Late Manifestation of Neurofibromatosis of the right Trigeminal Nerv

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.

Publication History

Publication Date:
18 April 2018 (online)