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OBJECTIVE Carotid body tumors (CBTs) are rare, slow-growing neoplasms derived from the parasympathetic paraganglia of the carotid bodies. Although inherently vascular lesions, the role of preoperative embolization prior to resection remains controversial. In this report, the authors describe an institutional series of patients with CBT successfully treated via resection following preoperative embolization and compare the results in this series to previously reported outcomes in the treatment of CBT. METHODS All CBTs resected between 2013 and 2019 at a single institution were retrospectively identified. All patients had undergone preoperative embolization performed by interventional neuroradiologists, and all had been operated on by a combined team of cerebrovascular neurosurgeons and otolaryngology–head and neck surgeons. The clinical, radiographic, endovascular, and perioperative data were collected. All procedural complications were recorded. RESULTS Among 22 patients with CBT, 63.6% were female and the median age was 55.5 years at the time of surgery. The most common presenting symptoms included a palpable neck mass (59.1%) and voice changes (22.7%). The average tumor volume was 15.01 ± 14.41 cm3. Most of the CBTs were Shamblin group 2 (95.5%). Blood was predominantly supplied from branches of the ascending pharyngeal artery, with an average of 2 vascular pedicles (range 1–4). Fifty percent of the tumors were embolized with more than one material: polyvinyl alcohol, 95.5%; Onyx, 50.0%; and N-butyl cyanoacrylate glue, 9.1%. The average reduction in tumor blush following embolization was 83% (range 40%–95%). No embolization procedural complications occurred. All resections were performed within 30 hours of embolization. The average operative time was 173.9 minutes, average estimated blood loss was 151.8 ml, and median length of hospital stay was 4 days. The rate of permanent postoperative complications was 0%; 2 patients experienced transient hoarseness, and 1 patient had medical complications related to alcohol withdrawal. CONCLUSIONS This series reveals that endovascular embolization of CBT is a safe and effective technique for tumor devascularization, making preoperative angiography and embolization an important consideration in the management of CBT. Moreover, the successful management of CBT at the authors’ institution rests on a multidisciplinary approach whereby endovascular surgeons, neurosurgeons, and ear, nose, and throat–head and neck surgeons work together to optimally manage each patient with CBT.

Fanconi anaemia (FA), a model syndrome of genome instability, is caused by a deficiency in DNA interstrand crosslink repair resulting in chromosome breakage1–3. The FA repair pathway protects against endogenous and exogenous carcinogenic aldehydes4–7. Individuals with FA are hundreds to thousands fold more likely to develop head and neck (HNSCC), oesophageal and anogenital squamous cell carcinomas8 (SCCs). Molecular studies of SCCs from individuals with FA (FA SCCs) are limited, and it is unclear how FA SCCs relate to sporadic HNSCCs primarily driven by tobacco and alcohol exposure or infection with human papillomavirus9 (HPV). Here, by sequencing genomes and exomes of FA SCCs, we demonstrate that the primary genomic signature of FA repair deficiency is the presence of high numbers of structural variants. Structural variants are enriched for small deletions, unbalanced translocations and fold-back inversions, and are often connected, thereby forming complex rearrangements. They arise in the context of TP53 loss, but not in the context of HPV infection, and lead to somatic copy-number alterations of HNSCC driver genes. We further show that FA pathway deficiency may lead to epithelial-to-mesenchymal transition and enhanced keratinocyte-intrinsic inflammatory signalling, which would contribute to the aggressive nature of FA SCCs. We propose that the genomic instability in sporadic HPV-negative HNSCC may arise as a result of the FA repair pathway being overwhelmed by DNA interstrand crosslink damage caused by alcohol and tobacco-derived aldehydes, making FA SCC a powerful model to study tumorigenesis resulting from DNA-crosslinking damage.

Most head and neck paragangliomas (PGLs) are biochemically silent and often present with recurrence and metastases in association with hereditary syndromes. Whole-body functional imaging is increasingly used to detect tumor extent and guide treatment planning of PGLs. [68Ga]-DOTATATE, which targets somatostatin receptor 2 (SSTR2) overexpression, has emerged as a sensitive functional imaging modality in PGLs. We present a patient with metastatic glomus caroticum PGL in whom [68Ga]-DOTATATE PET/MRI provided a more accurate characterization of metastatic extent, as compared to gadolinium-enhanced MRI of the neck and whole body [18F]-FDG PET/CT. We then review the current literature and discuss the imaging implications of [68Ga]-DOTATATE PET/MRI in PGLs.

The purpose of this study is to assess the efficacy of lymphoscintigraphy-guided neck dissection (LSG-ND) in the treatment of N0 oral squamous cell carcinoma.A retrospective cohort study of patients with N0 oral squamous cell carcinoma (SCC) who had either LSG-ND or neck dissection (ND) at our institution between 2008 and 2020.Eighty-seven patients met criteria with N0 oral squamous cell carcinoma with no previous treatment or neck surgery (27 LSG-ND, 60 ND). Sentinel lymph nodes were identified on the contralateral side in 14.8% of patients with unilateral tumors in the LSG-ND group, with 22.2% of cases with unexpected lymphatic drainage outside ipsilateral levels I-III. No recurrences to date have occurred in the LSG-ND cohort, while 13.3% of patients with ND had regional recurrence (p = 0.04).LSG-ND provides a greater ability to identify occult metastatic disease with a significant reduction in regional recurrence in N0 oral SCC.

IntroductionSurgery is the primary treatment for resectable, non-metastatic recurrent head and neck squamous cell carcinoma (HNSCC). We explore the safety and oncologic benefit of intraoperative Cesium-131 (Cs-131) brachytherapy combined with salvage local and/or regional surgical resection.Methods and MaterialsFindings were reported from a single arm multi-institutional prospective phase 1/2 trial involving surgery plus Cs-131 (surgery + Cs-131) treatment. The results of two retrospective cohorts—surgery alone and surgery plus intensity modulated radiation therapy (surgery + ReIMRT)—were also described. Included patients had recurrent HNSCC and radiation history. Safety, tumor re-occurrence, and survival were evaluated.ResultsForty-nine patients were enrolled in the surgery + Cs-131 prospective study. Grade 1 to 3 adverse events (AEs) occurred in 18 patients (37%), and grade 4 AEs occurred in 2 patients. Postoperative percutaneous endoscopic gastrostomy (PEG) tubes were needed in 10 surgery + Cs-131 patients (20%), and wound and vascular complications were observed in 12 patients (24%). No cases of osteoradionecrosis were reported in the surgery + Cs-131 cohort. We found a 49% 2-year disease-free survival at the site of treatment with a substantial number of patients (31%) developing metastatic disease, which led to a 31% overall survival at 5 years.ConclusionsAmong patients with local/regional recurrent HNSCC status-post radiation, surgery + Cs-131 demonstrated acceptable safety with compelling oncologic outcomes, as compared to historic control cohorts.Clinical Trial RegistrationClinicalTrials.gov, identifiers NCT02794675 and NCT02467738.

Objective The coronavirus disease 2019 (COVID-19) pandemic has reduced the demand for, and supply of, head and neck cancer services. This study compares the times to diagnosis, staging, and treatment of head and neck cancers before and during the COVID-19 pandemic. Study Design Retrospective cohort study. Setting Tertiary academic medical center in New York City (NYC). Methods The times to diagnosis, staging, and treatment of head and neck cancer for patients presenting to the clinics of 4 head and neck oncology surgeons with newly diagnosed head and neck cancers were compared between pre–COVID-19 and COVID-19 periods. Results Sixty-eight patients in the pre–COVID-19 period and 26 patients in the COVID-19 period presented with newly diagnosed head and neck cancer. Patients in the COVID-19 group had a significantly longer time to diagnosis than the pre–COVID-19 group after adjustment for age and cancer diagnosis ( P = .02; hazard ratio [HR], 0.54; 95% CI, 0.32-0.92). Patients in the pre–COVID-19 and COVID-19 groups had no statistically significant differences in time to staging ( P > .9; HR, 1.01; 95% CI, 0.58-1.74) or time to treatment ( P = .12; HR, 1.55; 95% CI, 0.89-2.72). Conclusion This study found that time to diagnosis for head and neck cancers was delayed during a COVID-19 period compared to a pre–COVID-19 period. However, there was no evidence of delays in time to staging and time to treatment during the COVID-19 period. Our results prompt further investigations into the factors contributing to diagnostic delays but provide reassurance that despite COVID-19, patients were receiving timely staging and treatment for head and neck cancers.

Background We describe a rare case of high-grade myxofibrosarcoma (MFS) of the maxillary sinus and review the literature on MFS of the paranasal sinuses. Methods A 66-year-old man presented for evaluation of 6–8 weeks of epistaxis and persistent nasal congestion and drainage. Imaging revealed a soft tissue mass extending from the left maxillary sinus into the left nasal cavity. After incomplete resection, the patient elected for post-operative radiotherapy, which was interrupted by rapid local progression. At this point, the patient underwent a total maxillectomy and adjuvant radiotherapy. Results The patient was without evidence of recurrence at a follow-up visit 8 months after diagnosis. Conclusions The recommended treatment of MFS is complete surgical resection. Margin status is the most important predictor of local recurrence. Our case, in which incomplete resection was followed by rapid local progression despite adjuvant radiotherapy, illustrates the unique challenges of treating MFS in the head and neck.

Patients with Fanconi anemia (FA) are at increased risk for head and neck cancers that often necessitate extensive reconstructions. Such patients have multiple comorbidities including anemia and thrombocytopenia frequently requiring bone marrow transplant, and they are at an increased risk of cancer recurrence and need for further extirpation. In the present study, charts from 3 patients with FA who underwent microvascular free tissue transfer by the senior author were retrospectively reviewed for pertinent pre- and peri-operative details in addition to functional and cosmetic outcomes. Two of these patients ultimately required metachronous free flap reconstructions for recurrence. All patients had acceptable functional and cosmetic outcomes following each instance of free flap reconstruction, thereby demonstrating the utility of microvascular free tissue transfer in patients with FA. The authors herein present each patient's clinical history in addition to a discussion of the current literature and an outline of our approach to these challenging cases.

Fanconi anemia (FA), a model syndrome of genome instability, is caused by a deficiency in DNA interstrand crosslink (ICL) repair resulting in chromosome breakage1–3. The FA repair pathway comprises at least 22 FANC proteins including BRCA1 and BRCA24–6, and protects against carcinogenic endogenous and exogenous aldehydes7–10. Individuals with FA are hundreds to thousands-fold more likely to develop head and neck (HNSCC), esophageal and anogenital squamous cell carcinomas (SCCs) with a median onset age of 31 years11. The aggressive nature of these tumors and poor patient tolerance of platinum and radiation-based therapy have been associated with short survival in FA11–16. Molecular studies of SCCs from individuals with FA (FA SCCs) have been limited, and it is unclear how they relate to sporadic HNSCCs primarily driven by tobacco and alcohol exposure or human papillomavirus (HPV) infection17. Here, by sequencing FA SCCs, we demonstrate that the primary genomic signature of FA-deficiency is the presence of a high number of structural variants (SVs). SVs are enriched for small deletions, unbalanced translocations, and fold-back inversions that arise in the context of TP53 loss. The SV breakpoints preferentially localize to early replicating regions, common fragile sites, tandem repeats, and SINE elements. SVs are often connected forming complex rearrangements. Resultant genomic instability underlies elevated copy number alteration (CNA) rates of key HNSCC-associated genes, including PIK3CA, MYC, CSMD1, PTPRD, YAP1, MXD4, and EGFR. In contrast to sporadic HNSCC, we find no evidence of HPV infection in FA HNSCC, although positive cases were identified in gynecologic tumors. A murine allograft model of FA pathway-deficient SCC was enriched in SVs, exhibited dramatic tumor growth advantage, more rapid epithelial-to-mesenchymal transition (EMT), and enhanced autonomous inflammatory signaling when compared to an FA pathway-proficient model. In light of the protective role of the FA pathway against SV formation uncovered here, and recent findings of FA pathway insufficiency in the setting of increased formaldehyde load resulting in hematopoietic stem cell failure and carcinogenesis18–20, we propose that high copy-number instability in sporadic HNSCC may result from functional overload of the FA pathway by endogenous and exogenous DNA crosslinking agents. Our work lays the foundation for improved FA patient treatment and demonstrates that FA SCC is a powerful model to study tumorigenesis resulting from DNA crosslinking damage.

BACKGROUND: For early-stage oral squamous cell carcinoma (OSCC), there is no existing risk-stratification modality beyond conventional TNM staging system to identify patients at high risk for cancer-specific mortality. METHODS: A total of 568 early-stage OSCC patients who had surgery only and also with available 5-year clinical outcomes data were identified. Signature microRNAs (miRNAs) were discovered using deep sequencing analysis and validated by qRT-PCR. The final 5-plex prognostic marker panel was utilized to generate a cancer-specific mortality risk score using the multivariate Cox regression analyses. The prognostic markers were validated in the internal and external validation cohorts. RESULTS: The risk score from the 5-plex marker panel consisting of miRNAs-127-3p, 4736, 655-3p, TNM stage and histologic grading stratified patients into four risk categories. Compared to the low-risk group, the high-risk group had 23-fold increased mortality risk (hazard ratio 23, 95% confidence interval 13-42), with a median time-to-recurrence of 6 months and time-to-death of 11 months (vs >60 months for each among low-risk patient; p < .001). CONCLUSION: The miRNA-based 5-plex marker panel driven mortality risk score formula provides clinically practical and reliable measures to assess the prognosis of patients assigned to an early-stage OSCC.

Objective The main objective of this study was to quantify daily pain and opioid use in patients after hemithyroidectomy and cervical lymph node biopsy (CLNB). The secondary objective was to identify factors associated with decreased pain and opioid use. Study Design Prospective cohort study from June 2017 to February 2019. Patients were given paper surveys to record daily postoperative opioid use and maximal pain on a visual analog scale. Setting Single institution (NewYork-Presbyterian/Weill Cornell Medical Center). Methods All adult patients undergoing hemithyroidectomy and CLNB by a single surgeon were consecutively selected for participation. Patients recorded daily pain and opioid analgesic use over a 2-week postoperative period. Results Of 33 patients enrolled, 29 (87.9%) returned a survey. Thirteen underwent CLNB, and 16 underwent hemithyroidectomy. Pain resolved after both procedures by the end of the 2-week period. CLNB patients used a median (interquartile range) of 15.0 (0-41.2) morphine milligram equivalents (MME), and 95% used 70 or fewer MME. Hemithyroidectomy patients used a median of 8.2 (4.5-13.9) MME, and 95% used 30 or fewer MME. Use of nonopioid analgesics was associated with a statistically significant decrease in pain (56.1 vs 171 visual analog scale, 95% confidence interval [CI] of Δ = [12.0 to 217.8]) and opioid use (12.2 vs 48.8 MME, 95% CI of Δ = [5.0 to 68.1 MME]) in CLNB but not in hemithyroidectomy. Conclusion Patients have low pain and opioid requirements after hemithyroidectomy and CLNB. Head and neck surgeons should evaluate their opioid-prescribing patterns for opportunities to safely decrease postoperative prescriptions.

The COVID-19 health crisis abruptly disrupted the practice of otolaryngology. This article aims to define the changes needed to operate an academic otolaryngology practice safely and efficiently from within the epicenter of the pandemic. We define the areas of normal patient workflow that have been affected by COVID-19, and we offer mitigation strategies with attention paid to the specific needs of subspecialties.The article includes data specific to the office practice metrics of the Weill Cornell Medicine Department of Otolaryngology-Head and Neck Surgery, as well as publically available data from New York Presbyterian Hospital system and theExpert opinion.Through careful planning and execution, it is possible to reestablish safe otolaryngologic patient care during the COVID-19 pandemic. It will require a significant change from prior practice models for successful implementation. Additionally, telemedicine can be positively integrated into the treatment of otolaryngology diseases for new and established patients.The information conveyed in this review can be used as a guide by large and small otolaryngology groups to identify aspects of the patient visit that are "at risk" due to COVID-19, and it suggests sensible responses that can be made without a significant disruption to normal practice. The methods used to identify vulnerabilities with the patient visit process can be applied to future unforeseen crises, such as a resurgence of COVID-19 or a novel pandemic.

To quantify pain and opioid use after otorhinolaryngologic surgery. To determine the effect of patient and surgical factors on primary outcomes.Prospective cohort.Tertiary academic hospital.Patients undergoing elective otorhinolaryngologic surgery were prospectively enrolled. Patients completed demographic surveys and psychometric questionnaires assessing attitudes toward pain and baseline anxiety and depression before surgery. After surgery, patients documented peak pain levels (0-100 mm, visual analog scale) and daily prescription and nonprescription analgesic requirements over a 2-week period. Average daily and cumulative pain and opioid use were calculated and compared among patient cohorts stratified by procedure and preoperative factors.A total of 134 patients were enrolled. Total tonsillectomy was associated with significantly higher pain scores and opioid consumption, as compared to all other procedures. There was moderate correlation between average cumulative pain and opioid use. Older patients required significantly fewer doses of opioids. There was no effect of sex, marital status, or education level on postoperative pain or opioid use. Psychometric instrument scores and chronic pain or analgesic use were not associated with significant differences in pain or opioid requirements. Most patients were prescribed substantially more opioids than they actually required.Postoperative pain following elective otorhinolaryngologic surgery decreases dramatically within the first week and requires only few days of opioid analgesia, with the exception of tonsillectomy. Almost all patients required fewer than 15 doses of opioids.

We have developed and validated a novel microRNA (miRNA)-based prognostic model to predict survival outcome in oral squamous cell carcinoma (OSCC) patients who are already categorized into ‘early-stage’ by the TNM system. A total of 836 early-stage OSCC patients were assigned the mortality risk scores. We evaluated the efficacy of various treatment regimens in terms of survival benefit compared to surgery only in patients stratified into high and low mortality risk categories. Within the high-risk group, surgery with neck dissection significantly improved the 5-year survival to 75% from 46% (p

We have previously constructed a novel microRNA (miRNA)-based prognostic model and cancer-specific mortality risk score formula to predict survival outcome in oral squamous cell carcinoma (OSCC) patients who are already categorized into “early-stage” by the TNM staging system. A total of 836 early-stage OSCC patients were assigned the mortality risk scores. We evaluated the efficacy of various treatment regimens in terms of survival benefit compared to surgery only in patients stratified into high (risk score ≥0) versus low (risk score p < 0.001 ); a Cox proportional hazard model on time-to-death demonstrated a hazard ratio of 0.37 for surgery with neck dissection (95% CI: 0.2–0.6; p = 0.0005 ). For the low-risk group, surgery only was the treatment of choice associated with 5-year survival benefit. Regardless of treatment selected, those with risk score ≥2 may benefit from additional therapy to prevent cancer relapse. We also identified hTERT (human telomerase reverse transcriptase) as a gene target common to the prognostic miRNAs. There was 22-fold increase in the hTERT expression level in patients with risk score ≥2 compared to healthy controls ( p < 0.0005 ). Overexpression of hTERT was also observed in the patient-derived OSCC organoid compared to that of normal organoid. The DNA cancer vaccine that targets hTERT-expressing cells currently undergoing rigorous clinical evaluation for other tumors can be repurposed to prevent cancer recurrence in these high-risk early-stage oral cancer patients.

Purpose To review brachytherapy use in recurrent head and neck carcinoma (RHNC) with focus on its efficacy and complication rates. Material and methods A literature search of PubMed, Ovid, Google Scholar, and Scopus was conducted from 1990 to 2017. Publications describing treatment of RHNC with brachytherapy with or without surgery were included. The focus of this review is on oncologic outcomes and the safety of brachytherapy in the recurrent setting. Results Thirty studies involving RHNC treatment with brachytherapy were reviewed. Brachytherapy as adjunctive treatment to surgical resection appears to be associated with an improved local regional control and overall survival, when compared with the published rates for re-irradiation utilizing external beam radiotherapy (RT) or brachytherapy alone. Safety data remains variable with different isotopes and dose rates with implantable brachytherapy demonstrating a tolerable side effect profile. Conclusions Although surgery remains a mainstay treatment for RHNC, intraoperative interstitial brachytherapy delivery as adjunctive therapy may improve the treatment outcome and may be associated with fewer complication rates as compared to reirradiation using external beam radiotherapy. Further investigations are required to elucidate the role of brachytherapy for RHNC.

Background and Objectives Transoral robotic surgery (TORS) has increased for treatment of oropharyngeal squamous cell carcinoma (OPSCC). To define the adoption of TORS, we analyzed patterns of surgical treatment for OPSCC in the US. Methods Cases of T1-T3 OPSCC treated with surgery between 2010 and 2013 from the National Cancer Database were queried. Results Of 3,071 patients who underwent primary surgical management for T1-T3 OPSCC, 846 (28%) underwent TORS. On multivariable analysis, low tumor stage (T2 vs. T1: OR 0.75, CI 0.37–0.51, P

Background The American Thyroid Association (ATA) has established guidelines for prophylactic thyroidectomy in multiple endocrine neoplasia type 2A (MEN2A) based on rearranged during transfection (RET) mutations. In silico analysis, which uses computer modeling to predict alterations in protein structure, is a new method for studying these mutations. Methods We describe a kindred with MEN2A, all sharing a well-documented RET mutation, p.C634Y, as well as a mutation of undetermined significance, p.I852M, which we analyzed via in silico analysis. Results The p.C634Y mutation resulted in severe predicted RET alterations, whereas the p.I852M resulted in only modest changes. Both mutations together resulted in only a small additional disruptive effect in protein structure beyond that which occurred with p.C634Y alone. Conclusion Although in silico analysis may be helpful in quantitating changes in protein structure that occur in patients who have novel RET mutations (single or multiple), additional factors must account for the highly variable aggressiveness of the disease (C-cell hyperplasia/medullary thyroid carcinoma [MTC]) noted in our kindred. © 2016 Wiley Periodicals, Inc. Head Neck, 2016

Introduction: Fanconi anemia (FA) patients have a high predilection for developing aggressive head and neck squamous cell carcinoma (HNSCC) at an early age. Because of their aggressive HNSCC, these patients frequently require multiple modality treatment, including radiation therapy. However, their underlying chromosomal instability makes giving radiation to these patients difficult because of increased radiation-induced complications. In order to better understand this problem, we evaluated a large population of FA patients with HNSCC who received radiation therapy for HNSCC. Hypothesis: FA patients who have undergone prior bone marrow transplant (BMT) and present with head and neck squamous cell carcinoma (HNSCC) demonstrate decreased relative risk of radiation-induced complications compared to FA patients who have not received BMT. Study Design: Retrospective Cohort Study Methods: Demographic information, genotypic data, prognostic factors, therapeutic management, and survival outcomes for FA patients enrolled in the International Fanconi Anemia Registry (IFAR) who developed HNSCC from 1981-2017 were analyzed. Results: Data were collected from the IFAR registry for n=50 patients. The mean age at time of HNSCC diagnosis was 30.5 + 9.0 years, and 36% of total patients were male. 42% of the patients received adjuvant or neoadjuvant radiotherapy for HNSCC, with the most common subsites involving the oral tongue (n=8), followed by alveolus (n=3) and supraglottis (n=2). The average dose given was 48.8 + 16.9 Gy over a 28- to 70-day period. Among patients receiving radiotherapy, 24% had received a bone marrow transplant. No patients who had a prior BMT were noted to develop pancytopenia as a radiation-related complication, while 68% of those without prior BMT developed pancytopenia from radiation. 6/16 patients who did not undergo transplant completed the full course of radiation, while 4/5 BMT patients were able to complete treatment. Both mucositis and sepsis were secondary complications more common in the non-BMT group (56% vs. 40%, and 12% vs. 0%, respectively). The relative risk of pancytopenia among patients receiving BMT and adjuvant radiation was 0.15 (95% CI 0.01-2.2); however, this was not statistically significant given the small BMT-group sample size (p=0.17). Interestingly, 80% of patients in the BMT group exhibited genetic mutations of the Fanconi complementation group C. Among patients who received a BMT and adjuvant radiotherapy, 5 patients died of XRT-related complications, while only 1 patient died from these complications in the BMT group. Conclusion: While it is well known that FA patients have a high predilection for developing aggressive HNSCC at an early age, among the patients who received preradiation bone marrow transplant the incidence of radiation-induced pancytopenia was reduced. This reduction suggests that FA patients undergoing radiotherapy for HNSCC may exhibit less adverse side effects of radiation treatment if they received a prior BMT. Citation Format: Ade Obayemi, K.R. Patel, O. Rosti, A. Smogorzewska, D. Kutler. The role of bone marrow transplantation in patients with Fanconi anemia and head and neck squamous cell carcinoma receiving radiotherapy: An examination of the International Fanconi Anemia (IFAR) Registry [abstract]. In: Proceedings of the AACR-AHNS Head and Neck Cancer Conference: Optimizing Survival and Quality of Life through Basic, Clinical, and Translational Research; 2019 Apr 29-30; Austin, TX. Philadelphia (PA): AACR; Clin Cancer Res 2020;26(12_Suppl_2):Abstract nr B23.

Purpose: The feasibility and efficacy of re-irradiation using contemporary radiation techniques to treat recurrent head and neck cancer has been demonstrated but the role of brachytherapy is unclear. Here we describe the use of 131 Cs brachytherapy with concurrent salvage surgery in 18 patients. Material and methods: Eligible patients underwent maximal gross resection of the tumor with implantation of brachytherapy seeds delivering a minimum dose of 80 Gy to the tumor bed. Rates of overall survival, locoregional progression free survival, disease-free survival, and radiation-induced toxicity were analyzed. Results: Retrospective Kaplan-Meier analysis shows median overall survival was 15 months and disease free survival was 12 months. Two patients developed grade 3 toxicity; all other complications were grade 1-2 with no grade 4 or 5 complications. Conclusions: Compared to prior literature, our study shows comparable rates of survival with a decreased rate of radiation-induced toxicity. J Contemp Brachytherapy 2015; 7, 6: 445-452 DOI: 10.5114/jcb.2015.56764

Pycnodysostosis is an extremely rare autosomal recessive osteosclerotic disorder that stems from inactivation of the cathepsin K gene resulting in a predisposition to multiple fractures. We present the case of a 46-year-old male with a history of pycnodysostosis and over 30 previous fractures, who presented with a pathologic fracture of the right mandibular angle which was successfully reconstructed using a fibula osteocutaneous free flap.

Introduction Ultrasonography-guided fine-needle aspiration (US-FNA) yields diagnostic material more often than palpation-guided FNA does. It is often performed by an interventional radiologist (IR) but rarely by a cytopathologist (CP). Herein we describe our method of performance and growing experience with this technique. Materials and methods Data from US-FNA of head and neck lesions performed over a 33-month period by both a CP and an IR were reviewed. Special attention was paid to cases for which histologic follow-up was available. Association in concordance between cytologic and histologic diagnoses was attempted using Fisher's exact test. Mean size of masses biopsied, number of passes performed, and passes needed to achieve adequacy were compared between groups using the Wilcoxon rank-sum test. Tests were 2-sided with P Results Of the 175 US-FNAs performed, 108 (62%) were done by the CP and 67 (38%) by the IR. Fifty-eight patients had histologic follow-up; 37 (64%) for the CP and 21 (36%) for the IR. Mean mass size was significantly smaller for the IR at 2.11 cm versus 2.9 cm for the CP ( P = 0.021). Adequacy was achieved after 1 pass in 70% of cases (26 of 37) by the CP and 67% (14 of 21) by the IR. Number of passes performed did not vary significantly between groups. A variety of masses were biopsied; however, the small sample size precluded meaningful evaluation of cytologic concordance to final histology. Conclusions CP-performed US-FNA has been successfully delivered to clinicians at our institution.

Objectives/Hypothesis To describe the management and outcomes of Fanconi anemia (FA) patients with head and neck squamous cell carcinoma. Study Design Cohort study. Methods Demographic information, prognostic factors, therapeutic management, and survival outcomes for FA patients enrolled in the International Fanconi Anemia Registry who developed head and neck squamous cell carcinoma (HNSCC) were analyzed. Results Thirty-five FA patients were diagnosed with HNSCC at a mean age of 32 years. The most common site of primary cancer was the oral cavity (26 of 35, 74%). Thirty patients underwent surgical resection of the cancer. Sixteen patients received radiation therapy with an average radiation dose of 5,050 cGy. The most common toxicities were high-grade mucositis (9 of 16, 56%), hematologic abnormalities (8 of 16, 50%), and dysphagia (8 of 16, 50%). Three patients received conventional chemotherapy and had significant complications, whereas three patients who received targeted chemotherapy with cetuximab had fewer toxicities. The 5-year overall survival rate was 39%, with a cause-specific survival rate of 47%. Conclusions Fanconi anemia patients have a high risk of developing aggressive HNSCC at an early age. Fanconi anemia patients can tolerate complex ablative and reconstructive surgeries, but careful postoperative care is required to reduce morbidity. The treatment of FA-associated HNSCC is difficult secondary to the poor tolerance of radiation and chemotherapy. However, radiation should be used for high-risk cancers due to the poor survival in these patients. Level of Evidence 4. Laryngoscope, 2015

Objective: To evaluate the Weill Cornell Medical College (WCMC)/New York Presbyterian Hospital (NYPH) experience with intraoperative frozen (IOF) section in the management of thyroid nodules with a fine needle aspiration (FNA) diagnosis of Bethesda IIâVI and to analyze the cost and pathology benefit it provides. Methods: The surgical and cytopathology files at WCMC/NYPH were searched within the time period of January 2008 to May 2013. A total of 435 thyroid specimens were identified for which both an FNA and subsequent IOF section was performed. The FNA was correlated with the locations of the resected nodule and the nodule frozen for intraoperative diagnosis. The results of the FNA were compared to the IOF section diagnosis and final diagnosis (FD). Results: Among 435 cases, the FNA diagnosis was Bethesda II: 149 cases, Bethesda III: 170 cases, Bethesda IV: 91 cases, Bethesda V: 19 cases, and Bethesda VI: 6 cases. There were a total of 83 carcinomas identified on FD, which included 69 papillary thyroid carcinomas (PTCs), 12 follicular carcinomas, and 2 poorly differentiated carcinomas. The preoperative FNA diagnosis for these carcinomas was as follows: Bethesda II, 11/149 (7.4%), Bethesda III, 24/170 (14%), Bethesda IV, 26/91 (29%), Bethesda V, 16/19 (84%), and Bethesda VI, 6/6Â (100%). IOF section contributed to the diagnosis of malignancy in 16/429 (4%) cases: 1/149 (0.7%) Bethesda II, 5/170 (3%) Bethesda III, 2/91 (1.1%) Bethesda IV, and 8/19 (42%) Bethesda V. The diagnosis of malignancy was confirmed in the 6 Bethesda VI cases by IOF section. There were no false positives on IOF section. IOF had a sensitivity and specificity of 26% and 100%, respectively. Conclusion: The role of IOF section is limited in the evaluation of thyroid nodules. IOF section is most useful for nodules with an FNA diagnosis of Bethesda V lesions. The diagnosis of follicular variant of PTC remains difficult on frozen section. Keywords: Thyroid, Nodule, Frozen section, Fine-needle aspiration, Sensitivity, Specificity

To analyze the utility of intraoperative parathyroid hormone (IOPTH) monitoring for patients with primary hyperparathyroidism who had evidence of single-gland disease on preoperative imaging with modified 4-dimensional computed tomography that was done in conjunction with ultrasonography (Mod 4D-CT/US).Case series with chart review.Tertiary care university medical center.Patients were drawn from consecutive directed parathyroidectomies performed between December 2001 and June 2013 by the senior authors. All patients had primary hyperparathyroidism and underwent a Mod 4D-CT/US study that showed findings on both studies that were consistent with a single adenoma. The modified Miami criteria were used for IOPTH monitoring (parathyroid hormone decrease by50% and into the normal range).Of 356 patients who underwent parathyroid surgery, 206 had a single gland localized on the Mod 4D-CT and the US studies. IOPTH monitoring was used in 172 cases, of which 169 had adequate clinical follow-up to assess the surgical outcome. Twenty-one patients (12.4%) had IOPTH values that did not meet modified Miami criteria after removal of one gland, of which 7 were found to have multigland disease (4.1%). Three patients (1.8%) had persistent primary hyperparathyroidism despite an IOPTH that met modified Miami criteria.Although IOPTH monitoring correctly identifies a small percentage of patients with multigland disease, some patients will be subjected to unnecessary neck explorations that can result in difficult intraoperative decisions, such as whether to remove normal or equivocal-sized glands when they are encountered.

Background/Aims: Preoperative identification of malignant parotid lesions remains challenging, and thus, some surgeons use frozen section (FS) to assist them in their decision making. We evaluated the pathologic and cost benefit of FS after fine-needle aspiration (FNA) at our institution. Methods: We assessed medical data for 260 patients undergoing parotidectomy with FS. The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value were calculated for radiology, FNA, and FS. Results: The sensitivities, specificities, and accuracies of FNA and FS were 75.0, 96.4, and 93.2%, and 75.0, 100, and 96.8%, respectively. FS detected 0% of FNA false negatives and 80% of false positives. The additional pathology charge for FS alone per correctly identified benign lesion after a positive FNA was USD 1,443. Conclusion: FNA and FS are more reliable in the prognostication of the final pathology than radiology. At our center, FS appears to be of limited clinical use after benign FNAs, but may be more useful after positive, indeterminate, and nondiagnostic FNAs.

The American Thyroid Association (ATA) has established guidelines for prophylactic thyroidectomy in multiple endocrine neoplasia type 2A (MEN2A) based on rearranged during transfection (RET) mutations. In silico analysis, which uses computer modeling to predict alterations in protein structure, is a new method for studying these mutations.We describe a kindred with MEN2A, all sharing a well-documented RET mutation, p.C634Y, as well as a mutation of undetermined significance, p.I852M, which we analyzed via in silico analysis.The p.C634Y mutation resulted in severe predicted RET alterations, whereas the p.I852M resulted in only modest changes. Both mutations together resulted in only a small additional disruptive effect in protein structure beyond that which occurred with p.C634Y alone.Although in silico analysis may be helpful in quantitating changes in protein structure that occur in patients who have novel RET mutations (single or multiple), additional factors must account for the highly variable aggressiveness of the disease (C-cell hyperplasia/medullary thyroid carcinoma [MTC]) noted in our kindred. © 2016 Wiley Periodicals, Inc. Head Neck 38: E1881-E1885, 2016.