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Primary and metastatic primitive neuroectodermal tumor of the heart: A systematic review

Objective Primary and metastatic primitive neuroectodermal tumors of the pericardium are uncommon. Two cases are presented and discussed. Methods The cases of a 17- and 38-year-old male patients with neuroectodermal tumors of the pericardium are presented. In addition, a systematic review was performed according to the Preferred Reporting Items and checklist for Systematic reviews and Meta-Analyses (PRISMA). All selected articles’ quality assessment was done using the Joanna Briggs Institute (JBI) Critical Appraisal Checklist for Case Reports. Results The systematic review yielded 29 patients with primary or metastatic PNET. Two patients underwent cardiac transplantation. It seems that unlike considering total resection in other organs, pericardiectomy in PNET patients with pericardial origin may lead to further invasion of the lesions, and it is not recommended. The average disease-free follow-up was 10 months. The combination of neoadjuvant and adjuvant chemotherapy along with surgery and radiotherapy offered the best disease-free outcomes. Conclusion Neuroectodermal heart tumors are rare, aggressive tumors requiring chemotherapy and radiotherapy in association with tumor resection surgery to have the best disease-free duration.