Characterization of cerebral radiation necrosis following the treatment of sinonasal malignancies.

Objectives: Our study aims to determine the incidence and potential risk factors for cerebral radiation necrosis (CRN) following treatment of sinonasal malignancies. Methods: One hundred thirty-two patients diagnosed with sinonasal malignancies over an 18-year period were identified at two institutions. Forty-six patients meeting inclusion criteria and treated with radiation therapy were included for analysis. Demographic and clinical-pathologic characteristics were collected and reviewed. Post-treatment magnetic resonance imaging (MRI) at least 1 year following treatment was reviewed to determine presence or absence of CRN. Results: CRN was identified on MRI in 8 of 46 patients (17.4%) following radiation treatment. Patients with a history of reirradiation were more likely to develop CRN (50% vs. 10.5%, p < .05). The BEDs of radiation were also higher in CRN patients compared to non-CRN patients, but this difference was not significant (p > .05). CRN patients had a higher proportion of tumors with skull base involvement than non-CRN patients (100% vs. 57.9%, p = .037). Demographics, comorbidities, pathology, primary tumor subsite, chemotherapy use, and stage of disease demonstrated no significant increase in risk of CRN. Conclusions: Reirradiation and tumor skull base involvement were significant risk factors associated with CRN. Higher average total prescribed and BEDs of radiation were seen in the CRN groups, but these differences were not statistically significant. Gender, comorbidities, tumor subsite, tumor location, and treatment type were not significantly different between groups. Level of evidence: Level 3.

A Rare Presentation of Primary Cutaneous Low-Grade Neuroendocrine Tumor of the External Auditory Canal.

OBJECTIVE: To describe the first case of a primary cutaneous low-grade neuroendocrine tumor (cLGNET) originating from the external auditory canal as well as our team's surgical management. PATIENT: A healthy 34-year-old female presented with a low-grade neuroendocrine tumor of her right external auditory canal (EAC) which extended from the posterior-superior aspect of the EAC into the middle ear. INTERVENTION: A complete otological examination was performed in addition to CT and MRI imaging. The low-grade neuroendocrine tumor was surgically biopsied and further surgery was recommended for complete resection. RESULTS: Audiogram revealed profound right sensorineural hearing loss. CT scan demonstrated complete opacification of the right EAC, middle ear, and mastoid air cells, dystrophic calcification in the mesotympanum overlying the cochlear promontory, and no associated osseous erosion. MRI revealed abnormal FLAIR hyperintensity and enhancement of the labyrinthine segment of the right facial nerve, cochlea, and horizontal and posterior semicircular canals. An enhancing mass opacifying the right EAC demonstrating restricted diffusion on diffusion-weighted image was also evident. Pathologic examination and immunohistochemical staining confirmed a diagnosis of primary cLGNET of the EAC. CONCLUSION: Primary cLGNETs of the external ear are exceedingly rare but should be considered if an adult patient presents with a mass in the EAC. Management should include early biopsy and surgical excision followed by histological and immunohistochemical confirmation.

Radiologic anatomy of the round window relevant to cochlear implantation and inner ear drug delivery.

OBJECTIVE: To determine anatomic relationships and variation of the round window membrane to bony surgical landmarks on computed tomography. STUDY DESIGN: Retrospective imaging review. METHODS: 100 temporal bone images were evaluated. Direct measurements were obtained for membrane position. Vector distances and angulation from umbo and bony annulus were calculated from image viewer software coordinates. RESULTS: The angle of round window membrane at junction with cochlear basal turn was (42.1 ± 8.6)°. The membrane's position relative to plane of the facial nerve through facial recess was (14.7 ± 5.2)° posterior from a reference line drawn through facial recess to carotid canal. Regarding transtympanic drug delivery, the round window membrane was directed 4.1 mm superiorly from the inferior annulus and 5.4 mm anteriorly from the posterior annulus. The round window membrane on average was angled superiorly from the inferior annulus (77.1 ± 27.9)° and slightly anteriorly from the posterior annulus (19.1 ± 11.1°). The mean distance of round window membrane from umbo was 4 mm and posteriorly rotated 30° clockwise from a perpendicular drawn from umbo to inferior annulus towards posterior annulus. Together, these measurements approximate the round window membrane in the tympanic membrane's posteroinferior quadrant. CONCLUSIONS: These radiologic measurements demonstrate normal variations seen in round window anatomy relative to facial recess approach and bony tympanic annulus, providing a baseline to assess round window insertion for cochlear implantation and outlines anatomic factors affecting transtympanic drug delivery.

Neurosarcoidosis Directly Involving the Cervical Vagus Nerve.

OBJECTIVES: To present a novel location in which neurosarcoidomatous inflammation is identified and its accompanying presentation. METHODS: The authors present a case of bilateral vocal fold paresis associated with non-caseating granulomatous inflammation of the cervical and intra-axial portions of the vagus nerve masquerading as a cranial nerve tumor. RESULTS: Examination revealed bilateral vocal fold paresis and asymmetric palate elevation. MRI demonstrated enhancing bilateral jugular foramen masses, and neck ultrasound demonstrated bilateral thickened appearance of the vagus nerves. Vagus nerve biopsy demonstrated non-caseating granulomas. CONCLUSIONS: Neurosarcoidosis may contribute to variable cranial neuropathies. Vocal fold paresis is usually thought to arise from mediastinal compression of the left recurrent laryngeal nerve. Rarely, though, lesions may arise in other parts of the vagus nerve. Failure of response to steroids does not rule out the diagnosis, making tissue diagnosis important in some cases.

Effect of multimodality treatment on overall survival for patients with metastatic or recurrent HPV-positive head and neck squamous cell carcinoma.

BACKGROUND: The optimal treatment for patients with recurrent human papillomavirus (HPV)-positive head and neck cancer is poorly understood. METHODS: We investigated treatments and outcomes in patients with recurrent head and neck cancer. Treatments included salvage neck surgery, metastasectomy, hypofractionated reirradiation, chemoembolization, and chemotherapy. Treatment outcomes were compared based on HPV status. RESULTS: A total of 37 patients were identified (12 HPV positive and 25 HPV negative). Demographics were similar. Overall, there was a trend toward a higher number of total treatment interventions in patients with HPV-positive disease (4.5 vs 2.6), but this was statistically insignificant (p=.066). After a mean follow-up of 21 months, median survival in HPV-negative patients was 10.6 months, whereas the median survival had not been reached for HPV-positive patients. Of the 12 HPV-positive patients, 7 were still alive (58%) after a mean follow-up period of 33 months. CONCLUSION: Multimodality aggressive therapy may improve overall survival in patients with recurrent HPV-positive disease. Further prospective research is warranted.

Refractory trigeminal neuralgia treatment outcomes following CyberKnife radiosurgery.

INTRODUCTION: A handful of studies have reported outcomes with CyberKnife radiosurgery (CKRS) for the treatment of trigeminal neuralgia. However, the follow-up has been short with no minimum follow-up required and have included patients with short duration of symptoms. Here we report our institutional experience on patients with a minimum follow-up of 1 year and a median follow-up of 28 months (mean 38.84 months). METHODS: Twenty-five patients with medically and surgically intractable TN received CKRS with a mean marginal radiation dose of 64 Gy applied to an average isodose line of 86% of the affected trigeminal nerve. Follow-up data were obtained by clinical examination and telephone questionnaire. Outcome results were categorized based on the Barrow Neurological Institute (BNI) pain scale with BNI I-III considered to be good outcomes and BNI IV-V considered as treatment failure. BNI facial numbness score was used to assess treatment complications. RESULTS: A large proportion of patients (42.9%) reported pain relief within 1 month following CKRS treatment. The mean time to recurrence of severe pain was 27.8 months (range 1-129 months). At median follow-up of 28 months (mean 38.84 months), actuarial rate of freedom from severe pain (BNI ≥ III) was 72%. At last follow-up 2 (8%) patients had freedom from any pain and no medications (BNI I) and the majority (48%) had some pain that was adequately controlled with medications. Seven patients (28%) had no response to treatment and continued to suffer from severe pain (BNI IV or V). Patient's diabetic status and overall post-treatment BNI facial numbness scores were statistically significant predictors of treatment outcomes. CONCLUSION: CKRS represents an acceptable salvage option for with medically and/or surgically refractory patients. Even patients with severely debilitating symptoms may experience significant and sustained pain relief after CKRS. Particularly, CKRS remains an attractive option in patients who are not good surgical candidates or possibly even failed surgical therapy. This data should help in setting realistic expectations for weighing the various available treatment options.

IMRT with Stereotactic Body Radiotherapy Boost for High Risk Malignant Salivary Gland Malignancies: A Case Series.

Patients with high risk salivary gland malignancies are at increased risk of local failure. We present our institutional experience with dose escalation using hypofractionated stereotactic body radiotherapy (SBRT) in a subset of this rare disease. Over the course of 9 years, 10 patients presenting with skull base invasion, gross disease with one or more adverse features, or those treated with adjuvant radiation with three or more pathologic features were treated with intensity-modulated radiation therapy followed by hypofractionated SBRT boost. Patients presented with variable tumor histologies, and in all but one, the tumors were classified as poorly differentiated high grade. Four patients had gross disease, three had gross residual disease, three had skull base invasion, and two patients had rapidly recurrent disease (≤6 months) that had been previously treated with surgical resection. The median stereotactic radiosurgery boost dose was 17.5 Gy (range 10-30 Gy) given in a median of five fractions (range 3-6 fractions) for a total median cumulative dose of 81.2 Gy (range 73.2-95.6 Gy). The majority of the patients received platinum based concurrent chemotherapy with their radiation. At a median follow-up of 32 months (range 12-120) for all patients and 43 months for surviving patients (range 12-120), actuarial 3-year locoregional control, distant control, progression-free survival, and overall survival were 88, 81, 68, and 79%, respectively. Only one patient failed locally and two failed distantly. Serious late toxicity included graft ulceration in one patient and osteoradionecrosis in another patient, both of which underwent surgical reconstruction. Six patients developed fibrosis. In a subset of patients with salivary gland malignancies with skull base invasion, gross disease, or those treated adjuvantly with three or more adverse pathologic features, hypofractionated SBRT boost to intensity-modulated radiotherapy yields good local control rates and acceptable toxicity.

Trigeminal neuralgia treatment outcomes following Gamma Knife radiosurgery with a minimum 3-year follow-up.

OBJECTIVE: Effective short-term outcomes have been well documented for trigeminal neuralgia (TN) patients treated with Gamma Knife radiosurgery (GKRS) with reported success rates of 70-90 % with median follow-up intervals of 19-75 months. Fewer series, however, have described uniform long-term follow-up data. In this study, we report our long-term institutional outcomes in patients treated with GKRS after a minimum follow-up of 36 months. METHODS: Thirty-six consecutive patients with medically intractable TN received a median radiation dose of 45 Gy applied with a single 4-mm isocenter to the affected trigeminal nerve. Follow-up data were obtained by clinical examination and telephone questionnaire. Outcome results were categorized based on the Barrow Neurological Institute (BNI) pain scale with BNI I-III considered to be good outcomes and BNI IV-V considered as treatment failure. BNI facial numbness score was used to assess treatment complications. RESULTS: The incidence of early pain relief was high (80.5 %) and relief was noted in an average of 1.6 months after treatment. At minimum follow-up of 3 years, 67 % were pain free (BNI I) and 75 % had good treatment outcome. At a mean last follow-up of 69 months, 32 % were free from any pain and 63 % were free from severe pain. Bothersome posttreatment facial numbness was reported in 11 % of the patients. A statistically significant correlation was found between age and recurrence of any pain with age >70 predicting a more favorable outcome after radiosurgery. CONCLUSION: The success rate of GKRS for treatment of medically intractable TN declines over time with 32 % reporting ideal outcome and 63 % reporting good outcome. Patients older than age 70 are good candidates for radiosurgery. This data should help in setting realistic expectations for weighing the various available treatment options.

Pathologic collision of inverted papilloma with esthesioneuroblastoma.

BACKGROUND: Inverted papilloma (IP) of the nasal cavity is a benign tumor that represents 0.5-4% of all nasal tumors and have been known to rarely undergo malignant transformation to squamous carcinoma and even more rarely adenocarcinoma. Synchronous association with low-grade esthesioneuroblastoma (ENB) has been reported in only one case report where a small-sized lesion was treated with surgery alone. Here we report the first case of invasion of IP by high-grade ENB with nodal metastasis that was treated with combined modality therapy. CASE PRESENTATION: A case of a 64-year-old African American gentleman presented to the otolaryngology with a 3-month history of recurrent epistaxis. Imaging revealed a large right nasal cavity mass extending into the right sphenoid sinus but without intracranial extension. Surgical pathology revealed high-grade ENB invading IP. An orbitofrontal craniotomy approach was used to achieve complete resection of the mass but with positive margins. Post-operative positron emission tomography/computed tomography showed nodal metastasis. The patient was then treated with adjuvant chemoradiation and remains without evidence of disease at 42 months post-treatment. We discuss the disease presentation, histopathologic features, and disease management with literature support. CONCLUSION: In this very rare disease presentation where two extremely rare malignancies collide, we show that aggressive management with trimodality therapy of surgery, adjuvant radiation with stereotactic radiosurgical boost, and adjuvant chemotherapy gives excellent results. Given the natural history of the disease, however, long follow-up is needed to declare complete freedom from the disease.

Frameless fractionated stereotactic radiosurgery for vestibular schwannomas: a single-institution experience.

OBJECTIVE: To examine tumor control, hearing preservation, and complication rates after frameless fractionated stereotactic radiosurgery (SRS) in patients with vestibular schwannomas (VS). METHODS: Thirty-seven patients treated with fractionated SRS from 2002 to 2011 were retrospectively analyzed. Ninety-five percent were treated with 25 Gy in five fractions, targeting a median tumor volume of 1.03 cc (range 0.14-7.60). RESULTS: With a median follow-up of 4.25 years (range, 15 months-9 years), no tumors required an additional treatment resulting in 100% tumor control rate. Radiographic control rate was 91% in 32 patients at a median follow-up of 3 years. Of the 14 patients with serviceable hearing and with audiograms, the hearing preservation rate was 78% at a median follow-up of 18 months. Twenty-six patients with serviceable hearing pretreatment, were evaluated by a phone survey with a hearing preservation rate of 73% at a 5 year median follow-up. There were two cases that developed both new increased trigeminal parasthesias and facial spasms but there were no cases of facial weakness. Patient had 96% of good to excellent satisfaction rate with the treatment at a median follow-up of 5 years. CONCLUSION: Frameless fractionated SRS treatment of VS results in good rate of tumor control. Hearing preservation rate and rates of cranial nerve toxicity are comparable to what is reported in the literature. Patients choose this modality because of its non-invasive nature and are generally very satisfied with their long term outcome.