Person:

Remenschneider, Aaron

Background and Importance Plasmacytomas are monoclonal proliferations of plasma cells that may arise within soft tissue or bone. The skull base is a rare site for plasmacytomas to occur, and few cases have been reported in the literature. When present in the skull base, plasmacytomas may result in cranial neuropathies and often progress to multiple myeloma more rapidly than other intracranial or skeletal plasmacytomas. Clinical Presentation A 69-year-old man presented with a primary complaint of diplopia and an examination consistent with bilateral abducens nerve palsy. No other deficits were noted. Magnetic resonance imaging of the skull base demonstrated a large T1 isointense moderately enhancing lesion centered within the clivus. Endoscopic biopsy of the mass revealed sheets and aggregates of mature monoclonal plasma cells. The patient's initial systemic work-up revealed that this was a solitary lesion, and he was treated with radiation therapy to the skull base with a durable local effect at 18-month follow-up. Unfortunately he progressed to multiple myeloma with peripheral osteolytic lesions but has been stabilized on chemotherapeutics. Conclusion: The clivus is an unusual site for intracranial plasmacytomas, and enhancing lesions must be differentiated from chordoma. Characteristic findings on histopathology include an immunoglobulin light-chain restricted clonal proliferation of plasma cells. Treatment is most commonly radiotherapy with surgery reserved for biopsy and palliation. Clinicians should be aware of the increased risk of progression to multiple myeloma in skull base plasmacytomas.

Objective: Lipochoristomas comprise 0.1% of all cerebellopontine angle tumors and may be misdiagnosed as vestibular schwannomas. We describe a lipochoristoma of the internal auditory canal (IAC) and present the salient features of the evaluation, diagnosis, and management. Study Design and Methods Retrospective case review. Results (Case Report) A 51-year-old woman presented with left-sided severe hearing loss, tinnitus, mild episodic vertigo, and facial paresthesias progressive over 1 year. Magnetic resonance imaging (MRI) demonstrated a small (5 × 4 mm) T1 hyperintense lobulated lesion in the distal internal auditory canal. With fat-suppressed images, there was no enhancement of the lesion. A diagnosis of IAC lipochoristoma was made. Conservative management was recommended, and on 17-month follow-up there was no interval growth. The patient remains symptomatically stable with improved equilibrium but persistent left-sided hearing loss. Conclusions: Differential diagnosis of an enhancing lesion in the IAC includes acoustic neuroma, meningioma, epidermoid and arachnoid cysts, lipochoristoma, and metastatic tumors. Fat-suppressed MRI sequences aid in a definitive diagnosis of lipochoristomas. Because lipochoristomas may have a tendency for more indolent growth and intimate involvement of the auditory nerve, conservative management with interval imaging is recommended. Surgical treatment is reserved for growing lesions or those with disabling vestibular symptoms.

Internal carotid artery injury is a rare and devastating complication of endoscopic sinus and skull base surgery that has an associated mortality rate of 15%. This case describes a patient who developed massive epistaxis following routine sinus surgery and was eventually diagnosed with a pseudoaneurysm of the cavernous internal carotid artery. Endovascular coiling and Onyx (Covidien, Irvine, California, United States) liquid embolization were ultimately used to completely occlude the internal carotid artery with resolution of bleeding; however, the patient had an unexpected late complication of coil extrusion through the pseudoaneurysm sac into the sphenoid sinus and nasal cavity. The endoscopic skull base team safely excised the coils endoscopically without recurrent bleeding. We describe the multidisciplinary operative management of this case of endovascular coil extrusion to increase awareness of this potentially life-threatening complication.

Objectives: To describe the presentation, work-up, and management of patients with metastatic renal cell carcinoma (RCC) to the sinonasal cavity and skull base, and to describe our current treatment algorithm of endoscopic surgical resection followed by radiation therapy. Design: Retrospective review of two recent cases from our institution over a 1-year period, with a relevant review of the literature. Setting: A large regional tertiary care facility. Participants: Consecutive cases of RCC with metastases to the sinonasal cavity presenting to our institution. Main Outcome Measures: Preoperative and postoperative sinonasal outcome test (SNOT)-22 scores, duration of hospital stay, complications, and local disease control Results: Patients in this series underwent preoperative embolization followed by endoscopic resection without complication. Postoperatively they were treated with radiation therapy. They experienced improvement in their SNOT-22 scores and are currently free of local disease. Conclusion: Metastatic RCC to the sinonasal cavity can be safely treated with preoperative embolization followed by endoscopic surgical resection and radiation therapy, which can result in improvement in sinonasal quality of life and is a potential adjunct for local control of disease.

Background: Sphenoid sinus fungal balls (SSFB) are rare entities that can result in serious orbital and intracranial complications. There are few published reports of complications that result from SSFB. Objective: To review the incidence of skull base erosion and orbital or intracranial complications in patients who present with SSFB. Methods: A retrospective review was performed of all the patients with SSFB who were treated at the Massachusetts Eye and Ear Infirmary from 2006 to 2014. Presenting clinical data, radiology, operative reports, pathology, and postoperative course were reviewed. Results: Forty-three patients with SSFB were identified. Demographic data were compared between patients with (39.5%) and those without (61.5%) skull base erosion. Two patients underwent emergent surgery for acute complications of SSFB (one patient with blindness, one patient who had a seizure). Both patients with acute complications had evidence of skull base erosion, whereas no patients with an intact skull base developed an orbital or intracranial complication (p = 0.15). All the patients were surgically managed via an endoscopic approach. Conclusion: SSFBs are rare but may cause significant skull base erosion and potentially severe orbital and intracranial complications if not treated appropriately. Endoscopic sphenoidotomy is effective in treating SSFB and should be performed emergently in patients who presented with associated complications.

Objective: To assess long‐term hearing outcomes in patients treated with third‐generation bisphosphonates for otosclerosis‐related progressive sensorineural hearing loss (SNHL). Study Design Retrospective case series review Methods: We performed a retrospective case series review of patients with otosclerosis and progressive SNHL. Patients were treated with either risedronate or zoledronate after a diagnosis of otosclerosis with a significant SNHL component. Bone conduction pure tone threshold averages (BC‐PTAs) and word recognition scores (WRS) before and after bisphosphonate administration in long‐term follow‐up was analyzed. Significant change in BC‐PTA was defined as greater than 10dB or between 4% and 18% in WRS based on binomial variance. Results: Seven patients were identified and 14 ears met inclusion criteria. Three patients were female and the mean age was 48.3 ± 10.3 years. The mean duration between treatment with bisphosphonate administration and long‐term post‐treatment follow‐up audiometry was 87.6 ± 18.3 months, with a range of 61.6 to 109.1 months and median of 89.2 months. Analysis using BC‐PTA and WRS demonstrated that 11 ears remained stable while 2 improved and 1 worsened. No patient experienced any major complication as the result of bisphosphonate therapy. Conclusion: Treatment with third‐generation bisphosphonates is associated with stability in otosclerosis‐related sensorineural hearing over 5‐ to 9‐year period. These results suggest that such medications may prevent the progression of SNHL in patients with otosclerosis. Level of Evidence 4 (Case series).