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Published on: September 18, 2013
A Challenging Case of Pediatric Cardiac Leiomyosarcoma: Surgery, Recurrence, and Remission With Tyrosine Kinase
Ankur Handa1, Swaminathan Krishnaswamy1, Lamk Kadiyani1
1Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.
Insights
A rare pediatric cardiac tumor, leiomyosarcoma, was treated successfully with pazopanib. This targeted therapy achieved complete remission in a child unresponsive to chemotherapy, avoiding risky surgery.
Area of Science:
- Pediatric Oncology
- Cardiovascular Pathology
- Medical Oncology
Background:
- Primary cardiac leiomyosarcoma is an exceptionally rare pediatric malignancy.
- Pediatric cardiac tumors often present with obstructive symptoms, such as syncope.
Purpose of the Study:
- To report a case of pediatric primary cardiac leiomyosarcoma.
- To highlight the efficacy of pazopanib in managing refractory pediatric sarcoma.
Main Methods:
- A four-year-old girl with syncope underwent tumor resection.
- Histopathology confirmed leiomyosarcoma.
- Treatment with pazopanib was initiated due to rapid tumor regrowth post-chemotherapy.
Main Results:
- Pazopanib treatment led to complete remission within six months.
- High-risk reoperation was successfully avoided.
- The patient showed sustained remission, underscoring pazopanib's effectiveness.
Conclusions:
- Pazopanib is a viable therapeutic option for pediatric sarcomas resistant to conventional treatments.
- Early diagnosis and novel therapeutic strategies are crucial for improving outcomes in rare pediatric cancers.
Abstract:
Primary cardiac leiomyosarcoma is exceedingly rare in children. We describe a four-year-old girl presenting with recurrent syncope due to a mass in the right ventricular outflow tract causing severe obstruction. She underwent surgical resection of the tumor, presumed as benign on cardiac imaging. The histopathology, however, confirmed it to be leiomyosarcoma. Post-surgery there was rapid tumor regrowth despite first-line chemotherapy, which prompted treatment with pazopanib, a multitargeted tyrosine kinase inhibitor, which led to complete remission within six months, obviating the need for a high-risk reoperation. This case highlights the utility of pazopanib in pediatric sarcomas unresponsive to conventional therapy.
