Cerebellopontine angle and intracanalicular masses mimicking vestibular schwannomas.

OBJECTIVE: To describe the clinical and radiographic characteristics in a series of patients with non-vestibular schwannoma cerebellopontine angle (CPA) and intracanalicular (IAC) masses, who underwent microsurgery for presumed vestibular schwannoma (VS). STUDY DESIGN: Retrospective case series. SETTING: Tertiary neurotologic referral center. PATIENTS: One thousand five hundred ninety-three patients underwent microsurgery for apparent VS from 2002 to 2013. Of these, 53 patients (3%) were discovered to have a diagnosis other than VS. INTERVENTIONS: Middle fossa, translabyrinthine, and retrosigmoid craniotomy. MAIN OUTCOME MEASURES: Clinical presentation, radiologic analysis, and histopathology examination. RESULTS: There were 17 facial schwannomas, 15 meningiomas, 9 hemangiomas, 6 lipochoristomas, 3 inflammatory reactions, and one each of lymphoma, glial heterotopia, solitary fibrous tumor, ependymoma, and a non-diagnostic mass. Excluding facial schwannomas, 23 cases were misdiagnosed as VS in the first half of the study period, compared to only 15 cases in the latter half (p = 0.09). Ninety-six percent of patients presented with some combination of sensorineural hearing loss, balance disturbance, and tinnitus. In the subset of patients with available preoperative MRI scans for retrospective review, only 4 of 28 patients had radiologic findings suggestive of pathology other than VS. The most common missed radiologic diagnoses were facial schwannoma, lipochoristoma, and meningioma. CONCLUSION: A subgroup of patients with CPA and IAC masses who present with radiologic findings diagnostic of VS will have an alternative histopathologic diagnosis. Optimal radiologic imaging and experienced interpretation can improve diagnostic accuracy. The most common tumors that mimic VS despite ideal radiologic imaging are facial schwannomas, meningiomas, and hemangiomas.

Otolithic membrane damage in patients with endolymphatic hydrops and drop attacks.

OBJECTIVES: 1. Evaluate the otolithic membrane in patients with endolymphatic hydrops (EH) and vestibular drop attacks (VDA) undergoing ablative labyrinthectomy. 2. Correlate intraoperative findings to archival temporal bone specimens of patients with EH. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. SPECIMEN SOURCE: 1. Patients undergoing labyrinthectomy for incapacitating Ménière's disease (MD), delayed EH, VDA, or acoustic neuroma (AN) between 2004 and 2011. 2. Archival temporal bone specimens of patients with MD. INTERVENTIONS: Ablative labyrinthectomy. MAIN OUTCOME MEASURES: Examination of the utricular otolithic membrane. RESULTS: The otolithic membrane of the utricle was evaluated intraoperatively in 28 patients undergoing labyrinthectomy. Seven (25%) had a history of VDA, 6 (21%) had delayed EH, 9 (32%) had MD, and 6 (21%) had AN. All patients with VDA showed evidence of a disrupted utricular otolithic membrane, whereas only 50% and 56% of patients with delayed EH and MD, respectively, demonstrated otolithic membrane disruption (p = 0.051). None of the patients with AN showed otolithic membrane disruption (p = 0.004). The mean thickness of the otolithic membrane in 5 archival temporal bone MD specimens was 11.45 micrometers versus 38 micrometers in normal specimens (p = 0.001). CONCLUSION: The otolithic membrane is consistently damaged in patients with VDA. In addition, there is a significantly higher incidence of otolithic membrane injury in patients with MD and delayed EH compared with patients without hydrops, suggesting that the underlying pathophysiology in VDA results from injury to the otolithic membrane of the saccule and utricle, resulting in free-floating otoliths and atrophy.

Cochlin expression in vestibular endorgans obtained from patients with Meniere's disease.

The distribution of cochlin and its associated basement membrane proteins (collagen IV, collagen II, laminin-ß2, and nidogen-1) were evaluated in the vestibular endorgans of subjects with Meniere's disease and compared with normal specimens. Cochlin mRNA expression in vestibular endorgans from Meniere's disease specimens was also investigated. Specimens were obtained from patients who had Meniere's disease and who were undergoing ablative labyrinthectomy. Control specimens were obtained both from autopsy specimens with documented normal audiovestibular function and from patients undergoing labyrinthectomy for acoustic neuroma excision. In the normal control specimens, cochlin immunoreactivity was found evenly distributed in the stroma of the cristae ampullaris and maculae of the utricle. In Meniere's specimens, cochlin immunoreactivity was markedly increased; this was associated with an increase in cochlin mRNA expression as shown by real-time reverse transcription with the polymerase chain reaction. Collagen IV and laminin-ß2 immunoreactivity was significantly decreased in Meniere's specimens. Nidogen-1 and collagen II immunoreactivity was unchanged in Meniere's specimens when compared with normal samples. Cochlin upregulation has been implicated in the hereditary audiovestibulopathy, DFNA9. The increased expression of cochlin and decreased expression of collagen IV and laminin in Meniere's disease are suggestive that the overexpression of cochlin contributes to the dysfunctional inner ear homeostasis seen in this disease.

Adenoid cystic carcinoma of the airway: a 30-year review at one institution.

PURPOSE: The purpose of the study was to evaluate the treatment results of adenoid cystic carcinoma (ACC) of the airway at a single institution during a 30-year period. MATERIALS AND METHODS: All cases of ACC of the airway over a 30-year period at one tertiary care institution were reviewed retrospectively. The demographics, treatment modalities, pathologic characteristics, and outcomes were evaluated. RESULTS: Eleven patients were treated for ACC of the airway with an age range of 25 to 72 years (median, 48 years). Six patients presented with ACC in the larynx, and 5 patients had ACC of the trachea. All patients underwent surgical excision and radiation; 9 of 11 patients had postoperative external beam radiation, 1 patient had preoperative external beam radiation, and the remaining patient had postoperative neutron beam therapy. Four patients with tracheal ACC and none with laryngeal ACC had microscopic or grossly positive margins after surgery (P = .048). Eighty percent of patients had perineural invasion on pathology. Two patients with tracheal ACC had local recurrence of disease, which occurred at 1 and 10 months postoperatively. One patient with laryngeal ACC died of distant metastatic disease at 16 months. Follow-up varied from 4 to 168 months (median, 31 months). CONCLUSIONS: We report high disease-free survival rates for ACC of the airway in patients who underwent definitive surgical resection followed by postoperative radiation. There is a higher risk for local recurrence and positive surgical margins with distal tracheal location. Distant disease ultimately determines survival.

Poorly differentiated adenocarcinoma arising from a cervical bronchial cyst.

Bronchogenic cysts with malignant change are rarely reported. We describe a case of poorly differentiated adenocarcinoma arising from a cervical bronchial cyst in a patient presenting with a thyroid mass, cervical lymphadenopathy, and initial biopsy suggestive of papillary thyroid carcinoma. The clinical presentation, intraoperative findings, radiographic images, and pathology slides are presented. To our knowledge, this is the first report of a poorly differentiated adenocarcinoma arising from a bronchial cyst in the cervical region.