Lymphoepithelioma in childhood

Nine black children with lymphoepithelioma, a rare malignancy of childhood, are the subject of this report. Unique clinical features included tender cervical lymphadenopathy with torticollis, trismus, epistaxis, and change in voice quality. A nasopharyngeal mass was demonstrable in seven children on careful examination, but none was resectable. Treatment with radiation alone or radiation plus cyclophosphamide resulted in complete tumor regression in eight of the nine children. Local recurrence or distant metastases occurred in four within 10 months, two of whom responded to additional radiation plus cyclophosphamide or adriamycin. At present, four children are alive without disease for periods of seven to 78 months, two are alive for seven to 53 months and are in remission from recurrent disease, and three have died with distant metastases. Freedom from disease for one year was associated with a favorable prognosis. Adjuvant chemotherapy appears warranted in view of the high incidence of local recurrence and distant metastases.