CC BY-NC-ND 4.0 · Laryngorhinootologie 2018; 97(S 02): S372
DOI: 10.1055/s-0038-1641006
Abstracts
Speicheldrüsen/Schilddrüsen: Salivary Glands/Thyroid Gland

Late Manifestation of Neurofibromatosis of the right Trigeminal Nerv

M Just
1   Uniklinikum, HNO-Klinik, Leipzig
,
J Rudolph
1   Uniklinikum, HNO-Klinik, Leipzig
,
A Dietz
1   Uniklinikum, HNO-Klinik, Leipzig
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    Introduction:

    Neurofibromatosis (NF) is a autosomal-dominant, monogenous inherited disease which affects multiple organs. NF typ 1 is one of the most common inherited neurological diseases, which is associated with tumours of the nervous system. Distinctive are cutaneous and subcutaneous neurofibroma, which typically appear in the adolescence.

    Case-history:A sixty-nine-year-old woman was presenting with unilateral, subcutaneous and painful small bumps in the face, scalp and behind the ear. Symptoms including light brown spots on the skin, freckles in the armpit and groin were absent. No motility disorder, no vision loss, but growths on the eye's iris. Symptoms occurred since 2009. In 2009 MRI showed an normal examination. In 2015 and 2016 MRI displayed an intraorbitale, partly extraconale tumour, max. 2,7 × 1,3 cm as well as small bumps of the lower eyelid. Because of severe algesia multiple bumps were surgical removed, although there is no strict recommendation.

    Results:

    The histopathological results showed a fibrosed connective tissue with nodular tumour tissue with interstitial edema and partially elongated cell nuclei with a vertebral arrangement of the tumour cells and low cell and nuclear polymorphism, corresponding to neurofibroma.

    Conclusions:

    This case-presentation should draw attention to the potential late manifestation of neurofibromatosis, especially regarding the involvement of just one single cranial nerv. Furthermore the importance of surgical resection in case of severe pains should be pointed out.


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    No conflict of interest has been declared by the author(s).

    Dr. med. Maren Just
    Universitätsklinikum Leipzig, HNO-Klinik,
    Liebigstr. 10 – 14, 04103,
    Leipzig

    Publication History

    Publication Date:
    18 April 2018 (online)

    © 2018. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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