The Japanese Journal of Pediatric Hematology
Online ISSN : 1884-4723
Print ISSN : 0913-8706
ISSN-L : 0913-8706
A 14-Year-Old Girl with Neurofibromatosis Type-1 with Cecal Non-Hodgkin's Lymphoma
Seiki HORITANatsuhito NISHIOTetsurou OOKI
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1992 Volume 6 Issue 3 Pages 241-244

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Abstract
A 14-year-old girl with neurofibromatosis type-1 complicating with the cecal non-Hodgkin's lymphoma is reported. She was referred to our hospital because of an abdominal mass, and was found to have a solid tumor of the cecal region, 4 cm in diameter, by ultrasonographic examination. Recurrent ileo-cecal intussusception occurred after admission. Precise barium enema study demonstrated two mass defects, 4.5 cm and 2.0 cm in diameter, of the cecal region. The cecum including tumor was widely resected with ileo-colonic anastomosis. Histological diagnosis was non-Hodgkin's lymphoma, diffuse medium-sized cell type (LSG class). The tumor cells showed CD 10+, CD 19+, CD20+, HLA-DR+, cytoplasmic, μ+, and surface IgM+ by immunophenotyping analysis, indicating that they were classified to the mature B-cell phenotype. Previous reports of neurofibromatosis type-1 with malignant lymphoma including our case were also reviewed.
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