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Lymphoepithelioma in childhood
T Pick1, H M Maurer, N B McWilliams
1Department of Pediatrics, Virginia Commonwealth University, Health Science Division, Medical College of Virginia, Richmond, Va., USA.
Insights
This study reports on nine Black children with lymphoepithelioma, a rare childhood cancer. Radiation therapy, with or without chemotherapy, achieved tumor regression in most cases, but recurrence necessitates further treatment strategies.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Medical Oncology
Background:
- Lymphoepithelioma is a rare malignancy predominantly affecting childhood.
- This report details a cohort of nine Black children diagnosed with this rare cancer.
Observation:
- Patients presented with unique clinical signs including cervical lymphadenopathy, torticollis, trismus, epistaxis, and voice changes.
- Nasopharyngeal masses were identified in seven children, though none were surgically resectable.
Findings:
- Eight of nine children achieved complete tumor regression with radiation therapy alone or combined with cyclophosphamide.
- Four patients experienced recurrence or metastasis within 10 months; two responded to further treatment.
- Long-term follow-up indicates four children remain disease-free, two are in remission from recurrent disease, and three succumbed to metastatic disease.
Implications:
- Achieving disease-free status for one year correlates with a favorable prognosis.
- Adjuvant chemotherapy is recommended due to the high rates of local recurrence and distant metastasis.
- Further research into optimal treatment protocols for pediatric lymphoepithelioma is warranted.
Abstract:
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.