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CASE REPORT
Year : 2004  |  Volume : 20  |  Issue : 2  |  Page : 60-62
 

Adult wilms' tumor : Three case reports with review of literature


Kolkata, India

Correspondence Address:
Subroto Ranjan Poddar
Dept of Urology, SSKM Hospital, 242, AJC Bose Road, Kolkata-20, West Bengal
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Poddar SR, Chakraborty SC, Das R K, Chatterjee U, Mondol S, Kundu AK, Sarkar PK. Adult wilms' tumor : Three case reports with review of literature. Indian J Urol 2004;20:60-2

How to cite this URL:
Poddar SR, Chakraborty SC, Das R K, Chatterjee U, Mondol S, Kundu AK, Sarkar PK. Adult wilms' tumor : Three case reports with review of literature. Indian J Urol [serial online] 2004 [cited 2019 Sep 15];20:60-2. Available from: http://www.indianjurol.com/text.asp?2004/20/2/60/37175



   Case Report Top


Case 1

In May 2001, a 25- years old male presented with gradually increasing painless left flank lump with abdominal discomfort, nausea & vomiting for last 4 months. Physical Examination revealed a normal built man with palpable huge hard left renal lump (20xl8cm). Ultra sonogram showed a heterogeneous lesion occupying left renal area. CT scan showed a predominantly hypo dense mass (15.2x16.6cm) with rim of enhancement along its periphery displacing bowel loops, pancreas, spleen & stomach [Figure - 1]. The patient was subjected to left radical nephrectomy. Histopathology of nephectomy specimen revealed Wilms' tumor of unfavorable histology. He received a multimodal adjuvant therapy as per NWTS V protocol but died after 4months of follow up because of cancercachexia with lung and liver metastasis.

Case 2

In July 2003, a 21-years old lady presented to us with right flank dull-aching pain for last 6months. Physical examination revealed a bimanual palpable hard lump in right flank. Ultra sonogram showed a well-circumscribed heterogeneous lesion occupying middle and lower pole of right kidney. CT scan showed a well- defined mass, predominantly hypo dense with peripheral rim of enhancement arising from right kidney[Figure - 2] and [Figure - 3]radical nephrectomy. The specimen showed features of Wilms' tumor of favorable histology with positive hilar lymph node (stage III). Her postoperative recovery was smooth. She has been on adjuvant multimodal therapy as per NWTS V protocol. She is on follow up for one-year (till date) and no recurrence or distant metastasis has been seen so far.

Case 3

In August 2003, a 30yrs old normotensive male presented with left flank lump associated with history of intermittent painlessgross total hematuria and intermittent excruciating left flank pain for one year. Physical examination revealed an anemic man with bilateral pedal edema, bilateral grade II varicocele and a hard palpable left flank lump. His blood biochemistry and hematological parameters were normal except low hemoglobin percentage (8g%). Ultra sonogram showed a heterogeneous solid lesion (11.Ox12.Ocm) arising from upper pole of left kidney almost replacing the kidney. CT scan revealed a hetero-dense septated mass measuring

13x11cm having both soft tissue density (HU--+68 to 77) & water density (HU--+18), pushing spleen anteriorly [Figure - 1] renal vein was displaced by the mass with thrombus in it extending to IVC [Figure - 2] Color Doppler ultrasonography showed an IVC thrombus extending upto hepatic vein level. In view of his poor general condition and high surgical risk, we did ultrasound guided FNAC from the SOL. Cytology revealed Wilms' tumor. The patient was advised to have chemotherapy as per NWTS V protocol but died before chemotherapy could be started with evidence of liver metastasis on repeat imaging (ultrasonography) before proposed chemo-radiation therapy (Stage IV)


   Comments Top


Wilms'tumor in the adult age group unlike that of childhood is a rare disease. The prognosis of adult Wilms' tumor is worse than in children [2] . The true incidence of adult Wilms' tumor is somewhat uncertain because of confusion in terminology and difficulties in clinical and pathological differential diagnosis. The diagnostic criteria necessary for adult Wilms' tumor suggested by Kilton et al are: -primary renal neoplasm in age group of more than 15 years with histological features of embryonic glomerulo-tubular structure with immature spindle or round cell stroma and no areas of tumor diagnostic of renal cell carcinoma [3] .The aspiration cytology of adult Wilms' tumor shows blastemal tissue, spindle cell, sarcomatous & rare epithelial components. The cytogenetic changes of isochromosome 17q are frequently seen with these types of tumor in adult age group [4] . The prognosis of adult Wilms' tumor is poorer than that of renal adenocarcinoma [5] . Adult Wilm's tumor is extremely difficult to diagnose preoperatively by imaging. But, in both of our cases, CT scan showed predominantly hypo dense renal mass unlike typical mixed density mass in RCC. Though the observation is too small but it may be a clue to suspect Wilms' tumor in adults and one can go for a needle aspiration cytology & cytogenetic study preoperatively. This approach may help to provide best multimodal management especially for high stage or bilateral adult Wilms' tumor before contemplating a major fruitless surgery. The third case is, of course a very rare case of adult Wilms' tumor with

IVC thrombus, which needs definitive neoadjuvant chemotherapy judiciously to have an acceptable survival.

A definitive treatment plan has not been established yet because of the rarity of these tumors in adults. After surgical removal, multimodal therapy should begin immediately for long lasting, complete remission [2],[8] . Inspite of multimodal approach similar to childhood Wilm's tumor, the treatment outcome in adults is disappointing. The majority of adult Wilms' tumor patients die of metastatic disease [6] . Due to the rarity of adult Wilms' tumor, prospective randomized trials of different adjuvant treatment protocols are not possible till date. Some treatment protocols can only be gathered from few retrospective analyses of reported cases.

 
   References Top

1.Henterich MU, Meister P, Brack NG, LutzLL,Hartenstein RC: Adult Wilms' Tumor. Cancer.1995: 75:545-51.  Back to cited text no. 1    
2.Camei C, Turk HM, Erkilic S, BuyuKberbe S, Uner A, Ozsarac C.Early multimodal therapy in adult Wilm's tumor. J Chemother.2002; 14: 530, 532  Back to cited text no. 2    
3.Kilton L, Methews MJ, Cohen MH. Adult Wilms'tumor. A repot of prolonged survival and review of literature. J. Urol 1980:124:1-5  Back to cited text no. 3    
4.Li P, Perte MA, Scholes JV, Yang GC. Wilms' tumor in adult: aspiration cytology & cytogenetics. Diagn Cytopathol 2002; 26: 103.  Back to cited text no. 4    
5.Ghadouane M, Alami M, Kasmaoun EH, Ameur A, Abbar M. Nephroblastoma in adults. Ann.Urol 2002; 36: 244.  Back to cited text no. 5    
6.Platt BB, Linden G: Wilms' tumor-A comparison of 2 criteria for survival. Cancer 1964; 17: 1573-1578.  Back to cited text no. 6    
7.kattan J, Tournade MF, CulineS, Terrier-LacombeMJ, DrozJP: Adult Wilms tumor Survival: EuroJ. Cancer: 1994:30A(12):1778-82.  Back to cited text no. 7    
8.Byrd RL, Evans AE, D 'Angio GJ: Adult Wilms' Tumor: Effect of combined therapy On survival. J.Urol: 1982: April 127(4); 648-51.  Back to cited text no. 8    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]



 

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