We report the presence of a schwannoma within a neurofibroma of the intratemporal facial nerve. This neurofibroma recurred 39 years after its first excision in the parotid gland. Although some believe that schwannomas and neurofibromas represent the same entity, these tumors present distinctive histopathologic and clinical characteristics, which are discussed. The extreme rarity of a schwannoma developing within a neurofibroma is underlined. This is the first report of such an association occurring within a cranial nerve.
BackgroundThis study aims to assess the effectiveness of delayed facial nerve decompression for Bell’s palsy (BP).MethodsWe performed a retrospective case review of all patients having undergone facial nerve decompression for severe refractory BP between 1984 and 2009 at our tertiary referral center. Demographics, timing between onset of symptoms and surgical decompression, degree of facial nerve dysfunction pre- and post-operatively, follow-up length after surgery and postoperative complications were recorded. Facial nerve dysfunction was assessed using the House-Brackmann (HB) scale. Electroneuronography, electromyography and imaging results were assessed when available.ResultsEighteen patients had surgery between 21 and 60 days after onset of BP (group I), and 18 patients had surgery more than 60 days after onset of symptoms (group II). In group II, 11 patients had surgery between 61 and 89 days and 7 patients after 90 days. Groups I and II showed similar functional gain and rates of improvement to HB 3 or better (11/18 vs. 11/18, p > 0.05). In group II, patients operated 60 to 89 days after onset of BP showed a significantly higher rate of improvement to HB 3 or better (9/11 vs. 2/6, p = 0.049) with higher functional gain compared to those operated after 90 days (p = 0.0293).ConclusionsWhen indicated, facial nerve decompression for BP is usually recommended within the first 2 weeks of onset of facial paralysis. Nonetheless, our results suggest that patients with severe BP could benefit from decompression surgery within 90 days after onset of symptoms in the absence of an opportunity to proceed earlier to surgery. Further investigation is still required to confirm our findings.Trial registrationRetrospective registered. IRB# 2016–6154, CE 15.154 – CA
F acial nerve schwannomas and neurofibromas form a rare clinical entity, comprising only a small part of the 6% of facial nerve palsies produced by neoplasms. 1,2 The term n e u r o m a is offen used to describe benign tumors of neural origin, inct, uding schwannomas and neurofibromas. These two benign tumors are offen incorrectly thought to represent the same entity. Although both are derived from the Schwann cell, they have clearly distinct histopathologic characteristics, and their clinical course is not necessarily the same.We present the case of a patient with a left intratemporal facial nerve neurofibroma. This patient initially sought treatment 39 years earlier and required excision of a left intraparotid facial neurofibroma. The histologic appearance of this new tumor is that of a neurofibroma, with the peculiar characteristic of a classic schwannoma having developed within this neurofibroma.Although this association has previously been described in a few isolated specimens, a review of the literature reveals that this is the first documented case of a schwannoma developing within a cranial nerve neurofibroma and only the fiRh documented case of an intraparotid facial neurotibroma.
CASE REPORTA 61-year-old healthy man was referred to our clinic in November 1990 with a 9-month histo .ry of progressive left-sided hearing loss. The patient's past history revealed From Notre Dame Hospital (Drs. Kayem, Dufouß and Robert) and the University of Montreal (Dr. Kayem).
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