| Abstract|| |
Eighteen children with biopsy-proven Wilm's tumor were diagnosed and treated between 1985-1994 at the King Fahd Hospital of the University, Alkhobar, Saudi Arabia. There were eight males and 10 females. The median age at diagnosis was 2.5 years. Two patients (11%) had stage I, six patients (33%) stage II, eight patients (44%) stage III and one patient each (6%) had stages IV and V respectively. Seventeen patients (94%) had favorable histology and one (6%) had anaplastic type. One patient had bilateral tumor and one had associated aniridia. Follow-up was from 2 to 10 years (mean:5 years). The treatment administered was according to the recommendations of the National Wilm's Tumor Study II and III. Two patients died resulting in an overall disease-free survival of 88.8%. Our study further confirms that Wilm's tumor is a curable malignancy even in advanced stage.
Keywords: Wilm′s tumor, National Wilm′s Tumor Study, Chemotherapy, Radiotherapy, Surgery.
|How to cite this article:|
Al Mulhim I. Wilm's Tumor in Children: A 10-year Experience from the Eastern Province of Saudi Arabia. Saudi J Kidney Dis Transpl 1997;8:123-6
|How to cite this URL:|
Al Mulhim I. Wilm's Tumor in Children: A 10-year Experience from the Eastern Province of Saudi Arabia. Saudi J Kidney Dis Transpl [serial online] 1997 [cited 2019 Dec 16];8:123-6. Available from: http://www.sjkdt.org/text.asp?1997/8/2/123/39383
| Introduction|| |
Wilm's tumor (WT) is an embryonal renal neoplasm which was first described by Wilm in 1899  . It is the fifth most common incidence in the United States is about 7.8 per million children below 15 years of age with an almost equal sex distribution  . The highest reported annual incidence is in Fin- land (10 per million children) and the lowest is 5.1 per million children in Manchester, England ,, . In Saudi Arabia, the reported annual incidence of WT is about 5.5 per million in children under 15 years of age  .
The median age at diagnosis of WT is 3 to 4 years. Bilateral involvement has been reported in 4 to 9.2% of cases ,, . The common presenting clinical features include abdominal mass (83%), abdominal pain (37%), fever (23%), hematuria (21%), hypertension (63%) and intra-abdominal emergency (12%) ,,,, . Prior to 1930, WT was considered incurable; but recently with the advent of good surgical techniques, radiotherapy and adjutant chemotherapy, the prognosis has changed dramatically ,, . We herewith present our experience over 10 years, in treating patients with WT at our hospital.
| Patients and Methods|| |
During the period between January 1985 and December 1994, a total of 18 children under 15 years of age, with biopsy-proven WT were seen at the King Fahd Hospital of the University, Alkhobar, Saudi Arabia. There were eight males and 10 females. Their age ranged from 1 to 5 years with a mean of 2.5 years. Detailed history, physical examination findings, complete blood count, renal function tests, liver function tests as well as results of radiological and pathological studies were available for all patients. The National Wilm's Tumor Study (NWTS) system was used for staging and histopathological classification in our study ,, .
| Results|| |
The clinical features and results of treatment of the 18 study children are presented in [Table - 1]. All patients presented with abdominal mass. In addition, five patients (27.8%) had abdominal pain, three (16.7%) had hematuria and two had hypertension (11%). Of the 18 patients, 17 (94.5%) had favorable histology and one (5.5%) had anaplastic variety. One patient had bilateral tumor and one other had associated aniridia. Two patients (11%) had stage I, six (33%) stage II, eight (44%) stage III and one patient each (5.5%) had stage IV and V. One patient had local recurrence with metastasis to the lungs and liver and expired. Another patient who had ascitis and pulmonary metastasis at diagnosis, showed partial response to treatment but died six months later from progressive disease. The overall disease free survival was 88.8% and survival for stage I and II was 100%.
The treatment protocol used was as recommended by the second and third NWTS and included surgery, radiotherapy and chemotherapy , . The details are as follows:
Stage I: nephrectomy followed by chemotherapy for six months,
Stage II: nephrectomy, chemotherapy for 15 months and radiotherapy for patients above two years of age,
Stage III: nephrectomy, radiotherapy and chemotherapy for 15 months,
Stage IV: same as stage III plus lung irradiation,
Stage V (bilateral): nephrectomy on the side with the larger tumor, partial resection of small tumor with radiotherapy and chemotherapy for 15 months.
For large unresectable tumors, two courses of vincristine and actinomycin D were given to shrink the tumor and allow second look surgery. Chemotherapy consisted of vincristine 1.5 mg/m 2 i.v. push and actionmycin D 15 µg/kg i.v. daily for five days. For stages III and IV, and those with unfavorable histology, adriamycin 60 mg/m 2 i.v. (total cumulative dose not exceeding 450 mg/m 2 ), was added. The dose of radiotherapy was 2000 CGS and used only for those above two years of age with stage II-IV or those with unfavorable histology.
| Discussion|| |
Improvement in the surgical management of WT during the period 1900 to 1960 has altered its prognosis from a uniformly fatal disease to a disease with 40% 2-year survival. With the addition of radiotherapy, survival increased to about 50% , . Subsequently, chemotherapy has been used with a fair degree of success in the treatment of metastatic disease. Farber and his colleagues in 1956, demonstrated clinical response of metastatic disease to dactinomycin with a 37% survival at 2 years  . In 1963, Sutow, et al demonstrated temporary response of metastatic WT to vincristine in 8 of 12 children  . The addition of actinomycin D to surgery and radiotherapy improved the two-year survival to 80% among those without metastasis at diagnosis, and to 53% among those who had metastasis  . Fernback, et al confirmed these early findings by obtaining a 92% survival with surgery, post-operative radiation and dactinomycin  . In another study, a combination of pulmonary and abdominal irradiation with vincristine resulted in a complete regression of metastatic disease in 73% of their patients and a 45% two-year survival was achieved  .
The National Wilm's Tumor Study I (NWTS I) demonstrated the effectiveness of the combination of vincristine and dactinomycin. In stage II and III patients, dactinomycin alone resulted in a 67% twoyear survival compared to 72% for vincristine. Combination of both these drugs resulted in a 82% two-year survival  . The next major advance has been the utilization of doxorubicin (adriamycin). This was evaluated by six oncology groups and showed 67% complete or partial response  . In NWTS II, the addition of adriamycin for stage IIIV improved the survival further  .
Our patients, although small in number, had the classical presentation of WT. All were under five years of age with an almost equal sex distribution. All had abdominal swelling as the initial manifestation with or without abdominal pain or hematuria. Most cases were in stage I, II or III except one who had stage IV with ascitis and pulmonary metastasis.
We treated our patients according to the recommendation of NWTS II, III. All the patients tolerated chemotherapy without developing any toxicity. Our overall survival rate of 88.8% is comparable to the results of NWTS III and other workers as well ,, .
The reported experience with this malignancy in Saudi Arabia is limited. A review of 12 patients from the Western region of this country, between 1983 and 1987, revealed survival in 83% of the cases  . Another report from Riyadh on five patients with advanced stage of the disease showed survival in 40% of the cases .
In conclusion, WT is a curable malignancy even in advanced stage and needs only minimal chemotherapy. Stage of the disease and histology are the most important prognostic factors. Further studies are necessary to further refine therapy in order to obtain maximum cure with less morbidity and long-term sequelae.
| References|| |
|1.||Water UW, Suto W, Donald J, Fern Bac H, Teresa J, Veitti. Clinical Pediattic Oncology 1984;25:588. |
|2.||Young JL Jr, Miller RW. Incidence of malignant tumors in U.S. Children. J Pediatrl975;86:245-8. |
|3.||Birch JM, Marsden HB, Swindell R. Incidence of malignant disease in childhood: a 24-year review of the Manchester Children's Tumor Registry data. Br J Cancer 1980;42:215-23. |
|4.||Ericson J, Karnstorm L, Mattson B. Childhood Cancer in Sweden 1958-1977, Acta Pediatr Scand 1978;67:425-32. |
|5.||McWhirter WR, Bacon JE. Incidence of childhood tumours in Queensland. Br J Cancer 1981;44:637-42. |
|6.||Al-Hamdan N, Al-Zahrani A, Koriech O. National Cancer Registry 1994 Report, May 1996 MOH, Saudi Arabia. |
|7.||Bishop HC, Tefft M, Evans AE, D'Angio GJ. Survival in bilateral Wilms' tumor. Review of 30 national Wilms' tumor study cases. J Pediatr Surg 1977;12:631-8.Bond JV. Bilateral Wilms' tumor. Cancer 1978;2:482-4. |
|8.||Wasiljcw BK, Besscr A, Rcffenspcrger J. Treatment of bilateral Wilms' tumors- a 22-year experience. J Pediatr Surg 1982;17:265-8. |
|9.||Aron BS. Wilms' tumor: a clinical study of 81 patients. Cancer 1974;33:637-46. |
|10.||Pearson D, Duncan WB, Pionton BCS. Wilms 1 tumor: a review of cases. Br J Radiol 1984;37:154-60. |
|11.||Green DM, Jaffe N. Wilms' tumor-model of a curable pediatric malignant solid tumor. Cancer Treat Rev 1978;5:143-72. |
|12.||Lemcrle J, Tournade MF, Marchant RG, et al. Wilm's tumor: natural history and prognostic factors: a retrospective study of 248 cases treated at the Institute Gustave Roussy 1952-1967. Cancer 1976;37:2557-66. |
|13.||Sukarochana K, Tolentino W, Kiesewetter WB. Wilms' tumor and hypertension. J Pediatr Surg 1972;7:573-6. |
|14.||D'Angio GJ, Evans AE, Breslow N, et al. The treatment of Wilms' tumor: results of the national Wilms' tumor study. Cancer l976;38:633-46. |
|15.||D'Angio GJ, Evans A, Breslow N, et al. The treatment of Wilms' tumor: results of the second national Wilms' tumor study. Cancer 1981;47:2302-11. |
|16.||D'Angio GJ, Breslow N, Beckwith JB, et al. Treatment of Wilms 1 tumor. Results of the third national Wilms' tumor study. Cancer 1989;64:349-60. |
|17.||Gross RE, Neuhauser EB. Treatment of mixed tumors of the kidney in childhood. Pediatrics 1950;6:843-52. |
|18.||Farber S. Clinical studies of actinomycin D with special reference to Wilms' tumor in children. Ann NY Acad Sci 1960;89:421-5. |
|19.||Sutow WW, Thurman WG, Windmiller J, et al. Vincristine sulphate in the treatment of children with metastatic Wilms' tumor. Pediatrics 1963;32:880-7. |
|20.||Farber S. Chemotherapy in the treatment of leukemia and Wilms' tumor. JAMA 1966;198:826-36. |
|21.||Fernback DJ, Martyn D, et al. Role of Dactinomycin in the improved survival of children with Wilms' tumor. JAMA 1966;195:1005-9. |
|22.||Vietti TJ, Sullivan MP, Haggard HE, Holcomb TM, Berry DH. Vincristine sulphate and radiation therapy in metastatic Wilms' tumor. Cancer 1970;25:12-20. |
|23.||Tan C, et al. Adriamycin (NSC-123127) in pediatric malignancies. Cancer Chemotherapy Rep 1975;(3)6:259-66. |
|24.||Tournade MF, Com-Nougue C, Voute PA, et al. Results of the Sixth International Society of Pediatric Oncology Wilms' Tumor Trial and Study: a risk-adopted therapeutic approach in Wilms' tumor. J Clin Oncol 1993;ll(6):1014-23. |
|25.||Pritchard J, Imeson J, Barnes J, et al. Results of the United Kingdom Children's Cancer Study Group first Wilms' Tumor Study. J Clin Oncol 1995;13(l):124-33. |
|26.||Rajih S, Sabbah MD, Rhomcs JA, et al. Management of the debilitated child with massive Wilms' tumor. KFSH Med J 1983;3(l):21-4. |
|27.||Hassan MA Faris, Hisham A, Mosli, Mohammed M, Rawas C, et al. Annal of Saudi Medicine 1989;9(6):576-78. |
Ibrahim Al Mulhim
Department of Pediatrics, King Fahd Hospital of the University, King Faisal University, P.O. Box 40093, Alkhobar 31952
[Table - 1]