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Background  Skull base chondrosarcomas (CSA) are difficult tumors to cure and there is little data regarding salvage therapy. Objective  This study aims to identify presentation and treatment-related factors which impact the progression free survival (PFS) and disease specific survival (DSS) for recurrent CSA, and to identify salvage treatment factors associated with successful restoration to the natural history following primary treatment. Methods  This single-institution retrospective review included patients with recurrent/progressive CSA over a 25-year period. Survival analysis for factors impacting PFS and DSS was performed. Salvage treatment factors associated with achieving PFS ≥newly diagnosed median PFS were identified using univariate statistics. Analysis was performed on first recurrences and all recurrences combined. Results  A total of 47 recurrence/progression events were analyzed from 17 patients (median two events/patient, range = 1–8). The overall PFS and DSS for the initial recurrence was 32 (range = 3–267) and 79 (range = 3–285) months, respectively. Conventional grade III or mesenchymal histology significantly predicted shorter PFS and DSS (p  < 0.0001). After stratification by histology, previous radiation predicted shorter PFS for low-grade tumors (p  = 0.009). Gross total resection (GTR) after a first time recurrence was significantly associated with successful salvage treatment (p  < 0.05); however, this was rare. Conclusion  In this series, high grade histology and prior radiation treatment negatively impacted salvage treatment outcomes, while GTR was associated with restoration to natural history following primary treatment. Careful consideration of histology, systemic disease status, previous treatments, and the anatomic extent of the skull base disease can optimize the outcomes of salvage intervention. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: Examine the presentation and management characteristics of seven patients with melanoma of the external auditory canal (EAC).Study Design: Retrospective case series and review of the relevant literature.Methods: Records of seven patients from 2003 to 2017 with melanoma of the EAC were reviewed for characteristics of presentation, subsequent management, and outcomes. A thorough review of relevant literature is presented.Results: The median age is 52 years, with four females. The average Breslow depth was 3.6 mm, with five patients having a Clark level IV or greater on presentation. Six patients underwent lateral temporal bone resection, and one patient underwent wide local excision of the cartilaginous canal. Sentinel lymph node biopsy (SLNB) was performed in three patients. Three patients experienced distant recurrence an average of 20 months following primary therapy. Median follow-up was 21 months. At last follow-up, four were free of disease, one had active disease, and two were deceased from melanoma.Conclusions: This is the largest series and the first to report the use of SLNB for patients with EAC melanoma in the peer-reviewed literature. Patients with external auditory canal melanoma present with higher Breslow thickness and stage relative to all external ear melanomas. Management should include wide local excision, which entails lateral temporal bone resection when the bony ear canal is involved. SLNB has a critical role in identifying patients with early metastatic disease. Postoperative radiation therapy should be considered for patients with high-risk features to reduce the risk of locoregional relapse. Chemotherapy, and especially immunotherapy, has an emerging role for this disease.Level Of Evidence: 4 Laryngoscope, 131:165-172, 2021. [ABSTRACT FROM AUTHOR]

Background: Recurrent immunotherapy‐induced audiovestibular toxicity despite cessation of therapy has not been reported. Methods: We report the first case of recurrent audiovestibular toxicity following immune‐checkpoint inhibitor (ICI) therapy. The patient was seen with sudden bilateral hearing loss and disequilibrium. After ruling out other etiologies, he was diagnosed with audiovestibular and neurological immune‐related adverse events (irAEs). He received systemic steroids, with significant hearing and balance recovery. Over the following 4 months, he experienced two other episodes of sudden bilateral hearing loss despite ICIs cessation. The second episode was treated with oral steroids, and hearing improved. On the third episode, he received oral and intratympanic steroids, and he was started on infliximab. Results: Audiogram 8 months following the last recurrence showed hearing improvement and stability. Conclusion: Immunotherapy‐induced ototoxicity may recur despite therapy cessation. High dose steroids remain the mainstay of treatment. If audiovestibular irAEs recur despite multiple courses of steroids, immunosuppressive agents may be considered. [ABSTRACT FROM AUTHOR]

Background: COVID‐19 pandemic has strained human and material resources around the world. Practices in surgical oncology had to change in response to these resource limitations, triaging based on acuity, expected oncologic outcomes, availability of supportive resources, and safety of health care personnel. Methods: The MD Anderson Head and Neck Surgery Treatment Guidelines Consortium devised the following to provide guidance on triaging head and neck cancer (HNC) surgeries based on multidisciplinary consensus. HNC subsites considered included aerodigestive tract mucosa, sinonasal, salivary, endocrine, cutaneous, and ocular. Recommendations Each subsite is presented separately with disease‐specific recommendations. Options for alternative treatment modalities are provided if surgical treatment needs to be deferred. Conclusion: These guidelines are intended to help clinicians caring for patients with HNC appropriately allocate resources during a health care crisis, such as the COVID‐19 pandemic. We continue to advocate for individual consideration of cases in a multidisciplinary fashion based on individual patient circumstances and resource availability. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: To review the presenting features, treatment, and outcomes for patients with basal cell carcinoma (BCC) involving the temporal bone or external auditory canal (EAC).Study Design: Retrospective case series.Methods: Presenting characteristics, treatment strategies, and outcomes for patients with BCC involving the temporal bone or EAC were reviewed. Main outcome measures included rates of overall and disease-free survival at 5 years.Results: Forty-two patients met the inclusion criteria (mean age, 67 years). The most common presenting symptoms were hearing loss (15 patients, 36%) and otorrhea (11 patients, 26%). The 5-year overall survival rate was 78%, and the 5-year disease-free survival rate was 77%. Patients who had undergone surgery elsewhere and presented with facial weakness had significantly worse overall survival (P = .004). Ten patients (24%) underwent sacrifice of at least a portion of the facial nerve at the time of their initial procedure at our institution. Two patients (5%) developed regional nodal disease. There was a trend toward better disease-free survival for patients who received adjuvant radiation therapy after surgery at our institution (P = .06).Conclusions: BCC originating from or extending to the temporal bone can exhibit a wide range of behavior, ranging from superficial lesions readily managed with surgery to extensive locally invasive tumors with propensity for recurrence, treated with multimodality therapy. BCC may be associated with better prognosis than other temporal bone malignancies.Level Of Evidence: 4. Laryngoscope, 128:1425-1430, 2018. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: To compare the complication rate for osseointegrated hearing aids (OIHA) in patients with or without irradiation.Study Design: Retrospective case review.Methods: We studied patients with OIHAs implanted between January 1, 2005, and July 15, 2013 in a tertiary university center with a referral otology and neurotology practice. Demographics, history of oncologic surgery, follow-up length after OIHA implantation, radiation history and dosage, postoperative complications, and chronologic relationship between oncologic resection, OIHA implantation, and irradiation were reviewed to collect information. Soft tissue complications were graded according to a modified Holgers classification.Results: The study included 48 patients. Twenty-nine patients (32 implants) did not undergo radiotherapy and 19 patients (19 implants) did. In the radiotherapy group, six patients had OIHAs implanted before radiotherapy, and 13 had OIHAs implanted in irradiated bone. Of these 13 patients, one had OIHA implanted during primary oncologic surgery; 11 had OIHA implanted during secondary surgery; and one patient did not have oncologic surgery. Patients with both OIHA implantation and radiotherapy had more complications than patients without radiotherapy (31.6% vs. 24.1%, P > 0.05) and more major complications than patients without radiotherapy (26.3% vs. 3.4%, P > 0.05). Patients with OIHAs implanted before radiotherapy did not have any complications. There were significantly fewer and less severe complications in patients with OIHAs implanted during primary oncologic resection than in patients with OIHAs implanted secondarily (0/8 vs. 8/11, P < 0.05).Conclusions: The rate and severity of complications of OIHAs can be minimized by implanting the device before irradiation, ideally at the time of primary oncologic surgery.Level Of Evidence: 4. Laryngoscope, 126:1187-1192, 2016. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis To examine our experience with surgically treated radiation-associated malignancy (RAM) of the temporal bone. Study Design Retrospective chart review. Methods Thirteen patients treated surgically for temporal-bone RAM during 1999 to 2012 were identified. Demographics, symptoms at presentation with RAM, interval between initial radiotherapy and diagnosis of RAM, pathologic features of RAM, treatment, and recurrence and survival were obtained from medical records. Results Ten patients had squamous cell carcinoma; three had sarcoma. The mean period between completion of radiotherapy and diagnosis of RAM was 13.38 years (range, 4.5-33.3 years). Three-year overall and disease-free survival rates were 59.8% and 42.3%, respectively. Seven patients (54%) had RAM limited to the external auditory canal (LEAC); six had RAM extending beyond it (EEAC). The mean age was 66.5 years for LEAC patients versus 41.1 years for EEAC patients ( P = .0161). Compared to patients with EEAC disease, patients with LEAC disease were more likely to have negative margins (100% vs. 33%; P = .021) and less likely to have postoperative adjuvant therapy (0% vs. 67%; P = .0209). The 3-year disease-specific survival was significantly better for patients with LEAC disease versus EEAC (log rank P = .023). Conclusions To our knowledge, this is the largest reported series of surgically treated RAM of the ear canal or temporal bone. This study highlights the need for vigilance for RAM in patients treated with head and neck radiation. Level of Evidence 4. Laryngoscope, 125:1198-1204, 2015 [ABSTRACT FROM AUTHOR]

Radiation therapy (RT) to the head and neck (H&N) region is critical in the management of various pediatric malignancies; however, it may result in late toxicity. This comprehensive review from the Pediatric Normal Tissue Effects in the Clinic (PENTEC) initiative focused on salivary dysfunction and dental abnormalities in survivors who received RT to the H&N region as children. This systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method. Of the 2,164 articles identified through a literature search, 40 were included in a qualitative synthesis and 3 were included in a quantitative synthesis. The dose-toxicity data regarding salivary function demonstrate that a mean parotid dose of 35 to 40 Gy is associated with a risk of acute and chronic grade ≥2 xerostomia of approximately 32% and 13% to 32%, respectively, in patients treated with chemo-radiation therapy. This risk increases with parotid dose; however, rates of xerostomia after lower dose exposure have not been reported. Dental developmental abnormalities are common after RT to the oral cavity. Risk factors include higher radiation dose to the developing teeth and younger age at RT. This PENTEC task force considers adoption of salivary gland dose constraints from the adult experience to be a reasonable strategy until more data specific to children become available; thus, we recommend limiting the parotid mean dose to ≤26 Gy. The minimum toxic dose for dental developmental abnormalities is unknown, suggesting that the dose to the teeth should be kept as low as possible particularly in younger patients, with special effort to keep doses <20 Gy in patients <4 years old. [ABSTRACT FROM AUTHOR]

Infratemporal fossa (ITF) tumors are difficult to access surgically due to anatomical constraints. Moreover, aggressive ITF carcinomas and sarcomas necessitate aggressive treatment strategies that, along with tumor-related symptoms, contribute to decreases in patient performance status. To assess factors that predict postoperative performance in patients undergoing surgery for ITF tumors. We reviewed medical records for all patients surgically treated for an ITF malignancy between January 1, 1999, and December 31, 2017, at our institution. We collected patient demographics, preoperative performance, tumor stage, tumor characteristics, treatment modalities, pathological data, and postoperative performance data. The 5-year survival rate was 62.2%. Higher preoperative Karnofsky Performance Status (KPS) score (n = 64; p < 0.001), short length of stay (p = 0.002), prior surgery at site (n = 61; p = 0.0164), and diagnosis of sarcoma (n = 62; p = 0.0398) were predictors of higher postoperative KPS scores. Percutaneous endoscopic gastrostomy (PEG) (n = 9; p = 0.0327), and tracheostomy tube placement (n = 20; p = 0.0436) were predictors of lower postoperative KPS scores, whereas age at presentation (p = 0.72), intracranial tumor spread (p = 0.8197), and perineural invasion (n = 40; p = 0.2195) were not. Male patients and patients with carcinomas showed the greatest decreases in KPS scores between pretreatment and posttreatment. Higher preoperative KPS score and short length of stay were the best predictors of higher postoperative KPS scores. This work provides treatment teams and patients with better information on outcomes for shared decision-making. [ABSTRACT FROM AUTHOR]

Background: The management of sub-totally resected sporadic vestibular schwannoma (VS) may include observation, re-resection or irradiation. Identifying the optimal choice can be difficult due to the disease's variable progression rate. We aimed to define an immune signature and associated transcriptomic fingerprint characteristic of rapidly-progressing VS to elucidate the underpinnings of rapidly progressing VS and identify a prognostic model for determining rate of progression. Methods: We used multiplex immunofluorescence to characterize the immune microenvironment in 17 patients with sporadic VS treated with subtotal surgical resection alone. Transcriptomic analysis revealed differentially-expressed genes and dysregulated pathways when comparing rapidly-progressing VS to slowly or non-progressing VS. Results: Rapidly progressing VS was distinctly enriched in CD4+, CD8+, CD20+, and CD68+ immune cells. RNA data indicated the upregulation of anti-viral innate immune response and T-cell senescence. K − Top Scoring Pair analysis identified 6 pairs of immunosenescence-related genes (CD38-KDR, CD22-STAT5A, APCS-CXCR6, MADCAM1-MPL, IL6-NFATC3, and CXCL2-TLR6) that had high sensitivity (100%) and specificity (78%) for identifying rapid VS progression. Conclusion: Rapid progression of residual vestibular schwannoma following subtotal surgical resection has an underlying immune etiology that may be virally originating; and despite an abundant adaptive immune response, T-cell immunosenescence may be associated with rapid progression of VS. These findings provide a rationale for clinical trials evaluating immunotherapy in patients with rapidly progressing VS. [ABSTRACT FROM AUTHOR]