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Ki-1 lymphoma with cardiac involvement at initial presentation

A L Papadopoulou1, M Argiriou, M Bonoris

  • 1First Department of Pediatrics, University of Athens, Aghia Sophia Children's Hospital, Greece.

Pediatric Hematology and Oncology
|June 6, 1998
PubMed
Summary

Pediatric anaplastic large cell lymphoma (ALCL) is rare. A 6-year-old boy with thymic Ki-1 lymphoma and an endocardial mass achieved complete remission with chemotherapy, highlighting successful treatment for this uncommon childhood cancer.

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Area of Science:

  • Pediatric oncology
  • Hematology
  • Cardiovascular pathology

Background:

  • Anaplastic large cell lymphoma (ALCL), a rare childhood malignancy, can present with extranodal involvement.
  • Thymic involvement by ALCL is uncommon, posing diagnostic challenges.
  • Endocardial masses in pediatric patients necessitate thorough investigation due to potential cardiac dysfunction.

Observation:

  • A 6-year-old boy presented with an endocardial mass.
  • Initial histology suggested a high-grade undifferentiated sarcoma.
  • Echocardiography was crucial for serial monitoring of the cardiac lesion.

Findings:

  • Reevaluation and immunohistochemistry confirmed the endocardial mass was a CD30-positive lymphoid neoplasm, specifically Ki-1 lymphoma of the thymus.

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  • The patient received chemotherapy for the diagnosed lymphoma.
  • The patient achieved complete remission and remained disease-free for 24 months post-treatment.

    • Implications:

    • This case highlights the importance of immunohistochemistry in diagnosing rare pediatric lymphomas, especially when initial histology is ambiguous.
    • It demonstrates that even rare presentations of ALCL, such as endocardial involvement, can be effectively treated with chemotherapy.
    • The successful management underscores the need for multidisciplinary care involving oncology, pathology, and cardiology in pediatric cases of lymphoma with cardiac manifestations.