P0878 - Cardiac Complications in an Adolescent with Stage IV Renal Cell Carcinoma

Introduction: Renal Cell Carcinoma (RCC) is an uncommon malignancy in children and adolescents, accounting for less than 5% of pediatric renal neoplasms, with an estimated incidence of 1/1000. In contrast to the adult presentation, pediatric RCC presents more symptomatically with locally advanced disease. Up to 30 % of pediatric patients with RCC present with metastatic disease. The most common locations for metastasis include the lungs, bone, liver and brain. To date, there are no published reports of cardiac complications of pediatric RCC.

Case Description: A 17 year-old, previously healthy, female presented with a three-week history of productive cough, orthopnea, paroxysmal nocturnal dyspnea and bilateral ankle pitting edema. She was transferred from a peripheral hospital for further work up of a right kidney mass found incidentally on physical examination. In addition, she experienced pleuritic chest pain and constitutional symptoms including chills, anorexia and weight loss. Abdominal ultrasound confirmed a 13.7 cm x 12.2 cm x 8.9 cm heterogeneous hypoechoic lesion in the inferior pole of the right kidney. A PA Chest X-ray revealed bilateral pleural effusions and a mediastinal mass. On initial examination she was tachycardic with a heart rate of 126 beats per minute and found to have pulsus paradoxus. An urgent echocardiogram was completed and confirmed a large pericardial effusion measuring 400 mL. An urgent bedside pericardiocentesis was performed in the pediatric intensive care unit which drained approximately 2 litres in 24 hours. The pericardial fluid specimen was found to have rare atypical cells, mesothelial cells and reactive changes. The patient was then treated for presumed pericarditis secondary to her malignancy. Her malignancy was confirmed with right renal mass node resection and biopsy demonstrating histological findings of translocation carcinoma, grade 4 with nuclei bizarre and multiloculated with immunohistochemistry positive for cytokeratin AE1/AE3 and vimetin with focally positive CD10-cells in keeping with RCC.

Discussion: It is well established that Hodgkin disease, lymphomas and leukemias are conditions that can result in pericardial effusions, yet to date, no studies have established this link with pediatric renal cell carcinoma. In the adult literature, there is very limited information on pericardial effusions secondary to renal cell carcinoma, and most case studies describe cardiac metastasis as the etiology of the effusion. For our patient, there was no suggestion of an intracardiac lesion on echocardiogram nor did the pericardial fluid demonstrate malignant cells. 

Conclusion: Pediatric renal cell carcinoma, an uncommon neoplasm in children and adolescents may have more extensive systemic involvement than previously thought. Pediatric renal cell carcinoma should be considered on the differential for a pericardial effusion related to the neoplastic disease and non-invasive cardiac investigations should be considered where indicated.