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Year : 2012  |  Volume : 55  |  Issue : 3  |  Page : 381-383
Wilms' tumor with right heart extension: Report of a post-chemotherapeutic fatality

Department of Pathology (Cardiovascular & Thoracic Division), Seth G.S. Medical College & KEM Hospital, Mumbai, India

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Date of Web Publication29-Sep-2012


Wilms' tumor (WT) has a strong propensity to invade the vasculature in the form of tumor-thrombus, into the renal veins, and inferior vena cava and even into the right atrium. This cavo-atrial propagation does not alter the prognosis and pre-operative chemotherapy produces shrinkage to the extent of even disappearance of caval or atrial extensions. We present a case of sudden death due to hemorrhagic expansion of the intra-atrial component of WT, immediately after commencement of chemotherapy, an uncommon incident.

Keywords: Chemotherapy, children, heart, intracardiac metastases, right atrium, wilm′s tumor

How to cite this article:
Vaideeswar P, Chaudhari JP. Wilms' tumor with right heart extension: Report of a post-chemotherapeutic fatality. Indian J Pathol Microbiol 2012;55:381-3

How to cite this URL:
Vaideeswar P, Chaudhari JP. Wilms' tumor with right heart extension: Report of a post-chemotherapeutic fatality. Indian J Pathol Microbiol [serial online] 2012 [cited 2021 Nov 30];55:381-3. Available from: https://www.ijpmonline.org/text.asp?2012/55/3/381/101752

   Introduction Top

Wilms' tumor (WT) is the most common malignant renal tumor among children and young adults. Appropriate chemotherapy has changed the natural history of WT as more than 85% of the patients with localized disease and over 70% with metastatic disease can be completely cured. [1] WT has a strong propensity to invade the vasculature in the form of tumor-thrombus, into the renal veins and inferior vena cava (infrahepatic or type I, retrohepatic or type II, and suprahepatic or type III extensions), a feature noted in 4-10% of patients, and occasionally, into the right atrium (type IV extension), seen in about 1% of the cases. [2] This cavo-atrial propagation does not alter the prognosis when compared to the kidney-restricted stage/histology-matched tumors though a surgical approach becomes more complicated. [1] Currently, pre-operative chemotherapy is advocated as it facilitates the surgical approach by producing tumor shrinkage to the extent of even disappearance of caval or atrial extensions. [1],[2] We present a case of sudden death due to hemorrhagic expansion of the intra-atrial component of WT, immediately after commencement of chemotherapy, a phenomenon not described previously.

   Case Report Top

A 6-year-old girl presented with a gradually increasing left-sided abdominal lump for a month with associated pain, vomiting, and weight loss. There was no history of hematuria, pyuria, or retention of urine. Clinical examination revealed fullness in left hypochondrium and lumbar region with a palpable large (12 cm x 10 cm), firm-to-hard, smooth-surfaced, non-tender immobile lump. Routine hematological and biochemical investigations were normal. Ultrasonography and contrast-enhanced computed tomography revealed

12 x 11 x 8 cm, well-defined mass with mixed echogenicity replacing the left kidney and extending into inferior vena cava (diameter 3 cm) and right atrium. After an oncologic consultation, a pre-operative chemotherapy was started using vincristine (1.5 mg/m 2 ), adriamycin (60 mg/m 2 ), and actinomycin (45 μg/kg). But, within 6 h of the therapy, the patient suddenly collapsed and could not be revived.

A complete autopsy was performed. The left kidney was markedly enlarged in size (20 x 12 x 5 cm, 1.1 kg) and was distorted due to large, lobulated grey- white tumor occupying almost the entire renal parenchyma; only a small part of the lower pole of the kidney was identified [Figure 1]a.
Figure 1: (a) Large tumor T with foci of necroses and hemorrhage, replacing most of the renal parenchyma. Only part of the lower pole of the left kidney K is spared. (b) Arrows point to marked dilatation of the inferior vena caval opening produced by hemorrhagic tumor (RA right atrium, RV right ventricle, LA left atrium, LV left ventricle)

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Chemotherapeutic effects were seen in the renal mass in the form of extensive necroses and hemorrhage amidst a classic triphasic pattern of WT; a fragment of tumor was also found to occlude the left ureter. The tumor was seen to extend, expand, and occlude the left renal vein and the inferior vena cava. The cross section of the tumor at the inferior vena caval orifice was 3.4 x 3.2 cm [Figure 1]b. The right atrial cavity [Figure 2] was markedly dilated and was occupied by soft, hemorrhagic, and dumbbell-shaped tumor (6.2 x 3.9 x 3.2 cm); the constricted portion of the dumbbell was at the level of the tricuspid orifice. The anterior papillary muscle was completely flattened. The remaining portion of this tumor projected into the right ventricle. Sudden death of our patient was therefore due to complete blockage of the systemic venous return. The left-sided chambers were normal.
Figure 2: (a) Dumbbell-shaped hemorrhagic metastatic tumor T, occupying the entire right atrium and extending into the inlet of right ventricle, sealing off the tricuspid valvular orifi ce. (b) The tumor
has been removed to show dilated right atrial cavity RAC, dilated tricuspid annulus TV, and flattened anterior papillary muscle, arrows (SVC superior vena cava, IVS interventricualr septum, LV left ventricle, AO aorta). (c) Scan of the section of the right atrial tumor stained by hematoxylin and eosin). (d) Blastemal cells in a hemorrhagic background (Hematoxylin and eosin, × 400)

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   Discussion Top

We have presented a patient who died suddenly within few hours of starting chemotherapy as a result of hemorrhagic expansion of right atrial extension of renal WT, thereby blocking the systemic venous return. Extension of WT into the right atium is rare and observed in 1% of patients, who often have large tumors. [2] It is frequently asymptomatic or is accompanied by subtle features like history of abdominal pain (as was present in the reported case) or occasionally, these patients may present with features of inferior vena caval obstruction or congestive cardiac failure with catastrophic outcomes. [3],[4] Since intra-operative tumor rupture, major hemorrhages, and tumor embolism are some of the known disadvantages of the primary surgical management, pre-operative chemotherapy (for few weeks) is employed to decrease the tumor size, intra-operative tumor rupture, tumor cell spillage, and/or vascularity. [5] The shrinkage occurs through fibrosis and/or necrosis and is especially recommended in patients with intravascular extension above the level of the hepatic veins (type III) and therefore holds true for right atrial extensions (type IV). Persistent cavoatrial tumor and even tumor growth/expansion, despite chemotherapy, is seen in about 30 % of patients and is explained on the basis of resistance of the tumor components; it is best removed by atriotomy under cardiopulmonary bypass. [6] But, herein, we had this patient who succumbed within a few hours of chemotherapy. Severe post-chemotherapeutic changes in the form of necroses, edema, and extensive hemorrhage were seen in the renal and atrial tumor components with even splintering of the renal mass into the pelvis and ureter. Such a phenomenon as reported in this index case is unusual and we wonder whether the tumor was exquisitely chemosensitive. Since there is paucity of data, this loss warrants in-depth analysis of probable factors along with up-gradation of the protocols if required; preferably, the treatment should be individualized. Moreover, in our country, the incidence of advanced and metastatic WT is considerably higher than in the western literature as the patients usually present in the late stages with large tumors and frequently with metastases. This calls for a multidisciplinary approach for laying specific guidelines for investigations and multimodality management and for monitoring during pre-operative chemotherapy.

   Conclusion Top

Cavo-atrial extension of WT indirectly affects the final outcome. Preoperative chemotherapy in children with this feature is the method of choice till date. However, sudden deaths with preoperative chemotherapy warrant fresh protocols to be laid separately for patients with intra-cardiac extension.

   References Top

1.Kalapurakal JA, Dome JS, Perlman EJ, Malogolowkin M, Haase GM, Grundy P, et al. Management of Wilms' tumour: Current practice and future goals. Lancet Oncol 2004;5:37-46.  Back to cited text no. 1
2.Cristofani LM, Duarte RJ, Almeida MT, Odone Filho V,, Maksoud JG, Srougi M. Intracaval and intracardiac extension of Wilms' tumor. The influence of preoperative chemotherapy on surgical morbidity. Int Braz J Urol 2007;33:683-9.  Back to cited text no. 2
3.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. 3
4.Namboodiri N, Krishnamoorthy KM, Tharakan JA. Intra-atrial extension of Wilms' tumor. J Am Soc Echocardiogr 2008;21:91.e3-4.  Back to cited text no. 4
5.Lall A, Pritchard-Jones K, Walker J, Hutton C, Stevens S, Azmy A, et al. Wilms' tumor with intracaval thrombus in the UK Children's Cancer Study Group UKW3 trial. J Pediatr Surg 2006;41:382-7.  Back to cited text no. 5
6.Murthi GV, Kocyildirim E, Sellathury S, Cuckow PM, Wilcox DT, Michalski A, et al. Wilms' tumour with persistent intravascular extension: A review of the surgical aspects of management. J Pediatr Urol 2006;2:439-45.  Back to cited text no. 6

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular & Thoracic Division), Seth G. S. Medical College and KEM Hospital, Parel, Mumbai 400 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.101752

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