Primary cardiac malignant peripheral nerve sheath tumor: a case report

Malignant peripheral nerve sheath tumors are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. Here we report a case of a primary cardiac malignant peripheral nerve sheath tumor without neurofibromatosis type I. A 53-year old woman presented having had cough, expectoration, and dyspnea for 20 days and was found to have a heart-involving tumor diagnosed as a malignant peripheral nerve sheath tumor, a rare cardiac sarcoma of 9 × 4.5 × 3 cm in size. The patient underwent a successful resection of the tumor but died 14 months postoperative. We report this case for its rarity and peculiar mode of morphologic and immunohistochemical presentation.