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• W1131635399 abstract "Facial nerve schwannoma involving the middle cranial fossa is quite rare, and its accurate diagnosis is very difficult before surgery. Here we present a case of schwannoma of the facial nerve at the middle cranial fossa that was misdiagnosed previously at a local hospital and then cured in our hospital. CASE REPORT A 53-year-old man was admitted to a local hospital on January 26, 2005, because of left hemifacial spasm and progressive facial paresis on the left side for 10 years, and tinnitus with decreased hearing in the left ear for 8 years. On admission, physical examination showed infra-nuclear facial palsy and sensorineural hearing loss on the left side. Erosion of the posterosuperior wall of the left external auditory canal and inflammatory discharges from it were noted. Cranial CT demonstrated a cystic mass sized 5.2 cm × 4.2 cm × 3.5 cm at the left middle cranial fossa with soft-tissue window settings. Images obtained with a bone window setting showed erosion of the underlying petrous bone, mastoid air cells, and tympanic cavity (Fig. 1). Thus, the patient was diagnosed as having middle ear cholesteatoma complicated with otogenic brain abscess, and received left radical mastoidectomy and burr hole aspiration of the abscess at the left middle cranial fossa. During the operation, the surgeons found that the tympanic cavity and sinuses were filled with reddish-gray neoplasm, which eroded the mastoid and petrous bone and invaded into the cranial cavity. Then, xanchromatic fluid was aspirated from the cystic mass in the middle cranial fossa. The diagnosis of schwannoma was confirmed by postoperative histological examination.Fig. 1. A: : Axial CT showing a heterogeneously enhanced cystic mass at the left middle cranial fossa mimicking brain abscess (arrow). B: Axial CT with a bone window setting showing that the left petrous bone was severely eroded (arrow).For further treatment, the patient was referred to our hospital on March 7, 2005. On neurological examination, facial weakness with House-Brackmann grade V was noted. The patient showed a complete hearing loss in the left ear, but the facial sensation and visual acuity were normal. The lower cranial nerves were intact and there was no evidence of ataxia. MRI showed a heterogenerously enhanced mass sized 5.0 cm × 4.4 cm × 3.5 cm and multiple cysts at the middle cranial fossa, bulging anteriorly and upwardly, causing “indentation” of the temporal lobe (Fig. 2A). Therefore, an exploration of the left middle fossa through an infratemporal craniotomy was performed, and the reddish-gray cystic and lobulated tumor with a clear border in temporal lobe was totally resected (Fig. 2B). Because the underlying petrous bone and dura mater, adjacent to where geniculate ganglion was anatomically located, were severely eroded, we repaired the dural defect in the petrous bone with an artificial dura. During the operation, the gasserian ganglion was exposed. Postoperative histological examination revealed a typical pattern of schwannoma consisting of Antoni types A and B (Fig. 3). Immuno-histochemistry showed that the tumor was positive for S-100 protein, myelin basic protein (MBP), but negative for epithelial membrane antigen (EMA) and glial fibrillary acidic protein (GFAP). Since the initial presentations of the patient were facial spasm and paresis, and the tumor was mainly located at the middle cranial fossa along the course of the facial nerve, the diagnosis of a schwannoma of the facial nerve involving the middle cranial fossa was established. The patient was followed-up for 2 years, no evidence of tumor recurrence was detected.Fig. 2.: Preoperative (A) and postoperative (B) sagittal T1-weighted MRI with gadolinium enhancement. The multicystic tumor (arrow in A) occupied the petrous bone and extended anteriorly and upwardly into the left middle cranial fossa.Fig. 3.: Histological examination showing the typical characteristics of a schwannoma. (HE, original magnification ×20)DISCUSSION Facial nerve schwannomas are relatively uncommon tumors accounting for 1.9% of all intracranial neuromas.1 Facial nerve schwannomas involving the middle cranial fossa are especially rare, only 40 cases have been reported.2 It has been noted that there is a predilection for facial nerve schwannomas to arise from the area of geniculate ganglion, which might be attributable to the major structural reorganization occurring between the labyrinthine/geniculate and tympanic segments of the facial nerve.3 Large facial nerve schwannomas originating from the labyrinthine/geniculate segment tends to involve the middle cranial fossa because the geniculate ganglion is adjacent to the anterior surface of the petrous bone, and sometimes even without bony cover. Facial nerve schwannomas arising from the greater superficial petrosal nerve may also involve the middle cranial fossa by anterior extension through the facial hiatus. A long history of a gradually progressive or fluctuating facial paresis with hearing disturbance is the typical manifestation of facial nerve schwannomas. High-resolution CT and contrast-enhanced MRI play a critical role in the preoperative assessment of the tumor. Although high-resolution CT is not the pre-requisite for the diagnosis of facial nerve schwannomas, it is valuable for showing details about bony changes. Erosion of the petrous bone or enlargement of bony canal such as the internal auditory meatus and facial nerve canal can be clearly revealed by CT.4 MRI is preferred to detect a facial nerve schwannoma, even in a small size, and to clearly delineate the location and the extent of the tumors, which is particularly important for the resection of the tumor and the preservation of the facial nerve. MRI of a facial nerve schwannomas shows mildly hypo- or hyper-intense to grey matter on T1-weighted images and hyper-intense on T2-wighted images, which can be markedly enhanced by using gadolinium. Cystic change commonly shown in other schwannomas is occasionally seen in large facial schwannomas.5 The initial accurate diagnosis of facial nerve schwannomas involving the middle cranial fossa is challenging. It may be easily mistaken for primary cholesteatoma by using CT alone, because primary cholesteatoma has a tendency to destroy the petrous bone and the facial nerve canal, resulting in facial palsy. However, primary cholesteatoma are not expected to be enhanced on MRI images, contrary to facial nerve schwannomas.6 Unfortunately, in this patient, MRI was not performed before the first operation at the local hospital. Since the initial and long-standing symptoms of facial spasm and progressive facial paresis were ignored, the lesion eroding the petrous bone and the enhanced cystic mass at the middle fossa were misdiagnosed as a middle ear cholesteatoma complicated with otogenic brain abscess according to the neurological examination and CT findings. Since facial nerve schwannomas involving the middle cranial fossa are benign and produce intracranial mass effect, surgery is the first-line treatment. The suboccipital, infratemporal, or frontotemporal epidural transpetrous approach can be used.7 To deal with facial nerve schwannoma with both the middle ear and intracranial involvement, cooperations between otologists and neurosurgeons are necessary. In this case, the patient was initially treated with radical mastoidectomy and the residual tumor was removed through an infratemporal approach. The most concerned issue in the surgical treatment for facial nerve schwannoma is the postoperative function of the facial nerve. To achieve a total removal of the tumor, the facial nerve usually has to be sacrificed, and postoperative facial nerve function will not recover to over House-Brackmann grade III.8 After being sacrificed, the facial nerve is usually repaired by an interpositioning the greater auricular nerve, sural nerve, or vestibular nerve.9 Restoration of the facial nerve can be occasionally achieved by directly anastomosing its proximal and distal ends.10 Rarely, the continuity of facial nerve can be preserved.11 The prognosis of the facial nerve schwannoma is good. Only 16 recurrent cases were reported in the literatures.12 The recurrent tumor grows very slowly, with an average growth rate of 0.02 cm3 per year.13 The average time to detection of recurrence was approximately 8.3 years, ranging from 6 months to 34 years. If patients present with facial weakness and CT examination shows a mass at the middle cranial fossa, facial nerve schwannoma should be considered, and MRI scan is necessary. Surgery with help from otologists and neurosurgeons is highly recommended in order to achieve a satisfactory resection and the reservation of the facial function." @default.
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• W1131635399 title "Schwannoma of the facial nerve involving the middle cranial fossa: case report" @default.
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