Unique case of a GLI1 amplified biphasic mesenchymal tumor of the orbit.

GLI1-altered mesenchymal tumors are an emerging entity in soft tissue pathology. In the head and neck region, they are most commonly in the tongue. Limited published data indicate a propensity for local recurrence, regional spread, and distant metastasis in both GLI1-rearranged and GLI1-amplified tumors. The purpose of this report is to present the rare case of a GLI1-amplified spindle cell tumor of the orbit and a focused review of the literature. A 54-year-old woman presented with proptosis, eye pain, and ocular motility restriction in the left eye. Imaging demonstrated a tumor occupying the superomedial intraconal orbit that was distinct from the extraocular muscles, optic nerve, and globe. The tumor was totally resected with a combined open transorbital and endoscopic, endonasal approach. Pathological analysis demonstrated a spindled and epithelioid mesenchymal tumor with diffuse nuclear GLI1 expression. PCR-based, next*-generation sarcoma fusion panel was negative for GLI1 fusions, including GLI1::ACTB fusions; however, DDIT3 breaks apart fluorescence in situ hybridization (FISH), which can be used as a surrogate for GLI1 alterations due to proximity to 12q13.3, showing amplification. Post-operatively, the patient had recovered visual acuity. She received adjuvant radiation therapy (60 Gy in 30 fractions). Surveillance for recurrence, regional spread, and distant metastasis has been negative at a 6-month follow-up. Ultimately, we report the first case of a GLI1-amplified mesenchymal neoplasm of the intraconal orbit managed with gross total resection via a combined approach followed by adjuvant radiation therapy.

CT findings of sinonasal respiratory epithelial adenomatoid hamartoma: a closer look at the olfactory clefts.

BACKGROUND AND PURPOSE: Respiratory epithelial adenomatoid hamartoma is a benign glandular neoplasm of the sinonasal cavities, which presents in isolation (REAHi) or in the setting of an adjacent inflammatory process such as sinonasal polyps. It is frequently found in the olfactory clefts. CT features of the 2 clinical presentations have not been well defined. We present the CT findings of REAH, focusing on the degree of associated sinusitis and changes in the OCs. We hypothesized that widening of the OCs and associated severity of the sinusitis are diagnostic features of REAH, differentiating it from SNP. MATERIALS AND METHODS: In this case-control study, we compared patients with REAHi, those with REAH in the setting of SNP (REAHsnp), and those with SNP only (control patients). Patients with REAH were excluded if they had an adjacent inflammatory process other than SNP or if they did not have disease in the OC. We analyzed Harvard sinus CT scores and OC dimensions. RESULTS: A total of 29 patients with REAH were included: 7 with REAHi and 22 with REAHsnp. A total of 26 control patients were identified. Patients with REAHi had significantly lower Harvard CT scores than did the other groups. The OC width and the ratio of OC to the total nasal distance were significantly larger in both REAH groups compared with those of the control patients. If the OC is 10 mm or more, the sensitivity and specificity for the presence of REAH are 88% and 74%, respectively. CONCLUSIONS: Both clinical presentations of REAH are associated with OC widening on CT scan. In the setting of polypoid disease, an OC width of > 10 mm should increase suspicion for the presence of REAH.

Approach to the vestibular patient and driving: A patient perspective.

OBJECTIVES: To learn about the impact of dizziness on driving from a patient perspective and to present an approach to the vestibular patient and driving. DESIGN: An anonymous questionnaire completed by 265 dizzy patients at 3 different centers. RESULTS: The participants were experienced drivers who needed to drive to function normally (83%). Those with constant or severe dizziness comprised a higher risk group of drivers. Although few had ever been warned not to drive, 52% said that if they were warned to stop driving, they would not. Most thought that it was the doctor's role to report unsafe drivers to the authorities (P < 0.001, chi2 = 87.2670). CONCLUSIONS: The diagnosis of a vestibular disorder should not alone be grounds to suspend a patient's driver's license. Legislation should be amended to better reflect the views of doctors and patients alike.

Epithelioid leiomyosarcoma of the larynx.

BACKGROUND: Tumors of smooth muscle origin are rare in the upper aerodigestive tract, due to the paucity of smooth muscle in the area. A review of the literature revealed 34 reported cases of leiomyoma, 9 cases of leiomyosarcoma, and only 2 cases of epithelioid leiomyoma arising in the larynx. METHODS: A case report of an epithelioid leiomyosarcoma arising in the right piriform fossa of a 38-year-old man is presented. RESULTS: The tumor was treated with conservative surgical resection and postoperative radiotherapy. There was no evidence of locoregional recurrence 2 years postsurgery, and laryngeal function was preserved. CONCLUSIONS: To our knowledge, this is the third case of a laryngeal epithelioid smooth muscle tumor to be reported in the world literature and the only documentation of an epithelioid leiomyosarcoma arising in the larynx.

Reporting of vestibular patients who are unfit to drive: survey of Canadian otolaryngologists.

OBJECTIVES: The objectives of this study were (1) to learn about the concerns and current practices of Canadian otolaryngologists with regard to the reporting of vestibular patients; and (2) to examine the three different reporting mandates in Canada by surveying otolaryngologists in Ontario, Alberta, and British Columbia, and to assess the level of satisfaction with reporting methods. METHOD: A survey was mailed to all members of the Canadian Society of Otolaryngology-Head and Neck Surgery in Ontario, Alberta, and British Columbia, using a modified version of the "Dillman Total Survey Design Method." RESULTS: The survey had an overall response rate of 62.8%. Although many respondents have considered reporting vestibular patients (82.2%) and have warned patients not to drive without reporting them (84.2%), only 25.3% actually have. The great majority (91.8%) felt that unfit drivers should be brought to the attention of the transportation authority. There was no consensus of opinion on the best method of reporting, but only 23.3% of those surveyed supported mandatory physician reporting. Ontario otolaryngologists were significantly less satisfied with the fitness-to-drive legislation in their province than were those from Alberta or British Columbia (chi 2 = 22.7, p = .001). Almost three times as many Ontario respondents have actually reported a vestibular patient (chi 2 = 6.7, p = .01). CONCLUSIONS: The study results suggest that mandatory physician reporting is not a satisfactory method of reporting unfit-to-drive vestibular patients. It is highly recommended that all physicians become familiar with the reporting guidelines in their province or territory and comply with their legal obligations.

Impact of vestibular disorders on fitness to drive: a census of the American Neurotology Society.

BACKGROUND: Legislation for reporting unfit-to-drive patients by physicians varies dramatically among U.S. states and Canadian provinces. The impact of vestibular disease on driving safety is not well described in the literature. OBJECTIVES: (1) to learn more about the critical issues and current practices of neurotologists regarding reporting of vestibular patients and (2) to make recommendations for reporting patients with vestibular disorders. STUDY DESIGN: A mailed census of members of the American Neurotology Society using a questionnaire based on a modified Dillman Total Design Survey Method. RESULTS: Most respondents are aware of the potential safety risks of patients who drive with vestibular diseases, most notably those with Tumarkins' attacks. Although many have counselled patients (94%) and considered reporting vestibular patients (75%), few actually have (14%). There was no consensus of opinion on a method of reporting unfit-to-drive patients. Only 18.9% of respondents supported mandatory physician reporting. Respondents who live in states where reporting is not mandatory and who are aware of this fact are (a) more satisfied with their state's legislation (chi 2 = 60.1, p = 0.001) and (b) less likely to report patients who they consider unfit-to-drive (chi 2 = 10.6, p = 0.03). CONCLUSIONS: In that there is no general consensus amongst respondents and the relative safety risks seem low in comparison to other disorders, at present we do not advocate mandatory reporting of patients with vestibular disorders.