Unusual Malignant Tumors of the Head and Neck

The purpose of this article is to present a brief review of a variety of malignant tumors of the head and neck region about which little is known with respect to clinical behavior and therapeutic management. Of the many publications concerned with these lesions, few are oriented toward radiation therapy. Included in this discussion are: (a) esthesioneuroepithelioma, (b) solitary plasmacytoma of bone and soft tissue, (c) Ewing's sarcoma, and (d) rhabdomyosarcoma. In addition to these entities, unclassified carcinomas and malignant tumors will also be discussed. Clinical Behavior Esthesioneuroepithelioma: Esthesioneuroepithelioma was first described in 1924 (2) as a tumor of the olfactory nerve histologically resembling retinoblastoma. Several terms have been used to describe this tumor, and the theories of its origin are multiple (8, 10, 17). The most popular hypothesis is that it arises from the neuroepithelial cells of the olfactory mucosa. Due to the anatomical site, the most common clinical manifestations are nasal obstruction, epistaxis, and localized pain. Examination usually reveals a gray polypoid lesion occluding the nasal cavity and projecting into the nasopharynx. A mass may also present adjacent to the inner canthus on the involved side with resulting lateral displacement of the eye and ptosis of the lid. Esthesioneuroepithelioma usually grows slowly, invades bone, and extends into the paranasal sinuses. Although metastatic spread was previously denied, recent investigators have confirmed both lymphatic and blood-borne metastases (1, 10, 17). Esthesioneuroepithelioma is radioresponsive, as might be predicted by its close histologic resemblance to neuroblastoma, and radiotherapy is considered the treatment of choice. Surgery is held in reserve for radiotherapeutic failures. Due to the low incidence of cervical metastases, only the primary lesion is included in the irradiated volume. We have seen 5 patients with esthesioneuroepithelioma, whose cases are summarized in Table I. These neoplasms must be treated aggressively, i.e., 6,000 rads tumor dose in six weeks, in spite of their allegedly high degree of radioresponsiveness. Definite regrowth of disease was confirmed histologically in one of our cases two and one-half months after therapy; the antrum and ethmoid sinus were resected, and the patient is now free of disease eleven months postoperatively. Plasmacytoma: Extramedullary plasmacytoma of the head and neck region was first described in 1905 (12). Lesions arising within the head and neck region should be thought of as separate entities because of their behavior. Approximately 90 per cent of the solitary tumors of this region arise in the soft tissue, whereas solitary bony lesions are rare.