Anaplastic sarcoma arising in a mature metachronous bilateral Wilms' tumor after irradiation and chemotherapy spontaneous versus induced malignant change
✍ Scribed by Joseph E. Oesterling; Joseph C. Eggleston; Robert D. Jeffs; Brigid G. Leventhal
- Publisher
- John Wiley and Sons
- Year
- 1987
- Tongue
- English
- Weight
- 883 KB
- Volume
- 59
- Category
- Article
- ISSN
- 0008-543X
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✦ Synopsis
A l-year-old male infant developed a classic Wilms' tumor of the left kidney. Treatment consisted of a left nephrectomy, chemotherapy, and irradiation to the left flank and associated abdomen. Two years later, a mass in the right kidney was discovered; open renal biopsy demonstrated a mature Wilms' tumor consisting entirely of rhabdomyomatous elements in the biopsy specimen. The patient was given a second course of chemotherapy and 2000 rad to the right flank. Over the next 8 years, the mass continued to grow without evidence of metastatic spread. Renal function deteriorated secondary to compression of the surrounding normal renal parenchyma by the enlarging tumor; creatinine clearance from the solitary kidney decreased from 120 ml/min to 40 ml/min during the 12 months prior to removal of the lesion.
Via a nephron-sparing procedure, the 3400 g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms' tumor with a malignant anaplastic sarcoma arising in the central portion. Postoperatively, the patient received a third course of chemotherapy with no irradiation to the tumor bed. Currently, he is disease-free with normal renal function more than 20 years after diagnosis of the metachronous bilateral Wilms' tumor. This is the first reported case of an anaplastic sarcoma arising within a Wilms' tumor; this individual also is the longest surviving patient with metachronous Wilms' tumor. The various possibilities regarding the development of the anaplastic sarcoma within the Wilms' tumor of the right kidney are discussed, including the possible role of chemotherapy and irradiation in the development of a second malignancy.
Cancer 59: 2000-2005. 1987.
ILATERAL WILMS' TUMORS are uncommon lesions. B Only 5.4% (103/1905) of all Wilms' tumors entered into the National Wilms' Tumor Study (NWTS) between October 1969 and March 198 1 were bilateral; 1 .O% ( 19/ 1905) were metachronous bilateral Wilms' tumors, making this variety the rarest type of Wilms' tumor.' The two lesions of metachronous Wilms' tumors are believed to represent distinct primary malignancies rather than metastatic spread and can be separated in time by as much as 10 years.2 Patients with bilateral disease often present at a younger age than do children who have a unilateral Wilms' tumor. In a review of 50 patients with metachronous bilateral Wilms' tumor, Casale ef al. found the median age at the time of presentation of the first tumor to