Key result
Resection and chemotherapy fail to prevent recurrence and brain metastasis in infant intracardiac myoepithelial carcinoma.
Why the study?
Intracardiac myoepithelial carcinoma is extremely rare in pediatric patients and presents diagnostic and management challenges.
Case Report (n=1)
This case highlights the extreme rarity of intracardiac myoepithelial carcinoma in pediatric patients and the significant challenges in achieving long-term remission with multimodal management.
Despite aggressive multimodal therapy, recurrence and brain metastasis developed; this rare case leaves open optimal management for pediatric intracardiac myoepithelial carcinoma.
Myoepithelial carcinomas (MC) represent aggressive tumors that occur in a myriad of ages and anatomic locations. The rarity and histologic similarity with other tumors make them difficult to diagnosis. We report an extremely rare case of a right ventricular outflow tract mass identified to be an intracardiac MC in a 4-month-old male infant. Pathology revealed an EWS-KLF15 translocation. Treatment included gross total resection and intensive chemotherapy. Recurrent cardiac mass with brain metastasis was seen 16 months after primary diagnosis. We describe the rarity of intracardiac MC in pediatric patients and the challenges encountered in the multimodal management of this patient.
No takes yet. Share an insight, caveat, or question.
Patel et al. (2018) conducted a case report in Intracardiac myoepithelial carcinoma (n=1). Gross total resection and intensive chemotherapy was evaluated on Clinical outcome (recurrence and metastasis). Gross total resection and intensive chemotherapy in a 4-month-old infant with intracardiac myoepithelial carcinoma resulted in a recurrent cardiac mass with brain metastasis 16 months later.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: