Goldman SM, Siegelman SS. The CT appearance ofWilms' tumor.J Comput Assist Tomog 1983; 7: 659-664. 21. Breslow NE, Palmer NF, Hill LR, BuringJ, D'Angio GJ. Wilms' tumor: Prognostic factors for patients without metastases at diagnosis. Results from the first National Wilms' Tumor Study. Cancer 1978; 41: 1577-1589. 22. BeckwithJB, Palmer NF. Histopathology and prognosis of Witms' tumor--Results from the first National Wilms' Tumor Study. Cancer 1978; 41: 1937-1948. 23. Chang SY, Ma CP, Lee WH. Adult Wilms' tumor. J Surg Oncol 1988; 39: 283-286. 24. Jagasia KH, Thurman WG. Wilms' tumor in the adult. Arch Int Med 1965; 115: 322-327. 25. Leappe LL. Diagnosis and management of Wilms' tumors. In: Genitourinary cancer. Skinner, D.G., deKernion,J.B. (Eds). W.B. Saunders, Philadelphia 1978, pp 179-199. 26. Hagiwara M, Tachibana M, Jitsukawa S, Mural M, Nakazono M, Hata M, Tazaki H. Multimodal treatment of advanced adult Wilms' tumor. J Urol 1982; 127: 535-538. 27. D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, WoelfJ. The treatment of Wilms' tumor: Results of the second National Wilms Tumor Study. Cancer 1981; 47: 2302-2311.
Wilms' tumor, nephroblastoma
INTRODUCTION
W
i
l
m
s
'
tumor is the most c o m m o n renal tumor in children and accounts for 20 per cent o f all childhood neoplasms? T h e introduction o f multimodal therapy, however, combining surgery, radiation therapy and chemotherapy has improved the survival o f these children to up to 90 per c e n C In adults, the true incidence o f this tumor is difficult to ascertain, but it is certainly very rare. T h e variation in the n u m b e r o f cases reported reflects the confusion in histologic features and nomenclature existing in the past? -7 In comparison to children, however,
The Departments of Surgery* and Pathology, ** All India Institute of Medical Sciences, New Delhi, India Reprint requests to: Brij Kapur, MD, The Department of Surgery, All India Institute of Medical Sciences, New Delhi, India
Wilms' tumor in adults is diagnosed at a more advanced clinical stage and the prognosis is much worse. 2 We report herein, a well documented case o f Wilms' tumor in an adult. We also present an exhaustive updated review o f the world medical literature and give guidelines for establishing the diagnosis and managing this rare disease. CASE REPORT A 48 year old male was admitted to our hospital in July, 1988 with a one and a half year history o f a progressively increasing, asymptomatic lump in the left hypochondrium without hematuria. Examination revealed a well patient with mild pallor, no lymphadenopathy, a pulse rate o f 8 2 / m and a blood pressure o f 115/90 mmHg. Abdominal examination revealed a
JAPANESEJOURNALOFSURGERY,VOL.20, No. 5 pp. 585-589, 1990
586
Kumar et at.
Fig: 1. Photomicrograph of the tumor showing a stroma of poorly differentiated cells. (HE • large, firm, irregular and tender mass in the left hypochondrium extending into the lumbar region. T h e mass was bimanually palpable and the left renal angle was dull on percussion. His chest X-ray was normal. An intravenous u,rogram revealed a normally placed and functioning right kidney, but the left kidney could not be visualized. CT scan of the abdomen revealed a large, mixed attenuating mass replacing the left kidney which itself was not seen separately. T h e r e was no extension into the renal vein. A preliminary clinical diagnosis of left renal cell carcinoma was made and the patient scheduled for surgery. Laparotomy subsequently revealed the left kidney to be replaced by a large, fleshy, well encapsulated tumor without any infiltration into the surrounding structures. T h e tumor was not extending into the renal vein. A left radical nephrectomy was done and the patient had an uneventful postoperative recovery. After the histological diagnosis had b e e n established, the patient was advised to undergo chemotherapy and local radiotherapy but he absconded and was lost to follow up. O n pathological examination, the kidney measured 30 X 20 X 8 cm and weighed 4700 g. T h e external surface was irregular and the capsule infiltrated at the upper pole. O n sectioning, the tumor involved the entire
Jpn. J. Surg. September 1990
Fig. 2. Photomicrograph of another area of the same tumor showing abortive tubular differentiation. (HE X240) kidney with areas o f hemorrhage, necrosis and cystic degeneration. T h e pelvicalyceal system was infiltrated with the tumor, though blood vessels at the hilum were free. O n microscopy, the tumor was poorly differentiated, comprising mostly of small r o u n d cells with hyperchromatic nuclei (Fig. 1). Focal areas o f abortive embryonal tubular as well as glomerular differentiation were seen (Fig. 2). These features were compatible with Wilms' tumor. DISCUSSION T h e first case o f Wilms' tumor in an adult was reported by Kocker and Langhans in 18788 and a review o f literature up to 1980 by Babaian et al. yielded a total of only 167 cases. 9 However, in the world literature, spanning i10 years from 1878 to 1988, we have recorded a total o f 211 casesY - ~ Culp and Hartman noted fifty three synonyms for Wilms' tumor, 3 yet considering the confusion in terminology and the propensity of renal cell carcinomas which mimic its histologic features, some of these may not represent a true Wilms' tumor. 9,a4 Controversy regarding the terminology of this tumor abated after the introduction of the term "nephroblastoma". This term reflects the accepted theory of the tumor's origin from metanephrogenic blastoma, the precursor of both the nephrons and intersti-
Volume 20 Number 5
Adult Wilms' tumor
tium o f the kidney? 5,~6 Presently, only two interchangeable terms remain-nephroblastoma, and Wilms' tumor; we prefer to use the latter. The diagnostic pathological features of Wilms' t u m o r in both adults and children are identical, Qthe most important feature being the p r e s e n c e o f abortive e m b r y o n a l g! omeruli in an immature spindle cell stroma? 6 Renal cell c a r c i n o m a s h a v i n g g l a n d u l a r snaactures along with sarcomatous or undifferentiated cells are difficult to distinguish from it, but Wilms' tumors do not contain the mature glandular structures seen in renal cell carcinoma? ~ Because the t u m o r is rare in adults, i t is difficult to d i a g n o s e p r e o p e r a t i v e l y . I n Eserskey's series o f 56 cases, n o n e were diagnosed correctly before surgerya as there is no test to differentiate it f r o m renal cell carcinoma. About two thirds o f these patients present with pain, while an abdominal mass a n d / o r hematuria is seen in three quarters.14 A plain abdominal X-ray may reveal fine calcification in 10-30 p e r cent? 7 Intravenous urograms show a space occupying lesion or n o n visualization o f the affected kidney TM and angiography may reveal varying degrees o f vascularity including a "creeping vine" or "spaghetti" pattern in some. 19CT scan usually shows a n o n h o m o g e n o u s intrarenal mass and is also useful in evaluating the presence of multiple masses, the extent o f the tumor, major vessel i n v o l v e m e n t a n d s e c o n d a r y spread. 2~ T h o u g h well established in children, the p r o g n o s t i c f a c t o r s r e m a i n u n c e r t a i n in adults. In chgildren, large tumor size, lymphnodal involvement and distant metastasis suggest p o o r prognosis21 T h e first National Wilms' T u m o r Study (NWTS) identified a small subset (11 p e r cent) of patients with anaplastic, r h a b d o i d or sarcomatous features, to have p o o r prognosis. T h e s e features are t e r m e d as " u n f a v o u r a b l e histology", while the remaining m o n o m o r p h o u s epithelial tumors are referred to as having a
587
"favourable histology"22 All authors 7,15,~a,24 agree that the adult subset o f Wilms' t u m o r patients b e h a v e "differently" both in terms o f natural history and response to treatment. However, after an extensive review of available literature, we have not f o u n d any consensus regarding the nature of this "difference". Altug et al. 7 stated that the adult Wilms' t u m o r was o f a m o r e malignant pathology t h a n that s e e n in children, but this decision was b a s e d on his 3 case reports o f which one had metastasis to the lungs and a n o t h e r to the brain. Conversely, Jagasia and T h u r m a n ~4 concluded o n the basis o f 12 cases, t h a t the Wilms' tumor in adults may behave in a less malign a n t m a n n e r than that seen in c h i l d r e n . Their series showed survival after the initial diagnosis o f Wilms' t u m o r to range from 3 m o n t h s to 15 years: Owing to the rarity o f this disease, no individual has b e e n able to collect a n d analyse any significantly large series with regard to the types o f histology in adults. Conclusions that can be confidently drawn from the literature, however, are that: in adults, 1. the pattern of lymph node involvement is the same as in childrenY 2. the locations of metastatic deposits are the same as in children ~with lungs being the most c o m m o n site o f metastasis, others b e i n g the liver, bone, skin, orbit, urinary bladder, colon, brain a n d the opposite kidney. 3. the histologic picture is the sante as in children. 9 4. the natural history is different from that in children. 7,a5,~,2~ 5. the stage at diagnosis is more advanced than in children. ~ 6. the response to treatment is p o o r e r than in children. 2 O n the o t h e r hand, the questions which remain unanswered are regarding: 1. the frequency of cases with " p o o r histology" in adults c o m p a r e d to the 11.5 per cent seen in children and,
Jpn. J. Surg. September 1990
Kumar et al.
588
Table 1. National Wilms' Tumor Study Grouping System2~ Group Group Group Group Group
I -II - III-IV-V --
Tumor limited to within the kidney and completely excised. Tumor extending beyond the kidney but completely excised. Residual non haematogenous tumor confined to the abdomen. Haematogenous metastases to the lung, liver, bone or brain. Bilateral renal involvement.
Table 2. Statistics Showing the Survival of Adults Compared to Children with Wilms' Tumoa~,1~ Survival Over-aU Survival 2 years 3 years 3 Year Survival Early stage tumor Stage IV tumor
2.
Adults (%) Children (%) 54 24
75 74
48 11
87 53
the natural history of adult tumors compared to tumors o f the same stage in children. T h e NWTS evolved a clinico-pathological grouping system for childhood Wilms' tumor (Table 1) by which Wilms' tumor in aduks should also be staged. 9,~* No definite treatment schedule for adult Wilms' tumor has b e e n firmly accepted, mainly because of the paucity of cases on which to base inferences. Surgery was the first therapeutic modality to be used and the surgical aspects were reviewed by Leappe 25 who r e c o m m e n d e d a transabdominal approach to allow inspection o f the extent of disease and biopsy o f suspicious lymph nodes. T h o u g h no longer the sole modality of treatment, surgery is an important part o f a n y regimen, serving the purposes of both therapy and diagnosis. 26 Along with surgery, radiation therapy aimed at giving up to 4000 rads to the operation site, and combination chemotherapy using Actinomycin D (AMD), Vincristine (VCR) and Doxorubicin (DRB) are now universally accepted as essential for the treatment of adult Wilms' tumors. 26 Nevertheless, specific indications for the use o f this multi-
modal therapy remain controversial. Kilt0n et aL suggested the same multimodal therapy for all stages of the disease 14 and based on the adult subset of the NWTS, D'Angio p r o p o s e d surgery, radiation and aggressive three drug chemotherapy for all stages of this diseaseY Graded treatment, based on the clinicopathological staging o f the tumor has also b e e n proposed, which is probably the best option available. 9 T h e following therapeutic guidelines are therefore proposed: Group I--surgery with chemotherapy. Group II--surgery with chemotherapy followed by radiation to the operation site. Group I I I - - s u r g e r y with chemotherapy, followed by radiation to the entire abdomen with shielding of the opposite kidney. Group I V - - t h e same treatm e n t as for Group III, with the addition of radiation to secondary deposits. T h o u g h multimodal therapy in children has seen cure rates approach 90 per cent, adults with Wilms' tumor have not fared as well, with cure rates o f only about 25 per cent ~ having b e e n reported (Table 2). Nevertheless, multimodal therapy is also the best available treatment for adult Wilms' tumor. However, a concerted and cooperative effort is required to determine the exact prognostic factors and improve the therapeutic alternatives so as to improve survival in this rare disease. (Received for publication on Aug. 1, 1989) REFERENCES 1. Klapproth HJ. Wilms tumor: A report of 45 cases and an analysis o f 1351 cases reported in the world literature from 1940 to 1958. J Urol 1959; 81:
Volume 20 Number 5
Adult Wilms' tumor
633-648. 2. Byrd RL, Evans AE, D'Angio GJ. Adult Wilms' tumor: Effect of combined therapy on survival. J Urol 1982; 127: 648-651. 3. Culp OS, Hartman FW. Mesoblastic nephroma in adults: a clinico-pathologic study of Wilms' tumors and related renal neoplasms. J Urol 1948; 60: 552-576. 4. Esersky GL, Saffer SH, Panoff LE, Jacobi M. Wilms' tumor in the adult. Review of the literature and report of 3 additional cases. J Urol 1947; 58: 397-411. 5. Smith KH. Wilms' tumor in the adult. BrJ Surg 1960; 47: 397-400. 6. Newman D, Vellios F. Adult carcinosarcoma (Adult Wilms' tumor of the kidney. A m J Clin Path 1964; 42: 45-54. 7. Altug M, Carmichael FA, Henry CL, Stockton RW. Wilms' tumor in an adult: Long term survival with palliative resection of lung and brain metastases. J Urol 1964; 91: 212-219. 8. Kocker T, Langhans T. Eine nephrotomie wegen nievensarcom. Deutsche Ztschr F Chir 1878; 9: 312. 9. Babaian RJ, Skinner DG, Wiasman J: Wilms' tumor in the adult patient. Diagnosis, management and review of world medical literature. Cancer 1980; 45: 1713-1719. 10. Chung TS, Reyes CV, Stefani SS. Wilms' tumor in adult. Urology 1984; 24: 275-277. 11. Lurie M, Sova I, Mecz Y, Lurie A. Adult nephroblastoma. Cancer 1988; 61: 2342-2347. 12. Roth DR, Wright J, Cawood CD, Pranke DW. Nephroblastoma in adults. J Urol 1984; 132: 108-110. 13. Koretz MJ. Extrarenal adult Wilms' tumor. Cancer 1987; 60: 2484-2488. 14. Kilton L, Matthews MJ, Cohen MH. Adult Wilms' tumor: a report of prolonged survival and review of literature. J Urol 1980; 124: 1-5. 15. Olson BF, Bischoff AJ. Wilms' tumor in an adult. Cancer 1970; 25: 21-25.
589
16. Bodian M, Rigby CC. The pathology of nephroblastoma. In: Tumors of the kidney and ureter. Riches, E. (Ed). Baltimore, Williams and Wilkins 1964, pp 219-234. 17. Cope JR, RoylanceJ, Gordon 1RS. The radiological features of Wilms' tumor. Clin Rad 1972; 23: 331-339. 18. Kumar R. Adult Wilms tumor: Clinical and radiographic features. Urol Radiol 1984; 6: 164-169. 19. Chien-Hsing M, Elkin M. Angiography manifestations of Wilms' tumor. AmJ Roentgenol 1969; t05: 95-104. 20. Fishman EK, Hartman DS, Goldman SM, Siegelman SS. The CT appearance ofWilms' tumor.J Comput Assist Tomog 1983; 7: 659-664. 21. Breslow NE, Palmer NF, Hill LR, BuringJ, D'Angio GJ. Wilms' tumor: Prognostic factors for patients without metastases at diagnosis. Results from the first National Wilms' Tumor Study. Cancer 1978; 41: 1577-1589. 22. BeckwithJB, Palmer NF. Histopathology and prognosis of Witms' tumor--Results from the first National Wilms' Tumor Study. Cancer 1978; 41: 1937-1948. 23. Chang SY, Ma CP, Lee WH. Adult Wilms' tumor. J Surg Oncol 1988; 39: 283-286. 24. Jagasia KH, Thurman WG. Wilms' tumor in the adult. Arch Int Med 1965; 115: 322-327. 25. Leappe LL. Diagnosis and management of Wilms' tumors. In: Genitourinary cancer. Skinner, D.G., deKernion,J.B. (Eds). W.B. Saunders, Philadelphia 1978, pp 179-199. 26. Hagiwara M, Tachibana M, Jitsukawa S, Mural M, Nakazono M, Hata M, Tazaki H. Multimodal treatment of advanced adult Wilms' tumor. J Urol 1982; 127: 535-538. 27. D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, WoelfJ. The treatment of Wilms' tumor: Results of the second National Wilms Tumor Study. Cancer 1981; 47: 2302-2311.
