Malignant peripheral nerve sheath tumor with local recurrence and cardiac invasion: case report

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon disease of soft tissue sarcoma which affects young and middle-aged adults. Intravascular and cardiac involvements were anecdotally reported. Due to the locally aggressive behavior of tumor, treatment requires multimodality such as surgical resection followed with radiotherapy to improve local control. We report a case of a 20-year-old female who presented with a mass in the left supraclavicular neck. MRI showed nerve sheath tumor originating from the C5/6 nerve root. The tumor was partially removed. The pathology report revealed benign nerve sheath tumor. Eight months later, the residual tumor increased in size and was partially removed again. This time the tumor was reported as MPNST. The patient received radiotherapy after the operation. One year later, she had superior vena cava syndrome. MRI showed increased size of the tumor at 10 × 8 × 9 cm with intravascular growth in the left brachiocephalic vein, superior vena cava, and right atrium. Therefore, we performed surgery with a multidisciplinary team (neurosurgeon, ENT, cardiovascular surgeon). The tumor was removed by left supraclavicular approach and medial sternotomy with cardiopulmonary bypass to remove tumor at the right SVC and right atrium. The tumor was removed sub-totally 80% remaining part of tumor that attaches to the subclavian and innominate vein. Our report showed the characteristic of MPNST with a high rate of relapse and rapid disease progression. The intravascular and cardiac invasion was uncommon. Multimodality treatment was needed to manage the extensive tumor invasion.