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CASE REPORT
Year : 2018  |  Volume : 45  |  Issue : 2  |  Page : 97-98

Synchronous bilateral wilms' tumor in a 2-year-old male child


1 Department of Urology, KLE Academy of Higher Education and Research (Deemed-to-be-University); Department of Urology, KLES Kidney Foundation, KLES Dr. Prabhakar Kore Hospital and MRC, Belagavi, Karnataka, India
2 Department of Urology, KLES Kidney Foundation, KLES Dr. Prabhakar Kore Hospital and MRC, Belagavi, Karnataka, India
3 Department of Biotechnology and Microbiology, Karnatak University, Dharwad, Karnataka, India

Date of Web Publication10-Dec-2018

Correspondence Address:
R B Nerli
KLES Kidney Foundation, KLES Dr. Prabhakar Kore Hospital and MRC, Nehru Nagar, Belagavi - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.JSS_4_18

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  Abstract 


Wilms' tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5%–7% of WT patients present with bilateral disease, either synchronously or metachronously. Bilateral WT usually occurs in younger children and more often in girls. Management of a child with bilateral WT is very challenging. We report a case of bilateral WT in a 2-year-old male child. The child has undergone preoperative chemotherapy followed by nephron-sparing surgery.

Keywords: Bilateral, stage V, Wilms' tumor


How to cite this article:
Nerli R B, Ghagane SC, Dixit NS, Hiremath MB. Synchronous bilateral wilms' tumor in a 2-year-old male child. J Sci Soc 2018;45:97-8

How to cite this URL:
Nerli R B, Ghagane SC, Dixit NS, Hiremath MB. Synchronous bilateral wilms' tumor in a 2-year-old male child. J Sci Soc [serial online] 2018 [cited 2019 Mar 23];45:97-8. Available from: http://www.jscisociety.com/text.asp?2018/45/2/97/247156




  Introduction Top


Wilms' tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5%–7% of WT patients present with bilateral disease, either synchronously or metachronously.[1] Bilateral WT usually occurs in younger children and more often in girls.[1] Bilateral WT is classified under Stage V whenever bilateral renal involvement occurs at the initial diagnosis. Management of a child with bilateral WT is very challenging, as preservation of the maximum amount of renal parenchyma is needed to prevent renal failure. However complete resection is required to optimize the chances for cure of the malignancy. In contrast to unilateral WT, there has not been uniform agreement about the therapeutic strategy in the management of bilateral WT.[1],[2],[3] We report a case of bilateral WT in a 2-year-old male child.


  Case Report Top


A 2-year-old male child was brought to the pediatric urological services of the hospital with complaints of distension of the abdomen. The child had normal milestones and was vaccinated as per the schedule. The child was anemic (Hb –9.2 g%) and weighed 10.5 kg. Serum creatinine was 0.24 mg%. Ultrasonographic examination revealed bilateral renal masses. Computed tomography (CT) showed a heterogeneously enhancing lobulated mass 2.8 cm × 3.1 cm × 4.2 cm in the upper pole of the right kidney and a hypoenhancing solid mass 6 cm × 6 cm × 7 cm upper and mid-polar mass in the left kidney. No other lesion was identified. Chest X-ray was normal [Figure 1].
Figure 1: (a and b) Computed tomography showing a heterogeneously enhancing lobulated mass 2.8 cm × 3.1 cm × 4.2 cm in the upper pole of the right kidney and a hypoenhancing solid mass 6 cm × 6 cm × 7 cm upper and mid-polar mass in the left kidney

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A diagnosis of bilateral WT was made, and the child was put on preoperative chemotherapy (SIOP-2001 guidelines) consisting of injection vincristine 0.75 mg on days 1, 8, 15, 22, 29, and 36 and injection actinomycin D 475 mcg on days 1, 15, and 29. The child was monitored with serial blood tests consisting of total white blood cell (WBC) counts, differential WBC count, liver function tests, hemoglobin, and electrolytes. There was a massive reduction in the size of the renal masses bilaterally as seen on CT. The child underwent bilateral nephron-sparing surgery. The child had an uneventful postoperative period and underwent adjuvant chemotherapy as per SIOP protocols. The histopathology examination revealed WT (favorable histology). Postoperative chemotherapy involved three drugs, namely vincristine 0.75 mg, injection actinomycin D 475 μg, and injection doxorubicin (adriamycin 23 mg) in a schedule as follows.

  • Week 1 – injection vincristine
  • Week 2 – injection vincristine + injection actinomycin + injection doxorubicin
  • Week 3 – injection vincristine
  • Week 4 – Off.


The child received six such cycles. The child withstood chemotherapy well and is on close follow-up. Repeat CT scan done 6 months following the end of chemotherapy revealed no evidence of disease. The child is taking oral feeds properly and has been gaining weight.


  Discussion Top


The management of bilateral WT has progressively evolved from radical nephrectomy to kidney-preserving resection after preoperative chemotherapy.[4],[5] Preoperative chemotherapy often results in significant reduction in tumor size, thereby facilitating subsequent renal salvage surgery. According to the SIOP-93 guidelines, children with bilateral WT are treated with preoperative chemotherapy consisting of vincristine (1.5 mg/m2/day, day 1) and dactinomycin (15 μg/kg/day, days 1, 2, and 3) every 2 weeks, with extra dose of vincristine on day 8. In children in whom sufficient regression has not occurred then, other drugs can be used. Once sufficient regression is observed, bilateral or at least unilateral nephron-sparing surgery is performed when technically possible usually after 6 weeks.

Surgery is performed in one step (bilateral surgery at the same time) or two steps to improve patient tolerance, or by a few weeks (>1 month) to allow for the administration of chemotherapy matched to the type and stage of the resected tumor. Postsurgical management is chemotherapy, adjusted to fit the highest histological grade and local stage of the tumor. In patients with stage III disease, complementary radiotherapy is delivered to a maximum dose of 12 Gy.[6] The duration and intensity of chemotherapy depends very much on the other therapeutic modalities used. Three drug conventional chemotherapy regimens are today commonly used in patients with bilateral WT.[7],[8] In conclusion, the management of bilateral WT is challenging despite the current multimodal treatments that are available and warrant further investigations.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sulkowski J, Kolon T, Mattei P. Nephron-sparing partial nephrectomy for bilateral Wilms' tumor. J Pediatr Surg 2012;47:1234-8.  Back to cited text no. 1
    
2.
Hamilton TE, Green DM, Perlman EJ, Argani P, Grundy P, Ritchey ML, et al. Bilateral Wilms' tumor with anaplasia: Lessons from the National Wilms' Tumor Study. J Pediatr Surg 2006;41:1641-4.  Back to cited text no. 2
    
3.
Millar AJ, Davidson A, Rode H, Numanoglu A, Hartley PS, Daubenton JD, et al. Bilateral Wilms' tumors: A single-center experience with 19 cases. J Pediatr Surg 2005;40:1289-94.  Back to cited text no. 3
    
4.
Nerli RB, Pujar VC, Hiremath MB, Jali SM, Joshi SS, Hiremath SC, et al. Nephron sparing surgery for unilateral non-syndromic Wilms tumor. Indian J Surg Oncol 2014;5:11-6.  Back to cited text no. 4
    
5.
Nerli R, Patil R, Ghagane S, Hiremath MB. Long term follow-up in children undergoing nephron sparing surgery for non-syndromic unilateral Wilms tumor. Clin Oncol 2017;2:1259.  Back to cited text no. 5
    
6.
Sudour H, Audry G, Schleimacher G, Patte C, Dussart S, Bergeron C, et al. Bilateral Wilms tumors (WT) treated with the SIOP 93 protocol in France: Epidemiological survey and patient outcome. Pediatr Blood Cancer 2012;59:57-61.  Back to cited text no. 6
    
7.
Saarinen-Pihkala UM, Wikström S, Vettenranta K. Maximal preservation of renal function in patients with bilateral Wilms' tumor: Therapeutic strategy of late kidney-sparing surgery and replacement of radiotherapy by high-dose melphalan and stem cell rescue. Bone Marrow Transplant 1998;22:53-9.  Back to cited text no. 7
    
8.
Nerli RB, Ghagane SC, Ram P, Deole S, Pentayala S, Bhadrannavar S. Nephron sparing surgery for synchronous bilateral Wilms – Operative steps. Indian J Surg Oncol 2018;1-4. Available from: https://doi.org/10.1007/s13193-018-0793-z.  Back to cited text no. 8
    


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