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Primary cardiac malignant peripheral nerve sheath tumor: a case report
Xiaoxia Li1, Chaoliang Liu2, Renya Zhang1
1Department of Pathology, Affiliated Hospital of Jining Medical University, Shandong, 272029, China.
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas. This report details a rare case of primary cardiac MPNST in an adult without neurofibromatosis type I.
Area of Science:
- Oncology
- Cardiovascular Pathology
- Sarcoma Research
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas typically affecting children and adolescents.
- These tumors have a high propensity for local recurrence, posing significant treatment challenges.
Observation:
- A 53-year-old woman presented with respiratory symptoms including cough, expectoration, and dyspnea.
- Imaging revealed a large (9 × 4.5 × 3 cm) tumor involving the heart.
Findings:
- The cardiac tumor was diagnosed as a primary malignant peripheral nerve sheath tumor.
- The patient underwent successful surgical resection, but unfortunately died 14 months postoperatively.
Implications:
- This case highlights the extreme rarity of primary cardiac MPNSTs, particularly in adults without neurofibromatosis type I.
- The unique morphologic and immunohistochemical presentation warrants further investigation into cardiac sarcoma subtypes.
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