Oncocytic carcinoma of the thyroid: Conclusions from a 20-year patient cohort.

BACKGROUND: Oncocytic carcinoma (OCA) was recently reclassified as a distinct differentiated thyroid carcinoma (DTC). Given its rarity, OCA studies are limited. This study describes the characteristics of OCA in a 20-year cohort. METHODS: Retrospective analysis of patients with OCA at a single tertiary care hospital from 2000 to 2021. RESULTS: Fifty-one OCA patients (22M:29F) were identified. The mean age at diagnosis was 60.3 years; 90% presented as palpable mass; 24% had a family history of thyroid cancer. None had vocal fold paresis. On ultrasound, most tumors were solid and hypoechoic. FNA (n = 14) showed Bethesda-4 lesions in 93%. All were treated surgically. Histologically, 63% demonstrated angioinvasion, 35% had lymphovascular invasion, and 15% had extrathyroidal extension. Radioactive iodine was used as adjunct therapy in 77%. CONCLUSION: OCA has distinct features that distinguish it from other DTCs, and additional focused studies will help clarify the aggressive nature, treatment options, and prognosis of the disease.

Thyroid cancer necrosis not evident on imaging: A cautionary case series on poorly differentiated thyroid carcinoma diagnosed only on final pathology.

OBJECTIVE: Poorly-differentiated thyroid cancer (PDTC) is a highly aggressive malignancy which is recently defined and understudied in the radiologic literature. Necrosis is a key histopathologic criterion for the diagnosis of PDTC. We illustrate the current difficulty in accurate identification of histopathologic necrosis on preoperative imaging. METHODS: A series of seven patients with the final diagnosis of PDTC from our institution were identified. Multimodality preoperative imaging was analyzed by two head and neck radiologists. Final pathology reports were queried confirming histopathologic evidence of necrosis. RESULTS: Patients presented with a wide range of preoperative imaging features. A consistent imaging appearance confirming necrosis was not identified. All patients were subsequently upstaged to PDTC following final pathological analysis. CONCLUSION: A lack of definitive evidence of necrosis on preoperative imaging does not exclude the possibility of PDTC. We demonstrate the need for further research to establish a clear methodology for the preoperative diagnosis of PDTC.

WPOI-5: Accurately Identified at Intraoperative Consultation and Predictive of Occult Cervical Metastases.

BACKGROUND: Frozen section analysis of oral cancer specimens is ideal for assessing margin distances and depth of invasion (DOI); the latter impacts intraoperative decisions regarding elective neck dissection (END). Here, we show that intraoperative determination of worst pattern of invasion (WPOI), specifically WPOI-5, has a high level of accuracy. This relates to our demonstration herein that WPOI-5 predicts occult cervical metastases (OCM) for pT1 oral squamous carcinoma (OSC). METHODS: The presence of OCM was correlated with WPOI in 228 patients with primary T1/T2/cN0 OSC undergoing resection and END. Concordance between intraoperative and final pathology WPOI determination was assessed on 51 cases of OSC. RESULTS: WPOI-5 predicts OCM in pT1 patients, compared with WPOI-4/WPOI-3 (p < 0.0001). Most pT1 WPOI-5 tumors had DOI of 4-5 mm (24/59 or 40.7%). Only two pT1 WPOI-5 tumors had DOI < 4 mm (3.0 and 3.5 mm). If END were performed in this pT1 cohort for all WPOI-5 OSC patients regardless of DOI, OR all OSC patients with DOI ≥ 4 mm regardless of WPOI, then no OCM would be missed (p = 0.017, 100% sensitivity, 29% specificity, 77% positive predictive value, 23% negative predictive value). With respect to intraoperative WPOI-5 determination, the accuracy, sensitivity, and specificity was 92.16, 73.33, and 100.0%, respectively. CONCLUSIONS: DOI ≥ 4 mm is the dominant predictor of OCM. For the rare WPOI-5 OSC with DOI < 4 mm, it is reasonable to suggest that surgeons perform END. WPOI-5 may be accurately determined intraoperatively. As microscopic instruction is needed to accurately assess WPOI-5, a teaching link is included in this manuscript.

Psychological adjustment to initial treatment for low-risk thyroid cancer: Preliminary study.

BACKGROUND: Low-risk papillary thyroid carcinoma (LR-PTC) can be managed by immediate surgery (IS) or active surveillance (AS). We compare the psychological impact of these treatments on patients with LR-PTC. METHODS: Psychological data were collected over 1 year, with assessments at the time of treatment decision (T1), at 6 months (T2) and 12 months (T3) follow-up. Assessments included 13 validated psychological tools. RESULTS: Of 27 enrolled patients, 20 chose AS and 7 chose IS. The average times to T2 and T3 were 5.7 and 11.3 months, respectively. For both groups, Impact of Events Scale scores significantly decreased (p = 0.001) at T2, and depressive/anxiety symptoms remained low. CONCLUSIONS: This study demonstrates the feasibility of assessing psychological outcomes among patients treated for LR-PTC. Further studies are needed to evaluate the impact of AS versus IS on quality of life and changes that patients experience over longer time periods following their treatment decision.

Utilizing 3D head and neck specimen scanning for intraoperative margin discussions: Proof of concept of our novel approach.

BACKGROUND: The current standard of documenting and communicating frozen section margin results is inefficient. We present a novel method of generating 3D digital models of gross tumor specimens to more clearly visualize histopathological margin results. METHODS: Fifty-five head and neck specimens were scanned and virtually "inked" using 3D software. These 3D specimen maps were displayed in the operating room to provide the surgeon with a real-time specimen-to-defect relationship by which further resections could be guided. RESULTS: Margin results were reported within an average of 34 min using the proposed workflow. The scanner rendered accurate models of specimens that exceeded 3.0 × 3.0 × 3.0 cm. Critical specimen features to consider were size, color, textural complexity, and the presence of discernible anatomic landmarks. CONCLUSIONS: Optical 3D scanning technology can improve the quality of head and neck margin documentation and the efficiency with which results are communicated between the pathologist and surgeon.

Novel technique to prevent or correct open-mouth deformity after bilateral mandibular resection: Use of tensor fascia lata slings.

Open-mouth deformity after mandibular resection presents a challenge for surgeons and patients, contributing to significant functional and cosmetic morbidity. We present an innovative surgical technique to prevent or correct open-mouth deformity. Tensor fascia lata slings were utilized in combination with maxillomandibular fixation to surgically correct or prevent open-mouth deformity in four patients who had previously undergone mandibulectomy or at the time of a contralateral mandibulectomy following prior hemimandibulectomy and reconstruction. Two patients achieved favorable outcomes, including oral competence and improved resting jaw position, while open-mouth deformity could not be corrected for one patient. Another patient remains in the early postoperative period following a secondary procedure to correct open-mouth deformity. Open-mouth deformity is a functional/aesthetic problem that has not been addressed in the literature. Use of tensor fascia lata slings to suspend the mandible is a novel approach to the surgical management of open-mouth deformity.

A novel technique for management of stenosis of the postlaryngectomy stoma with preservation of a functional tracheoesophageal puncture following tracheal resection.

Tracheostomal stenosis following total laryngectomy presents a serious concern for surgeons and patients. Although various techniques correct tracheostomal stenosis, none address an existing tracheoesophageal puncture (TEP). We present an approach to repair tracheostomal stenosis, requiring tracheal resection, and preserve the TEP in a functional position needed for speech rehabilitation. A 62-year-old male with squamous carcinoma of the right true vocal fold underwent a salvage total laryngectomy with placement of a tracheoesophageal prosthesis. Seven years later, he developed tracheal narrowing. A tracheal resection and tracheoplasty were performed to manage the stenosis while maintaining the tracheoesophageal puncture. Six months postoperatively, the patient was well, with no stomal narrowing or trend toward collapse and uneventful healing. He achieved fluent voice easily with stomal occlusion. We present a novel surgical technique to correct for tracheostomal stenosis following total laryngectomy. Our technique allows for TEP preservation to facilitate speech rehabilitation postoperatively.

Does surgical management still play a role in the management of ectopic lingual thyroid: Institutional experience and systematic review of the literature.

OBJECTIVE: Management of ectopic lingual thyroid (ELT) must balance the morbidity of disease with the morbidity of treatment. We investigate clinical outcomes associated with modern treatment options and analyze the role of transoral surgery in the treatment algorithm for ELT. METHODS: This was a retrospective chart review of ELT patients treated at a tertiary care center from 1/1/1979 to 12/31/2019. In addition, a systematic review of the literature from 1979 to 2021 for reports of ELT was performed. Symptoms defined as high-risk were dysphagia, dysphonia, dyspnea, neck swelling, bleeding, and obstructive sleep apnea (OSA). RESULTS: 36 patients within the institutional cohort (IC) and 224 cases in the systematic review (SRC) met criteria. The most common presenting symptoms for both cohorts were dysphagia, globus sensation, and dysphonia. One third of each cohort were hypothyroid, while 3% (n = 1) and 9% (n = 21) of the IC and SRC, respectively, had clinical suspicion of malignancy at presentation. 27% (n = 10) of the IC and 55% (n = 121) of the SRC underwent surgical therapy. There was a 4% (3/72) transoral bleed rate for all patients undergoing transoral surgery. Other reported complications were minor. There were no tracheostomies, and no deaths. Among observed, medically treated, and surgically treated patients, symptoms improved during follow up for 43% or 68% in the IC and SRC, respectively. Following surgery, symptoms improved or resolved for 86% or 93% in the IC and SRC, respectively. CONCLUSIONS: Asymptomatic ELTs with no concern for malignancy can be managed with observation. Patients with mild symptoms or hypothyroidism may trial thyroid suppressive therapy or RAI. RAI can be considered for patients with high-risk symptoms. For patients with symptoms resistant to conservative therapy, concern for malignancy or high-risk symptoms not conducive to RAI, surgery should be considered. Transoral approaches offer acceptable morbidity, and most patients experience resolution of symptoms following this approach.

Cuneiform (cuniculatum) variant of oral squamous cell carcinoma revisited: Presentation of two cases and literature review of a rare entity.

BACKGROUND: Oral carcinoma cuniculatum (OCC) is a rare, locally aggressive tumor, which tends to invade underlying bone. We present two cases of OCC, one demonstrating invasion of the mandible and the other limited to the tongue. METHODS: An 87-year-old male presented with a right-sided buccogingival lesion. Biopsy results led to a diagnosis of verrucous hyperplasia, which was later revised to OCC. Additionally, a 94-year-old female presented with a left lateral tongue lesion. A biopsy showed in-situ and invasive keratinizing squamous cell carcinoma that was later defined as a soft tissue OCC. RESULTS: Following surgical resection, the diagnosis of OCC was established in both patients. We provide a comprehensive literature review of OCC in the context of both case presentations. CONCLUSIONS: OCC is a rare entity, which has a tendency to be misdiagnosed. We emphasize the importance of recognizing the common features of OCC in order to aid in accurate diagnosis.

Assessing Operative Competency in Cochlear Implantation Across the Residency Training Continuum.

OBJECTIVE: To derive normative data based on postgraduate year (PGY) and number of completed otology rotations using a validated tool for cochlear implant surgical competency assessment. STUDY DESIGN: Prospective, blinded validation study. SETTING: Otolaryngology-Head and Neck Surgery residency training program at a tertiary academic medical center. PATIENTS: Fourteen unique otolaryngology trainees, ranging from PGY-1 to PGY-6, were assessed a total of 26 times in a temporal bone laboratory setting using a validated assessment tool for cochlear implantation. MAIN OUTCOME MEASURES: Construct validity, internal consistency, and inter-rater reliability. RESULTS: Inter-rater reliability was calculated using the Cohen's kappa. Overall agreement between raters was excellent (κ = 0.82). Construct validity was supported by a positive association between higher task-based checklist and global rating scale scores with both trainee PGY level and number of otology rotations completed. Time to complete the exercise was inversely associated with PGY level and number of otology rotations completed. CONCLUSION: Herein, we build upon a previously validated instrument to provide a method to reliably assess surgical competency in a core otologic procedure among residents across the training continuum. This data can be used to provide objective feedback on overall and task-specific competency in cochlear implantation to identify surgical deficiencies early on and to guide supportive course correction.

Contextualizing the Modern Epidemiology of Neurofibromatosis Type 2 in an Era of Heightened Detection of Sporadic Vestibular Schwannoma.

OBJECTIVE: Closely paralleling previous radiologic studies, recent population-based prevalence data suggest sporadic vestibular schwannoma (VS) affects over one in 2,000 adults and up to one in 500 in those aged 70 years or older. Attributable to increased utilization of magnetic resonance imaging and screening protocols for asymmetrical sensorineural hearing loss, the increasing detection rate of sporadic VS fundamentally changes the perception of VS as a whole. The primary objective of the current study was to contextualize modern epidemiological trends in neurofibromatosis type 2 (NF2) in light of these recent advancements in the understanding of sporadic VS. STUDY DESIGN: Population-based study. SETTING: Olmsted County, Minnesota. Population size on January 1, 2017: 159,689 people. PATIENTS: All patients with NF2 diagnosed between Jan 1, 1966 and Dec 31, 2016, identified using the Rochester Epidemiology Project. MAIN OUTCOME MEASURES: Incidence, prevalence. RESULTS: Six incident cases were identified over the past 50 years. From 1966 to 2016, the age- and sex-adjusted incidence rate was 0.10 per 100,000 person-years. The incidence rate remained at 0.12 over the most recent decade. Five cases met disease prevalence criteria, and the age- and sex-adjusted prevalence of NF2 on Jan 1, 2017 was 3.1 per 100,000 persons. All prevalent cases were women, and the resultant prevalence among women only was 6.0 per 100,000 persons. CONCLUSIONS: The modern age- and sex-adjusted incidence rate and prevalence of NF2 is 0.10 per 100,000 person-years and 3.1 per 100,000 persons, respectively. In contrast to trends in incidence rates of sporadic VS over the last half-century, this study demonstrates that the incidence of NF2 has remained relatively stable since 1966. This divergence in epidemiological trends is likely attributed to the nature of NF2, with early clinical manifestations resulting in diagnosis regardless of modern advances, whereas the increased incidence of sporadic VS is heavily influenced by improved detection in a greater population of patients with minimally symptomatic or asymptomatic tumors.

The Influence of Vestibular Schwannoma Tumor Volume and Growth on Hearing Loss.

OBJECTIVE: To ascertain the relationship among vestibular schwannoma (VS) tumor volume, growth, and hearing loss. STUDY DESIGN: Retrospective cohort study. SETTING: Single tertiary center. SUBJECTS AND METHODS: Adults with observed VS and serviceable hearing at diagnosis were included. The primary outcome was the development of nonserviceable hearing as estimated using the Kaplan-Meier method. Associations of tumor volume with baseline hearing were assessed using Spearman rank correlation coefficients. Associations of volume and growth with the development of nonserviceable hearing over time were assessed using Cox proportional hazards models and summarized with hazard ratios (HRs). RESULTS: Of 230 patients with VS and serviceable hearing at diagnosis, 213 had serial volumetric tumor data for analysis. Larger tumor volume at diagnosis was associated with increased pure-tone average (PTA) (P < .001) and decreased word recognition score (WRS) (P = .014). Estimated rates of maintaining serviceable hearing at 6 and 10 years following diagnosis were 67% and 49%, respectively. Larger initial tumor volume was associated with development of nonserviceable hearing in a univariable setting (HR for 1-cm3 increase: 1.36, P = .040) but not after adjusting for PTA and WRS. Tumor growth was not significantly associated with time to nonserviceable hearing (HR, 1.57; P = .14), although estimated rates of maintaining serviceable hearing during observation were poorer in the group that experienced growth. CONCLUSION: Larger initial VS tumor volume was associated with poorer hearing at baseline. Larger initial tumor volume was also associated with the development of nonserviceable hearing during observation in a univariable setting; however, this association was not statistically significant after adjusting for baseline hearing status.

Manifestations of Skull Base IgG4-Related Disease: A Multi-Institutional Study.

OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. CONCLUSIONS: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2574-2580, 2020.

Durability of Hearing Preservation Following Microsurgical Resection of Vestibular Schwannoma.

OBJECTIVE: To ascertain long-term hearing outcomes in patients with serviceable hearing following microsurgical resection of sporadic vestibular schwannoma (VS). STUDY DESIGN: Retrospective cohort. SETTING: Tertiary academic referral center. PATIENTS: Forty-three adult subjects with unilateral sporadic VS who had serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] class A or B) on initial postoperative audiogram following microsurgical resection between 2003 and 2016 with a minimum of two postoperative audiograms available for review. INTERVENTION: Surgical treatment with a retrosigmoid or middle cranial fossa approach. MAIN OUTCOME MEASURE: Rate of maintaining serviceable hearing, as estimated using the Kaplan-Meier method, in accordance with the 1995 and 2012 AAO-HNS guidelines on reporting hearing outcomes. RESULTS: The median immediate postoperative pure-tone average (PTA) and word recognition score (WRS) were 31 dB and 95%, respectively. At last follow-up, the median PTA was 38 dB with a median change of 5 dB from initial postoperative audiogram, and the median WRS was 90% with a median change of 0% from initial postoperative audiogram. Eight patients developed non-serviceable hearing at a median of 4.1 years following microsurgical resection (interquartile range, 2.9-7.0). The median duration of hearing follow-up for the 35 patients who maintained serviceable hearing was 3.1 years (interquartile range, 2.2-7.5). Tumor control was achieved in 41 (95%) patients. The rate of maintaining serviceable hearing at 5 years was 81%. CONCLUSION: Microsurgical resection provides excellent tumor control and durable long-term hearing in those with AAO-HNS class A or B hearing postoperatively. The paradigm of proactive microsurgical resection-when the tumor is small and hearing is good-hinges on the surgeon's ability to preserve residual hearing in a very high percentage of cases at or near preoperative hearing levels to maintain an advantage over conservative observation with regard to long-term hearing preservation.

Rate of Initial Hearing Loss During Early Observation Predicts Time to Non-Serviceable Hearing in Patients With Conservatively Managed Sporadic Vestibular Schwannoma.

OBJECTIVE: To date, prediction models for estimating risk of acquiring non-serviceable hearing in subjects with observed vestibular schwannoma (VS) have evaluated outcomes primarily based on features at initial diagnosis. Herein, we evaluate the association of rate of hearing decline during the initial period of observation with time to non-serviceable hearing. If significant, rate of hearing decline may inform decision making after an introductory period of observation. SETTING: Two tertiary care centers. PATIENTS: VS patients with serviceable hearing who underwent at least three audiograms and two magnetic resonance imaging (MRI) studies before intervention or being lost to follow-up. The rate of change in pure-tone average (PTA) and word recognition score (WRS) was calculated as the score from the second audiogram minus the score from the first audiogram, divided by the duration in months between the two. MAIN OUTCOME MEASURE(S): Serviceable hearing, defined as PTA ≤50 dB HL and WRS ≥50%. RESULTS: Among 266 patients meeting inclusion criteria, 52 developed non-serviceable hearing at last follow-up. Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years were 97% (95-100; 206), 78% (72-85; 98), 68% (60-77; 39), 60% (50-73; 17), and 44% (29-67; 2), respectively. In a univariable setting, each 1 dB increase per month in the rate of initial PTA change was associated with a 96% increased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 1.96; 95% CI 1.44-2.68; p < 0.001). Each 1% increase per month in the rate of initial WRS change was associated with a decreased likelihood of acquiring non-serviceable hearing (hazard ratio [HR] 0.79; 95% confidence interval [CI] 0.66-0.94; p = 0.009). After multivariable adjustment, both rate of PTA change (HR 2.42; 95% CI 1.72-3.41; p < 0.001) and rate of WRS change (HR 0.81; 95% CI 0.67-0.99; p = 0.043) remained statistically significantly associated with time to non-serviceable hearing. CONCLUSION: Rate of early PTA and WRS decline during the initial period of observation are significantly associated with time to development of non-serviceable hearing. This information may facilitate accurate patient counseling and inform decision-making regarding prospective disease management.

Cochlear Implantation: An Overview.

A cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

Hearing Outcomes in Conservatively Managed Vestibular Schwannoma Patients With Serviceable Hearing.

OBJECTIVE: To characterize the risk of progression to nonserviceable hearing in patients with sporadic vestibular schwannomas (VS) who elect initial observation. STUDY DESIGN: Retrospective case series. SETTING: Two tertiary care centers. PATIENTS: VS patients with serviceable hearing who underwent at least two audiograms and two MRI studies before intervention or loss to follow-up. MAIN OUTCOME MEASURE(S): Serviceable hearing, defined as the pure tone average ≤ 50 dB HL and word recognition score ≥ 50%. RESULTS: Four-hundred sixty-six patients (median age of 57 yr and median tumor diameter of 7.3 mm) had serviceable hearing at presentation and were followed for a median of 2.3 years (IQR 1.0 - 4.0). Kaplan-Meier estimated rates of maintaining serviceable hearing (95% CI; number still at risk) at 1, 3, 5, 7, and 10 years following diagnosis were 94% (91-96; 357), 77% (73-82; 172), 66% (60-73; 81), 56% (49-65; 31), and 44% (33-59; 10), respectively. Each 10-dB increase in pure-tone averages at diagnosis was associated with a 2-fold increased likelihood of developing nonserviceable hearing (hazard ratio 2.07; p < 0.001). Each 10% decrease in word recognition score was associated with a 1.5-fold increased likelihood of developing nonserviceable hearing (hazard ratio 1.48; p < 0.001). CONCLUSIONS: Among patients with sporadic VS, good baseline word recognition score and low pure-tone average are jointly associated with maintenance of serviceable hearing. These data may be used to guide patient counseling and optimize management.