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Mass protruding out from pulmonary vein to left atrium
  1. A Shaheer Ahmed1,
  2. Sudheer Kumar Arava2,
  3. Sivasubramanian Ramakrishnan1
  1. 1 Cardiology, All India Institute of Medical Sciences, New Delhi, India
  2. 2 Pathology, All India Institute of Medical Sciences, New Delhi, India
  1. Correspondence to Professor Sivasubramanian Ramakrishnan, Cardiology, All India Institute of Medical Sciences, New Delhi 110029, India; ramaaiims{at}

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A 6-year-old boy, with a history of being operated for Wilms tumour, had presented to us with symptoms of insidious onset gradually increasing breathlessness on exertion. Echocardiography revealed 46×30 mm pedunculated homogenous mass attached to free wall of the left atrium prolapsing into left ventricle during diastole, severe pulmonary hypertension and right ventricular dysfunction (figure 1). Since he had a contrast-enhanced CT scan abdomen a few months back which did not show any recurrence, provisional diagnosis of atypical left atrial myxoma was made. However, his chest X-ray revealed homogenous opacity in the left upper and middle zones, arising from the mediastinum (figure 1). Hence, a contrast-enhanced CT scan of the chest was repeated at our centre which revealed mass arising from the mediastinum invading the left pulmonary vein and entering into the left atrium (figure 2). He underwent excision of left atrial mass and incisional biopsy of the mediastinal mass. Histopathology revealed malignant small round cell tumour immunopositive for Wilms tumour suppressor gene 1 (WT1) and desmin, while negative for myogenin suggestive of Wilms tumour (figure 3). He received chemotherapy and radiation therapy. Wilms tumour is the most common paediatric renal tumour in children. About 50% of relapses are seen in the mediastinum (including lung and pleura).1 Extension of Wilms tumour to left atrium without the involvement of right atrium is exceedingly rare.2 Left atrial myxoma typically presents with pedunculated mass with stalk attached to fossa ovalis. Presence of left atrial mass in other locations should undergo extensive evaluation before being taken up for surgery.

Figure 1

(A) Chest X-ray showing homogenous opacity in the left upper and middle zones. (B) Echocardiography showing mass in the LA.

Figure 2

(A) Contrast-enhanced CT scan of the chest showing mediastinal mass entering the left atrium (LA) through the pulmonary vein. (B) Mass in the LA. LV, left ventricle; RA, right atrium; RV, right ventricle.

Figure 3

(A) High magnification shows typical histopathology of malignant small round cell tumour. (B) Immunohistochemistry (IHC) shows strong nuclear immunopositivity for WT1 protein.



  • Contributors ASA was involved in patient care and preparing the manuscript. SKA did the histopathologic examination of the specimen. SR was involved in patient care and final approval of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.