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Malignant peripheral neuroectodermal tumor in an infant with neurofibromatosis type 1

✍ Scribed by Chan, Godfrey C.F.; Nicholls, John M.; Lee, Anselm C.W.; Chan, Li Chong; Lau, Yu Lung

Publisher: John Wiley and Sons
Year: 1996
Tongue: English
Weight: 610 KB
Volume: 26
Category: Article
ISSN: 0098-1532
DOI: 10.1002/(sici)1096-911x(199603)26:3<215::aid-mpo12>3.0.co;2-f

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✦ Synopsis

A case of multifocal malignant peripheral neuroectodermal tumor (PNET) arising from a plexiform neurofibroma in a 4-month-old Chinese boy with neurofibromatosis type 1 (NF-1) is described. Cytogenetic culture demonstrated hypotriploid karyotype with an abnormal clone characterized by 5-0, XY, +2, +3, +6, +8, +8, +12, +i(13)(qIO), +der(14)t(1;14)(q21;q32), +16, +19, +20, +mar[cp3] with no apparent abnormality of chromosome 17. The child was treated with combination chemotherapy comprising ifosphamide, vincristine and doxorubicin. Despite initial partial response the child finally died of tumor progression and pulmonary metastases 8 months after diagnosis. We believe this is the first reported case of PNET in a child with NF-1 and may support an association between these two disorders of neural crest origin.

o 1996Wiley-Liss, Inc.

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