Wilms' tumor and multicystic dysplastic kidney disease

George W Holcomb

doi:10.1016/S0022-3468(98)90579-6

$INTERNATIONAL ABSTRACTS cant, and these parameters were not predictive of a good outcome. Treatment with DDAVP can produce a socially acceptable level of dryness in some patients with refractory nocturnal enuresis, but moming osmolality was not useful in predicting success. It was interesting to note that enuresis was worse with placebo in the crossover part of the study than it was in the baseline phase.-M.N. de la Hunt Study Group. D.M. Green, N.E. Breslow, Clin Oncol 16:237-245, (January), 1998. J.B. Beckwith, etal. NEOPLASMS Wilmsâ Tumor in a Prenatally Diagnosed Multicystic A.G. De Oliveira-Filho, M.H. Carvalho, L. Sbragia-Neto, Urol 158:1926-1927, (November), 1997. Kidney. eta/. J This report from the NWTS-4 Study showed that single-dose (pulse intensive [PI]) combination chemotherapy for either low-risk or highrisk Wilmsâ tumor patients was equivalent in efficacy to divided-dose regimens. Because of the reduced hematologic toxicity and decreased physician/hospital encounters associated with this PI regimen, the PI schedule has become the regimen of choice.-Michael P. Hirsh Although conservative treatment of multicystic dysplastic kidney is generally accepted, there are reports of complications when such a kidney is retained, particularly malignant degeneration. The authors report on a neonate with Wilmsâ tumor in a prenatally diagnosed multicystic dysplastic kidney. To their knowledge, this is the fifth case of Wilmsâ tumor arising in a multicystic dysplastic kidney, but only the second diagnosed prenatally.-George W Holcomb, Jr Editorial Comment: The authors are to be congratulated on following through and identifying Wilmsâ tumor in a prenatally diagnosed multicystic kidney. As noted by Beckwith in previous communications on multicystic kidneys. true multicystic kidneys do not have an increased risk of Wilmsâ tumor. In his experience with more than 8,500 pediatric renal tumors. only three cases of Wilmsâ tumor arising in a dysplastic kidney were encountered. All these cases have been filed in the National Wilmsâ Tumor Study. Because of his data and those of the Multicystic Kidney Registry, we believe that these patients should be followed, but we should continue to question the need for nephrectomy in all with multicystic kidney disease. It has been stressed that these patients should be followed closely every 3 to 6 months in the first year of life. Also, ultrasound scans should be reviewed by the urologist or surgeon with the radiologist to ensure that multicystic kidney has been diagnosed correctly.-Jeffrey Wacksmnn Wilmsâ Tumor and Multicystic Dysplastic Kidney YL. Homsy, J.H. Anderson, K. Oudjhane, etal. J Urol 2260, (December), 1997. Disease. 158:2256- PET FDG Studies of Wilmsâ Tumors. B.L. Shulkin, E. Change, EJ. Strouse, et al. J Pediatr Hem/Oncol 19:334-338, (July/ August), 1997. This report from the University of Michigan presents three patients with Wilmsâ tumor in whom PET scanning was used to help determine the diagnosis. It establishes the tumor as one that does preferentially absorb FDG isotope, whereas nephroblastomatosis does not. This scan may be a useful adjunct in the event that CT scanning alone fails to differentiate dysmorphic kidney from true Wilmsâ tumor.-Michnel P. Hirsh Pediatric Phaeochromocytoma-A J. Pullerits, R. Creighton, (November), 1997. 36-Year Review. S.H. Ein, et al. Pediatr Surg Int 12:595-598, Two well-documented cases of malignancy associated with multicystic dysplastic kidney disease are presented in two female infants (5 and 3 months old). The 5 month old was followed for multicystic dysplastic kidney disease and had no evidence of tumor either antenatally or at birth. The 3 month old presented with hypertension and an interventricular septal defect. A renal tumor was present on the initial ultrasound scan. Even though malignant degeneration is rare in multicystic dysplastic kidney disease, nine cases have been reported in the literature. Of these cases; three were Wilmsâ tumor, five were renal cell carcinoma. and one was mesothelioma. The authorsâ two cases lend support to the surgical management of multicystic dysplastic kidney disease, particularly because nephrectomy can now be performed in a day-surgery setting with minimal morbidity. Only the risks of coexisting malignancy and possible malignant degeneration transformation are addressed specifically in this report. Other complications of multicystic dysplastic kidney disease, such as hypertension, infection, abdominal pain, hematuria, and persistent dysplastic renal tissue despite ultrasonographic resolution of multicystic dysplastic kidney disease; are additional risk factors to be considered. Recommendation for nephrectomy in cases of multicystic dysplastic kidney disease cannot be based solely on these two cases. Several other factors must be weighed before that decision can be made.-George w Holcomb, Jr Comparison Between Single-Dose and Divided-Dose Administration of Dactinomycin and Doxorubicin for Patients With Wilmsâ Tumor: A Report From the National Wilmsâ Tumor The authors report on 14 children with pheochromocytoma treated at the Hospital for Sick Children, Toronto, from 1959 to 1995. The male:female ratio was 2.5: 1. The 14 patients had a total of 20 tumors. (One boy had 4 tumors; 2 girls and 1 boy had 2 each.) A familial history of phaechromocytoma, van Hippel-Lindau syndrome, thyroid carcinoma, or renal stones was encountered in four cases. No patient had multiple endocrine neoplasia syndrome. Hypertension was the most common finding, and sweating, headache, and visual blurring were the most common symptoms. Before 1980, assessment of urinary catecholamines and plasma renin activity provided the most accurate diagnostic tools, but vanillin mandelic acid and metanepluine also were used. In recent years, urinary dopamine, norepinephrine, and epinephrine usually were used, and norepinephrine was positive in all examined patients. Ultrasonography and computed tomography were introduced during the late 1970s and replaced intravenous pyelography and angiography as reliable radiological diagnostic methods. Sixteen of the phaeochromocytomas were adrenal and four were extra-adrenal in origin. The tumors were radically resected in all cases, except two malignant phechromocytomas. Hypertension was cured in all patients, but four patients had a total of five recurrent tumors. The recurrent tumors could be treated, and all patients are alive. However, one patient with a malignant phechromocytoma remains on chemotherapy 8 years after surgery and has bone metastasis. This patient had a bile fistula, obstructive jaundice, chronic pain, and cirrhosis after operation. Another patient had extradural metastasis causing neurological symptoms but recovered after chemotherapy. The recurrences presented with symptoms reminiscent of those associated with the primary tumors. The authors conclude by recommending that patients with pheochromocytomas have long-term follow-up, probably lifelong, because of the possibility of recurrence.--P. Puri Primary Ewingâs Sarcoma of the Bladder Associated Elevated Antinuclear Antibody Titer. AI. Gousse, E.J. Popek, eta/. J Urol 158:2265-2266, (December), With an D-R. Roth, 1997. Lower urinary tract malignancy is an uncommon cause of hematuria in children. The most common pediatric bladder malignancies are rhabdomyosarcoma, leiomyosarcoma, and transitional cell carcinoma. The authors report a case of primary Ewingâs sarcoma of the bladder$

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Wilms' tumor and multicystic dysplastic kidney disease

Holcomb, George W

Follow Journal of Pediatric Surgery , Volume 33 (7) Elsevier – Jul 1, 1998

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Wilms' tumor and multicystic dysplastic kidney disease

Holcomb, George W

Follow Journal of Pediatric Surgery , Volume 33 (7) Elsevier – Jul 1, 1998

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