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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 20  |  Issue : 5  |  Page : 835-837
Adult wilms' tumor


1 Department of General Surgery, GTB Hospital, Dilshad Garden, Delhi, India
2 Department of General Surgery, AMC & Hospital, Dibrugarh, Assam, India

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Date of Web Publication2-Sep-2009
 

   Abstract 

Adult Wilms' Tumor (AWT) is a rare entity arising from the metanephric blastema. There are only about 200 cases reported in world literature. The staging of AWT is done in the same way as in children according to the National Wilms' Tumour Stage Group (NWTSG). Defini­tive treatment plans for AWT are undefined but surgical treatment has the highest priority. There is also consensus on the need for multimodality approach. We report a case of AWT who remains disease free, three years after undergoing multimodality treatment.

How to cite this article:
Sharma MS, Ahmed MZ. Adult wilms' tumor. Saudi J Kidney Dis Transpl 2009;20:835-7

How to cite this URL:
Sharma MS, Ahmed MZ. Adult wilms' tumor. Saudi J Kidney Dis Transpl [serial online] 2009 [cited 2019 Nov 19];20:835-7. Available from: http://www.sjkdt.org/text.asp?2009/20/5/835/55372

   Introduction Top


Wilms' tumor is a malignant embryonic renal neoplasm that is rare in adults. It has an inci­dence of less than 0.2 per million per year. [1] The rarity of the disease, lack of understanding about its biological behaviour and the absence of clear guidelines about its management makes it a clinician's nightmare. Herewith, we report a case of adult Wilms' tu­mor (AWT). The patient was successfully ma­naged with multimodality treatment and re­mains disease free, three years after treatment.


   Case History Top


An 18-year-old female presented with chief complaint of sudden onset of pain abdomen which started in the left upper abdomen with radiation to the back. There was no history of fever, urinary symptoms or weight loss. Exami­nation revealed pallor with a huge (15 × 12 cm) smooth, hard and tender lump in the left upper abdomen. The lump was bimanually palpable and was not mobile. Routine investigations revealed a hemoglobin (Hb) level of 8.7 g/dL and nor­mal urine analysis. Ultrasound examination of the abdomen showed an echogenic area mea­suring 15.1 × 14 cm in the upper pole of the left kidney. Intravenous urography (IVU) showed a space occupying lesion (SOL) in the upper pole of the left kidney displacing the pelvi-calyceal system inferiorly. A contrast enhanced compu­terized tomographic (CECT) scan of the abdo­men revealed a heterogenously attenuated, well encapsulated mass lesion (20.0 × 16.6 × 15.6 cm) in the upper pole displacing the kidney cau­dally and ventrally. The renal veins and inferior vena cava (IVC) were normal. The impression at this stage was a malignant left renal mass.

Based on these findings, the patient was taken up for surgery. The incision used was a left thoraco-abdominal one. The operative findings included a normal right kidney, absence of lym­phadenopathy on the left side, a hugely enlarged left kidney adherent to the diaphragm, muscles of the posterior abdominal wall and the spleen. The left renal vein and the IVC were free of tumor thrombus. A radical nephrectomy was performed along with splenectomy and excision of the adherent portions of the diaphragm and the retroperitoneal muscles. The post-operative period was uneventful.

The excised left kidney weighed 3.5 kg and the cut section showed a thick capsule with mul­tiple areas of necrosis and hemorrhage [Figure 1]. Histopathological examination of the kidney showed a malignant tumor composed of un­differentiated blastema and spindle shaped me­senchymal cells. The epithelial component was seen as multiple proglomeruli and the impre­ssion given was that of a nephroblastoma [Figure 2].

The patient was administered chemotherapy with pulse intensive Actinomycin-D (0.5 mg/day × 5 days) and Vincristine (1.4 mg/sqm × 1 day) for 18 weeks.

The patient is doing well and remains on follow-up three years after completion of treat­ment.


   Discussion Top


Wilms' tumor is a malignant embryogenic tu­mor of the kidney arising from the metanephric blastema. While it is the commonest renal neo­plasm in children, accounting for about 20% of all malignant tumors in this age group, in adults, its incidence is estimated to be approximately 1% with only about 200 cases having been re­ported in world literature. [2]

The staging of AWT is done in the same way as in children according to the NWTSG. [1] It is currently recommended that only tumors that have biphasic or triphasic histology be diag­nosed as Wilms' tumor. [4] To do away with the uncertainty in diagnosis, Kilton et al in 1980, laid down criteria for the diagnosis of AWT. These are:

  1. It should be a primary renal neoplasm
  2. There should be presence of primitive blas­tematous spindle or round cell component
  3. Formation of abortive or embryonal tubularor glomeruloid structures is necessary
  4. No area of the tumor should show features
  5. suggestive of renal cell carcinoma
  6. Pictoral confirmation of histology is required
  7. Age should be >15 years. [5]


Our case conformed to all these criteria and had triphasic histology.

Pre-operative diagnosis of AWT is extremely difficult, because diagnostic imaging techniques cannot distinguish it from renal cell carcinoma. [6] Due to the rarity of the disease; definitive treat­ment plans are undefined. Still, investigators agree on the need for multimodality approach in the treatment of AWT, but differ on how aggressive it should be. While some advocate the adoption of current pediatric protocols based on tumor size and grade, others recommend using advanced disease regimens for all stages and grades. [7],[8]

In AWT, surgical treatment has the highest priority and even the discovery of metastatic di­sease should not prevent exploration or the attempted removal of the primary tumor. If the primary tumor is initially inoperable, then fol­lowing chemotherapy, a second look laparoto­my is worth a consideration. [2]

In conclusion, the possibility of Wilms' tumor should be borne in mind in an adult with flank pain, large tumor mass and fast tumor growth. We would also like to add that the combination of adequate surgical excision followed by che­motherapy (as per NWTSG-5 guidelines for pe­diatric population) offers the best chance of cure to these patients.

 
   References Top

1.Mitry E, Ciccolallo L, Coleman MP, Gatta G, Pritchard-Jones K. Incidence of and survival from Wilms' tumour in adults in Europe: data from the EUROCARE study. Eur J Cancer 2006;42(14):2363-8.  Back to cited text no. 1    
2.Grant Williams RA, Colbeck NF. Growing adult Wilms' tumour: Review of 14 patients. Br J Urol 1992;70:230-5.  Back to cited text no. 2    
3.Akmansu M, Yapici T, Tulay E. Adult Wilms' Tumour. A Report of Two Cases and their Treatment and Prognosis. Int Urol Nephrol 1998;30(5):529-33.  Back to cited text no. 3    
4.Rubin BP, Pins MR, Nielsen GP, et al. Isochrome 7q in adult Wilms' tumors: diagnostic and pathogenic implications. Am J Surg Pathol 2000;24(12):1663-9.  Back to cited text no. 4    
5.Kilton L, Mathews MJ, Cohen MH. Adult Wilms' tumour: a report of prolonged survival and review of literature. J Urol 1980;124:1-5.  Back to cited text no. 5    
6.Pascual Samaniego M, Calleja Escudero J, Alvarez Gago T, Gonzalo Rodriguez V, Muller Arteaga C, Fernandez del Busto E. Adult Wilms' tumor. Actas Urol Esp 2004;28(7):544-8.  Back to cited text no. 6    
7.Tawil A, Cox JN, Roth AD, Briner J, Droz JP, Remadi S. Wilms' tumour in the adult- report of a case and review of the literature. Pathol Res Pract 1999;195(2):105-11;discussion 113-4.  Back to cited text no. 7    
8.Kalapurakal JA, Nan B, Norkool P, et al. Treat­ment outcomes in adults with favourable histo­logic type Wilms tumour- an update from the National Wilms' Tumour Study Group. Int J Radiat Oncol Biol Phys 2004;60(5):1379-84.  Back to cited text no. 8    

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Correspondence Address:
Mriganka S Sharma
C-56/ Z-4 (3rd Floor), Dilshad Garden, Delhi-110095
India
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PMID: 19736484

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    Abstract
    Introduction
    Case History
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    References
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