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LETTER TO EDITOR  
Year : 2013  |  Volume : 56  |  Issue : 1  |  Page : 68-69
Giant untreated Wilms tumor with intracardiac extension: A rare case


Department of Pathology, Seth G. S. Medical College, Mumbai, Maharashtra, India

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Date of Web Publication6-Aug-2013
 

How to cite this article:
Yadav SC, Sathe PA, Ghodke RK, Fernandes GC. Giant untreated Wilms tumor with intracardiac extension: A rare case. Indian J Pathol Microbiol 2013;56:68-9

How to cite this URL:
Yadav SC, Sathe PA, Ghodke RK, Fernandes GC. Giant untreated Wilms tumor with intracardiac extension: A rare case. Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Feb 28];56:68-9. Available from: http://www.ijpmonline.org/text.asp?2013/56/1/68/116159


Sir,

We have read the article published in your journal by Vaideeswar et al. [1] We have also had a case of Wilms tumor with intracardiac extension, who unfortunately, could not be treated.

A two-year-old male child, born from a non-consanguineous marriage, presented to our hospital with complaints of high-grade fever, abdominal distension, pedal edema and significant weight loss of 20 days duration. On general examination, the child was febrile, malnourished, pale and was found to have blood pressure of 140/90 mm Hg. His immunization and development history was normal. Systemic examination showed diffuse abdominal distension. Investigations revealed: Hemoglobin -7.0 gm/dl, Total leucocyte count -7900/cmm and platelet count -1.0 lac/cmm. Renal biochemistry was normal. Computerized tomography of the abdomen confirmed a large heterogeneous mass measuring 35 × 20 × 11 cm arising from the lower pole of left kidney [Figure 1] with extension into left renal vein, inferior vena cava and the right atrium. The liver showed multiple metastases.

The child expired after a ward stay of 16 hours and an autopsy was requested. At autopsy, the body was severely pale and malnourished. External examination showed marked distension of the abdomen. On opening the abdominal cavity, a large, lobulated, grey-white mass was seen measuring 34 × 20 × 10 cm and weighing 3.5 kg occupying all the quadrants of the abdomen [Figure 1] with areas of necrosis and hemorrhage [Figure 2]. The liver showed two grey-white metastatic nodules [Figure 2]. The left renal vein and the inferior vena cava showed a serpiginous grey-white tumor thrombus [Figure 3]. The right atrium had a protruding nodule (two cm in diameter) occluding the inferior vena caval opening [Figure 3]. The right kidney and other organs were unremarkable. Microscopy confirmed a triphasic Wilms tumor comprising diffuse blastemal, mesenchymal and epithelial (tubular) components without anaplasia thus suggesting a favorable histology [Figure 2].
Figure 1: (a) Computerized tomography of the abdomen showing the large heterogeneous tumor (arrow). (b) Tumor occupying the whole of abdominal cavity with the intesti nal loop stretched over it

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Figure 2: (a) Cut surface of the tumor showing large areas of necrosis. (b) Multiple liver metastasis; one of them shows the tumor on cut surface. (c) Photomicrograph showing the classic triphasic histology of Wilms tumor (arrows pointing to the tubules). (H and E, ×40). (d) Photomicrograph showing a epithelial
component (tubule), blastemal component and mesenchymal component (H and E, ×400)


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Figure 3: (a) Opened inferior vena cava showing the tumor (ti p of forceps); the other kidney seen in the photograph is normal. (b) Right atrial tumor almost occluding the inferior vena caval opening (shown by arrow)

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Extension of Wilms tumor into inferior vena cava is a well-known phenomenon. [2] Right atrial involvement is reported only in 4% cases. [3]

Current imaging modalities help to identify most patients even with intravascular extension at an early stage. [4],[5] Hence, large tumors are rare nowadays. In patients with large tumors, the current practice of preoperative chemotherapy helps to reduce the tumor load and facilitates surgery. Wilms tumor seen in our case is one of the largest tumors reported in literature. [1],[2],[3],[4],[5] The tumor stage, presence of diffuse anaplasia and patient age are the prognostic determinants that have an impact on the oncological outcome. [4] Our case had favorable histological features. When detected at an early stage and with favorable histology, the prognosis is excellent with survival rates between 90% and 100%. Our patient presented to us at Stage IV of the disease due to lack of timely medical aid.

The unusual features of this case are the large tumor size, right atrial tumor extension and mortality due to higher stage despite favorable histology, all of which have been rarely reported together in literature. This case highlights the importance of early diagnosis in this most treatable solid pediatric malignancy.

 
   References Top

1.Vaideeswar P, Chaudhari JP. Wilms' tumor with right heart extension: Report of a post-chemotherapeutic fatality. Indian J Pathol Microbiol 2012;55:381-3.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Kim S, Chung DH. Pediatric solid malignancies: Neuroblastoma and Wilms tumor. Surg Clin North Am 2006;86:469-87.  Back to cited text no. 2
    
3.Ritchey ML, Kelalis PP, Breslow N, Offord KP, Shochat SJ, D'Angio GJ. Intracaval and atrial involvement with nephroblastoma: Review of National Wilms tumor study-3. J Urol 1988;140:1113-8.  Back to cited text no. 3
    
4.Tongaonkar HB, Qureshi SS, Kurkure PA, Muckaden MA, Arora B, Yuvaraja TB. Wilms' tumor: An update. Indian J Urol 2007;23:458-66.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.Shamberger RC, Ritchey ML, Haase GM, Bergemann TL, Loechelt-Yoshioka T, Breslow NE, et al. Intravascular extension of Wilms tumor. Ann Surg 2001;234:116-21.  Back to cited text no. 5
    

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Correspondence Address:
Subhash C Yadav
Department of Pathology,Seth G. S. Medical College,Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.116159

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