Life threatening plexiform neurofibroma of a young child

Edit Bardi, Peter Masat, Mihaly Kisely, Janos Huszka, Balazs Tolvaj, Ferenc Garzuly, Bernadette Kalman

Abstract


Aim: To report a child with an extensive tumor invading multiple anatomical structures within the left side of her neck.

Material and methods: Presentation of a case including patient’s history, physical exam, molecular data, MRI imaging, histopathology and molecular genetics.

Results: The life-threatening compression as well as the dislocation of the trachea required meticulous surgical removal of the tumor. Histology revealed a plexiform neurofibroma related to a frame shift mutation in the NF1 gene.

Conclusion: Surgical removal of this extensive tumor saved the patient’s life. The long term success of management will be related to the effectiveness of the maintenance therapy that currently includes interferon-α2a. This report highlights unique aspects of a rare but life threatening condition in a young girl.

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DOI: https://doi.org/10.5430/crcp.v2n1p34

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Case Reports in Clinical Pathology

ISSN 2331-2726(Print)  ISSN 2331-2734(Online)

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