Malignant peripheral nerve sheath tumor (MPNST) arising from angiosarcoma: a rare case report.

PURPOSE: An extremely rare case of divergent differentiation of a cutaneous angiosarcoma to a malignant peripheral nerve sheath tumor (MPNST) is presented.

METHODS

A 62-year-old male patient presented with an angiosarcoma of the left buccal region, which was excised with wide surgical margins. Four months after the operation, the tumor recurred locally as a MPNST. The recurrent tumor was treated with surgical excision followed by postoperative radiotherapy. Four years later, the patient presented with solitary lung metastasis. The patient underwent upper right lobectomy.

RESULTS

The patient is alive and disease-free 2 years after the lobectomy and 6 years after initial presentation.

CONCLUSIONS

Divergent differentiation of an angiosarcoma to a neurosarcoma supports the theory that cells of the neural crest capable of differentiating both to neuroectoderm and to mesenchyme give origin to these tumors.