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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.
The Journal of Pediatrics
|January 1, 1974
Summary
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.