Schwannoma with angiosarcoma - Report of a case and comparison with other types of nerve tumors with angiosarcoma

BACKGROUND. Schwannoma with angiosarcomatous change is a rare tumor, the cl inical characteristics of which have not been analyzed. METHODS. A patient with schwannoma with angiosarcoma arising in the mid-rec k and clinically mimicking a carotid body paraganglioma is described with a literature review of all previously reported cases and a comparison of the ir clinical features with those of schwannoma with conventional malignant t ransformation and cases of neurofibroma and malignant peripheral nerve shea th tumor (MPNST) with angiosarcoma. RESULTS. There are four reported cases, including the present case. Schwann oma with angiosarcoma affects older adults, mainly men. Three tumors arose from the vagus nerve in the neck Three of the four angiosarcomas were epith elioid in type. Treatment in all cases was surgical resection followed by r adiation and chemotherapy in one case and by radiation alone in another. On e patient died with residual local angiosarcoma 5 months after the diagnosi s. The remaining three patients were alive and disease free at 27 months, 4 3 months, and 90 months, with distant metastasis (after 15 months) reported only in the patient described in this case report. CONCLUSIONS. Schwannoma with angiosarcoma should be included in the differe ntial diagnosis of presumed carotid body paragangliomas. Like angiosarcoma alone and schwannoma with conventional malignant transformation, but unlike cases of neurofibroma and MPNST with angiosarcoma, the patients are older adults, and there is a male prevalence. Schwannoma with angiosarcoma is cap able of local spread with a fatal outcome and of distant metastasis, but fo llow-up strongly suggests that these patients have a better prognosis than patients with neurofibroma or MPNST with angiosarcoma. Recommended treatmen t is attempted complete surgical resection followed by radiation therapy an d chemotherapy, if it can be tolerated by the patient. Cancer 2000;89:1577- 85. (C) 2000 American Cancer Society..