Malignant Peripheral Nerve Sheath Tumor of the Larynx / 대한이비인후과학회지

ABSTRACT

Malignant peripheral nerve sheath tumors arising de novo or from preexisting benign schwannoma or neurofibromatosis are highly aggressive tumors. Malignant peripheral nerve sheath tumor of the larynx is extremely rare neoplasm, for which accurate diagnosis is difficult and therapy is frequently delayed. Although wide surgical excision is the mainstay of treatment, radiation therapy plays a role in the control and treatment of locally recurrent disease and is useful for unresectable lesions. We present a case of malignant peripheral nerve sheath tumor of the larynx without definite evidences of von Recklinghausen's disease or preexisting benign peripheral nerve sheath tumor. This case is the third report of primary malignant peripheral nerve sheath tumor of the larynx according to the review of the literature.