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1.
Head Neck ; 2024 Feb 23.
Artigo em Inglês | MEDLINE | ID: mdl-38390640

RESUMO

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.

2.
Am J Otolaryngol ; 45(1): 104054, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37729774

RESUMO

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.


Assuntos
Adenocarcinoma , Neoplasias da Glândula Tireoide , Humanos , Neoplasias da Glândula Tireoide/diagnóstico por imagem , Neoplasias da Glândula Tireoide/cirurgia , Necrose
3.
JAMA Otolaryngol Head Neck Surg ; 149(12): 1149-1150, 2023 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-37824138

RESUMO

A 27-year-old woman had primary complaints of headaches and visual-spatial difficulties when driving at night. She also had a progressively enlarging lesion on her left scalp. What is your diagnosis?


Assuntos
Couro Cabeludo , Neoplasias Cutâneas , Humanos , Neoplasias Cutâneas/cirurgia
4.
Head Neck ; 45(8): 1894-1902, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37255301

RESUMO

BACKGROUND: While a single-stage free-flap reconstruction is the preferred approach for oromandibular defects, a multistaged approach may be necessary in rare cases. These patients can still be effectively restored with functional and aesthetic improvements. METHODS: We report two cases with a history of bilateral failed fibula free flaps. We detail the multistaged reconstruction to repair these complex defects and discuss the considerations when planning such procedures. RESULTS: Both patients successfully underwent a staged reconstruction with an iliac crest osteocutaneous flap following either a rectus abdominis or pectoralis major myocutaneous flap. CONCLUSION: Oromandibular reconstruction is an expected outcome in the contemporary management of oral cavity cancer and osteoradionecrosis. However, complications do occur and can be devastating. In cases of bilateral failed fibula free flaps, a staged approach is a favorable option. Moreover, the iliac crest provides an important reconstructive option with the documented potential for implant born dental rehabilitation.


Assuntos
Retalhos de Tecido Biológico , Neoplasias Bucais , Procedimentos de Cirurgia Plástica , Humanos , Retalhos de Tecido Biológico/cirurgia , Neoplasias Bucais/cirurgia , Fíbula/transplante , Ílio/cirurgia
5.
Laryngoscope ; 133(11): 3228-3231, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37067021

RESUMO

Surgical treatment for thyroid carcinoma invading the trachea often involves circumferential tracheal resection and primary tracheal repair. This procedure involves a significant risk of anastomotic breakdown. We present a novel approach to cricotracheal repair using an SCM flap bolster designed to reduce the risk of anastomotic complications. Laryngoscope, 133:3228-3231, 2023.


Assuntos
Neoplasias da Glândula Tireoide , Traqueia , Humanos , Traqueia/cirurgia , Traqueia/patologia , Neoplasias da Glândula Tireoide/cirurgia , Neoplasias da Glândula Tireoide/patologia , Retalhos Cirúrgicos/patologia , Tireoidectomia/métodos
6.
Diagn Pathol ; 18(1): 47, 2023 Apr 18.
Artigo em Inglês | MEDLINE | ID: mdl-37072862

RESUMO

BACKGROUND: Struma ovarii is an unusual ovarian teratoma containing predominantly thyroid tissue. Less than 10% of cases undergo malignant transformation in the thyroid tissue and are considered malignant struma ovarii (MSO). MSO have been reported with concurrent thyroid lesions, but molecular data is lacking. CASE PRESENTATION: A 42-year-old female developed MSO and synchronous multifocal subcentimeter papillary thyroid carcinoma (PTC). The patient underwent a salpingo-oophrectomy, thyroidectomy, and low-dose radioactive iodine ablation. Both the thyroid subcentimeter PTC and MSO were positive for BRAF V600E mutation, and microRNA expression profiles were similar across all tumor deposits. However, only the malignant component demonstrated extensive loss of heterozygosity (LOH) involving multiple tumor suppressor gene (TSG) chromosomal loci. CONCLUSIONS: We present the first reported case of MSO with synchronous multifocal subcentimeter PTC in the thyroid containing concordant BRAF V600E mutations and resulting with discordant LOH findings. This data suggests that loss of expression in tumor suppressor gene(s) may be an important contributor to phenotypic expression of malignancy.


Assuntos
MicroRNAs , Neoplasias Ovarianas , Estruma Ovariano , Neoplasias da Glândula Tireoide , Feminino , Humanos , Adulto , Neoplasias da Glândula Tireoide/patologia , Estruma Ovariano/genética , Estruma Ovariano/metabolismo , Estruma Ovariano/patologia , Radioisótopos do Iodo , Proteínas Proto-Oncogênicas B-raf/genética , Câncer Papilífero da Tireoide/genética , Mutação , Perda de Heterozigosidade , Neoplasias Ovarianas/patologia , MicroRNAs/genética
7.
Head Neck Pathol ; 17(2): 479-486, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36849672

RESUMO

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.


Assuntos
Carcinoma de Células Escamosas , Neoplasias Bucais , Humanos , Invasividade Neoplásica/patologia , Neoplasias Bucais/patologia , Carcinoma de Células Escamosas/cirurgia , Carcinoma de Células Escamosas/patologia , Recidiva Local de Neoplasia/patologia , Estudos Retrospectivos , Estadiamento de Neoplasias
8.
Am J Otolaryngol ; 44(2): 103756, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36603379

RESUMO

BACKGROUND: Oropharyngeal squamous carcinomas cause significant morbidity and mortality. Poor prognosticators include lymphovascular and perineural invasion. Extratumoral phenotypes of these histologic findings confer worse prognoses. METHODS: We report eight cases of recurrent oropharyngeal cancer with diffuse extratumoral lymphovascular invasion (ELVI) or extratumoral perineural invasion (EPNI) and review the existing literature. RESULTS: On salvage resection for recurrence following primary radiation or chemoradiation, six patients manifested ELVI and two showed EPNI. These patterns conferred difficulty with complete surgical clearance; final pathologic analysis demonstrated positive margins for all eight patients. The six patients with ELVI were p16+ and the two with EPNI were p16-. Currently, two patients are deceased and six patients are alive at an average follow-up of 17.4 months. Of the six living patients, 2 have a new recurrence and are in hospice while 4 have no evidence of disease. CONCLUSIONS: ELVI and EPNI have received little consideration in the literature as unique histopathologic features of oropharyngeal squamous carcinoma. We present the first series on these adverse extratumoral features in recurrent disease. We call attention to these unique histologic features in the setting of recurrent oropharyngeal cancer to encourage others to track the results of therapeutic intervention and to identify successful strategies for treatment.


Assuntos
Carcinoma de Células Escamosas , Neoplasias de Cabeça e Pescoço , Neoplasias Bucais , Neoplasias Orofaríngeas , Humanos , Carcinoma de Células Escamosas de Cabeça e Pescoço/patologia , Carcinoma de Células Escamosas/cirurgia , Estadiamento de Neoplasias , Estudos Retrospectivos , Recidiva Local de Neoplasia/patologia , Neoplasias Bucais/patologia , Neoplasias de Cabeça e Pescoço/patologia
9.
Head Neck ; 45(2): 439-448, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36495223

RESUMO

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.


Assuntos
Ajustamento Emocional , Neoplasias da Glândula Tireoide , Humanos , Tireoidectomia , Qualidade de Vida , Neoplasias da Glândula Tireoide/cirurgia , Neoplasias da Glândula Tireoide/epidemiologia , Risco , Câncer Papilífero da Tireoide/cirurgia , Estudos Retrospectivos
10.
Ann Otol Rhinol Laryngol ; 132(2): 173-181, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35249359

RESUMO

BACKGROUND: Instruments to assess surgical skills have been validated for several key indicator procedures in otolaryngology. Selective neck dissection is a core procedure for which trainees must integrate knowledge of complex head and neck anatomy with technical surgical skills. An instrument for assessment of surgical performance in selective neck dissection has not been previously developed. The objective of the current study is to develop and validate an instrument for assessing surgical competency for level II-IV selective neck dissection. DESIGN: A Delphi working group comprised of 23 fellowship trained head and neck surgeons from 17 institutions was assembled. The modified Delphi method encompassed a 3-step process, including 2 anonymous voting rounds to successively refine individual items and establish levels of consensus. Thresholds for achieving strong consensus, at >80% agreement, were determined a priori. The resulting instrument was subsequently validated in a prospective cohort of 17 resident surgeons, spanning postgraduate year 1 to 5 training experience. Participants were asked to perform a level II-IV selective neck dissection on fresh-frozen cadaveric specimens. Performance was scored by 2 independent, blinded observers using the devised instrument and construct validity was assessed. RESULTS: Through the modified Delphi process a final list of 30 items, considered to be the most essential items for achieving the goals of a level II-IV selective neck dissection, was developed. Construct validity was supported by a positive association between instrument scores compared to both resident postgraduate year level and number of head and neck rotations completed. CONCLUSION: The development and validation of a novel instrument for assessment of surgical competency in level II-IV selective neck dissection, a key indicator case in otolaryngology, is described. This new instrument may be used to provide objective feedback on overall and task-specific competency to identify surgical deficiencies and offer granular feedback to enhance surgical training.


Assuntos
Internato e Residência , Otolaringologia , Humanos , Esvaziamento Cervical , Estudos Prospectivos , Otolaringologia/educação , Avaliação Educacional , Competência Clínica
11.
Head Neck ; 45(1): 10-21, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36065715

RESUMO

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.


Assuntos
Secções Congeladas , Neoplasias de Cabeça e Pescoço , Humanos , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/cirurgia , Cintilografia
13.
Head Neck ; 44(8): 1995-2000, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35638703

RESUMO

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.


Assuntos
Fascia Lata , Procedimentos de Cirurgia Plástica , Fascia Lata/transplante , Humanos , Mandíbula/cirurgia , Boca/cirurgia , Procedimentos de Cirurgia Plástica/efeitos adversos , Procedimentos de Cirurgia Plástica/métodos , Coxa da Perna/cirurgia
14.
Am J Otolaryngol ; 43(3): 103461, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35427937

RESUMO

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.


Assuntos
Transtornos de Deglutição , Disfonia , Hipotireoidismo , Tireoide Lingual , Transtornos de Deglutição/etiologia , Humanos , Tireoide Lingual/cirurgia , Estudos Retrospectivos , Língua/patologia
15.
Head Neck ; 44(7): 1737-1741, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35388943

RESUMO

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.


Assuntos
Neoplasias Laríngeas , Laringe Artificial , Estomas Cirúrgicos , Constrição Patológica/cirurgia , Humanos , Neoplasias Laríngeas/cirurgia , Laringectomia/efeitos adversos , Laringectomia/reabilitação , Masculino , Pessoa de Meia-Idade , Punções , Estudos Retrospectivos , Traqueia/cirurgia
16.
Head Neck ; 44(4): E16-E24, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35064717

RESUMO

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.


Assuntos
Carcinoma de Células Escamosas , Carcinoma Verrucoso , Neoplasias de Cabeça e Pescoço , Neoplasias Bucais , Idoso de 80 Anos ou mais , Carcinoma de Células Escamosas/patologia , Carcinoma Verrucoso/diagnóstico , Carcinoma Verrucoso/patologia , Carcinoma Verrucoso/cirurgia , Feminino , Humanos , Masculino , Neoplasias Bucais/cirurgia , Carcinoma de Células Escamosas de Cabeça e Pescoço
18.
Otol Neurotol ; 42(2): e153-e156, 2021 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-33273307

RESUMO

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.


Assuntos
Implante Coclear , Internato e Residência , Competência Clínica , Avaliação Educacional , Humanos , Estudos Prospectivos , Reprodutibilidade dos Testes
19.
Otol Neurotol ; 41(4): e501-e506, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32176143

RESUMO

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.


Assuntos
Neurofibromatose 2 , Neuroma Acústico , Adulto , Idoso , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Minnesota , Neurofibromatose 2/diagnóstico por imagem , Neurofibromatose 2/epidemiologia , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/epidemiologia , Prevalência
20.
Otolaryngol Head Neck Surg ; 162(4): 530-537, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31986971

RESUMO

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.


Assuntos
Perda Auditiva/etiologia , Neuroma Acústico/complicações , Neuroma Acústico/patologia , Carga Tumoral , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
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