Citations

Objectives: To describe the clinical presentation, radiological features, and outcome of patients with plasmacytoma of the temporal bone. Methods: Multicenter retrospective case series of all patients diagnosed with plasmacytoma of the temporal bone between 1990 and 2015. Comprehensive literature review of all previously published cases. Results: A total of seven patients (average age, 57.3 yr; 57% women) met inclusion criteria. Three (43%) had a known history of multiple myeloma (MM). The most common symptoms at presentation included otalgia, headache, and dizziness (43%, each). Five (71%) presented with a vascular appearing middle ear mass visible on otoscopy. Four of these patients did not have an established history of MM and were referred with presumptive diagnoses of paraganglioma or endolymphatic sac tumor (ELST). The average maximum tumor diameter was 3.7 cm (median, 3.5 cm; range, 0.9–6.6 cm) and the most commonly involved temporal bone subsites were the middle ear and mastoid (71% each). MM was ultimately diagnosed in six cases (86%). In addition to the new cases presented herein, 18 previously published reports were analyzed. In the 25 aggregate cases, 44% of cases were associated with MM, and the mastoid (72%) and middle ear (53%) were the most commonly involved temporal bone subsites. Conclusion: Plasmacytoma of the temporal bone is rare, and the clinical presentation can closely mimic that of other primary temporal bone tumors. In patients with an established history of MM, it is imperative to have a high index of suspicion for temporal bone plasmacytoma, even when radiological features suggest an alternate diagnosis.

ObjectivesTo elucidate the clinical behavior, treatment, and outcomes of tenosynovial giant cell tumors (TGCT) involving the temporomandibular joint (TMJ) and adjacent temporal bone.Study DesignRetrospective case series with histopathologic review.MethodsA retrospective chart review was performed identifying and collecting data from all cases of TGCT involving the TMJ and adjacent temporal bone that were treated at the authors' center between January 1960 and December 2015.ResultsEleven histopathologically confirmed cases met inclusion criteria. The median age at diagnosis was 49 years, eight patients were men, and the median follow‐up was 116 months. Computed tomographic (CT) imaging revealed a lytic expansile mass centered on the TMJ. Magnetic resonance imaging (MRI) most commonly exhibited hypointense signal on precontrast T1‐ and T2‐weighted sequences and variable postcontrast enhancement. The median delay in diagnosis was 24 months, and the most common presenting symptoms were hearing loss and pain. All patients underwent surgical resection, eight receiving gross total removal, one receiving near total removal, and two patients from early in the series receiving subtotal resection with neoadjuvant or adjuvant radiation. Histopathological review of surgical specimens revealed chondroid metaplasia in seven tumors. Eight of nine cases receiving gross total or near total resection have no evidence of recurrence to date.ConclusionsTGCT of the TMJ and temporal bone are rare and locally aggressive tumors that commonly present with nonspecific symptoms. A careful review of CT and MRI followed by early biopsy is critical in establishing an accurate diagnosis and facilitating appropriate treatment. TGCT of the TMJ more commonly contain chondroid metaplasia when compared to TGCT at other anatomic locations. Gross total resection is achievable in most cases and offers long‐term cure. Radiation may be considered for recurrent disease or adjuvant therapy following subtotal resection.Level of Evidence4. Laryngoscope, 127:2340–2346, 2017

Objective: Fibrovascular polyps of the hypopharynx and esophagus are rare, with few case reports in the literature. In this article, we present our institutional experience with a focus on airway and surgical management. Study Design: Case series. Setting: Tertiary academic institution. Methods: A retrospective review was conducted of 4 patients that presented to a tertiary medical center with fibrovascular polyps between 1990 and 2012. Patient demographics, clinical presentation, diagnostic studies, and surgical approaches were reviewed. A review of the published literature was also performed. Results: The average age at presentation was 72 years (range, 59-85 years). Among the 4 patients, 2 presented with airway compromise requiring tracheotomy. All patients had removal of the polyp shortly after presentation; 2 underwent transcervical approaches with lateral pharyngotomy/esophagotomy, and the other 2 had endoscopic removal. The polyps arose from the hypopharynx in 3 patients and upper esophagus in 1. Three patients had complete resolution of their symptoms and remained disease free. One patient had recurrence of the polyp 2 years later and is currently being observed. Conclusion: Fibrovascular polyps are rare tumors of the hypopharynx/esophagus that present in older adults. Although benign, they can cause life-threatening airway compromise that may necessitate tracheotomy. We present 4 cases of fibrovascular polyps and discuss our evolving surgical management, including endoscopic removal.

ObjectiveDemonstrate the association of radiographic dural enhancement with increased tumor adherence at the porus acusticus, which may influence completeness of resection and facial nerve outcome.Study DesignCase series with chart review.SettingAcademic referral center.Subjects and MethodsA total of 205 consecutive patients with histopathologically confirmed vestibular schwannoma (VS) were evaluated. Patients with tumors exhibiting dural enhancement on postgadolinium T1‐weighted magnetic resonance imaging were identified and compared with matched controls. Completeness of resection, intraoperative details, and facial nerve outcomes were analyzed.ResultsExcluding strictly intracanalicular tumors (n = 32, 16%) and those with NF2 (n = 10, 4.9%), the presence of dural enhancement was radiographically confirmed in 16 (9.8%) cases. Paired analysis did not reveal significant baseline differences between cases and controls. Subtotal resection was performed in 5 (31%) of the 16 patients with tumors exhibiting dural enhancement, in contrast to 1 (3%) of the matched controls (P =. 01). Four (25%) demonstrated increased tumor adherence at the porus acusticus intraoperatively, compared with 1 control (3%, P =. 04). Long‐term facial nerve function was similar between cases and controls (81% vs 84% House‐Brackmann I‐II function, P = 1.00).ConclusionDural enhancement is present in approximately 10% of extracanalicular VS. Dural enhancement at the porus acusticus may represent hypervascularity, dural reaction, or infiltration, and portends increased tumor adherence and greater likelihood of subtotal resection to preserve facial nerve function. To our knowledge, this is the first series that reports the prevalence of this phenomenon in VS and the potential surgical implications. Recognition preoperatively may be valuable toward patient counseling.

Abstract Microtubule-targeting agents (MTA) have been employed in the treatment of many cancers for decades. BAL101553 is a highly soluble prodrug of BAL27862, a novel, small molecule, microtubule-depolymerizing agent that induces tumor cell death by activating the ‘spindle assembly checkpoint’. Given intravenously or orally, the drug penetrates the brain and has anti-cancer activity in diverse tumor models refractory to standard MTA or radiotherapy (RT). In this study, BAL101553 was evaluated in orthotopic xenografts from 16 GBM PDX models; 7 of 16 lines demonstrated significant (p<0.01) increases in median survival with BAL101553 versus placebo (range in median survival extension 24-87%). The combination of BAL101553 with conventional therapies for GBM (RT and temozolomide (TMZ) was then evaluated in select lines. In the MGMT methylated GBM12 line, combination of RT with TMZ increased survival compared to placebo (median survival 80 days vs. 23 days, respectively; p<0.001). Extended BAL101553 monotherapy provided a short but significant extension in survival (median survival 31 days, p<0.001), while extended BAL101553 dosing during and after RT/TMZ (median survival 85 days) did not extend survival relative to RT/TMZ alone (p = 0.56). In contrast, in the MGMT unmethylated GBM6 line, combination of RT and extended BAL101553 increased survival (median 90 days, p<0.001) relative to either treatment alone (median survival BAL101553 63 days; RT 69 days) or placebo (46 days). Additionally, the combination of BAL101553 with TMZ (median survival 70 days) was more effective than TMZ alone (median survival 60 days; p = 0.009). Consistent with the unmethylated MGMT status, the TMZ/RT combination (median survival 66 days) was similar to RT alone (p = 0.62), but the combination of extended BAL101553 with RT/TMZ (median survival 101 days; p<0.001 compared to other combination groups) was significantly more effective. To further evaluate whether BAL101553 is a true radiosensitizer, a second GBM6 study was performed. Also here, combination of RT (20Gy, 2wks) with extended BAL101553 dosing (median survival 66 days) significantly extended survival compared to RT alone (median survival 54 days; p = 002). Interestingly, when BAL101553 dosing was limited to 2 weeks with RT, there was no increase in median survival (58 days; p = 0.16). To evaluate effects on tumor repopulation during RT, the efficacy of an extended RT schedule (36 Gy, 6 wks) with or without 6 weeks of BAL101553 was evaluated. In this case, BAL101553 given during the RT schedule (median survival 78 days) extended median survival as compared to RT alone (61 days; p<0.001). Collectively, these data demonstrate that BAL101553 has broad single agent activity across a panel of GBM PDX models and suggests that combination with RT/TMZ therapy may provide additional benefits for survival extension. Citation Format: Ann C. Mladek, Jenny L. Pokorny, Heidi Lane, Felix Bachmann, Mark A. Schroeder, Katrina K. Bakken, Brett L. Carlson, Paul A. Decker, Jeanette E. Eckel-Passow, Jann N. Sarkaria. The novel tubulin-binding ‘tumor checkpoint controller’ BAL101553 has anti-cancer activity alone and in combination treatments across a panel of GBM patient-derived xenografts. [abstract]. In: Proceedings of the 107th Annual Meeting of the American Association for Cancer Research; 2016 Apr 16-20; New Orleans, LA. Philadelphia (PA): AACR; Cancer Res 2016;76(14 Suppl):Abstract nr 4781.

ABSTRACTBACKGROUND/PURPOSEBrain herniation into presumed arachnoid granulations (BHAG) is an increasingly recognized entity. Though it has previously been described as isointense to brain matter on magnetic resonance imaging (MRI), we have encountered 21 cases in our clinical practice, many of which have signal abnormality in the herniated or adjacent parenchyma. We performed a systematic analysis on these cases to further understand the clinical significance.METHODSThis is a retrospective analysis of cases collected from our clinical MRI practice. Each case was evaluated for location, size, lobe of origin, associated signal abnormality, symptoms, and the presence or absence of intracranial hypertension.RESULTSTwenty‐one cases of BHAG identified in 16 patients. Seven cases (7/21, 33%) demonstrated signal abnormality in the herniated or underlying parenchyma. On serial imaging, the size of herniation either fluctuated (2/10, 20%) or stayed the same (8/10, 80%). The associated signal abnormalities increased (1/5, 20%), decreased (1/5, 20%), or remained stable (3/5, 60%). Four patients (4/16, 25%) had signs/symptoms that could be associated with the BHAG. Ten patients (10/16, 63%) had either imaging findings or clinical signs that could be associated with intracranial hypertension.CONCLUSIONIn contrast to previously reported cases, our study demonstrates that BHAG can be associated with MRI signal abnormalities of the herniated and adjacent brain. Symptoms are typically absent, though may be present in a small subset of patients, regardless of the presence of signal change or encephalomalacia of the involved brain. Indirect evidence of intracranial hypertension is present in a majority of patients but completely lacking in others.