Ameloblastoma demographic,clinical and treatment study - analysis of 40 cases

Dental lesions represent about 1% of oral cavity tumors being ameloblastoma the most common one. It is a tumor of epithelial origin that mainly affects the jaw, and less commonly the maxilla. Its clinical presentation is that of an asymptomatic slow-growing tumor. Despite being a benign tumor, it has an invasive behavior with a high rate of recurrence if not treated properly.ObjectiveTo describe the cases of ameloblastoma in a reference department.MethodsRetrospective analysis of 40 cases. The variables analyzed were: age, gender, ethnicity, tumor location, type of treatment, complications and recurrence.ResultsThe most affected gender was male - 21 cases (52.5%); with a predominance of Caucasians - 24 cases (60%). The mean age was 35.45 years; the most common location was in the jaw - 37 cases (92.5%). Facial asymmetry was the most frequent complaint. Of the 40 cases, 33 were submitted to surgery. Of those submitted to surgery, 24 (72.72%) underwent segmental resection, with recurrence in 4 (12.12%) cases.ConclusionAmeloblastoma may relapse when treatment is not performed with broad surgical resection of the lesion with wide safety margins.     

Adenomatoid odontogenic tumor: two cases

INTRODUCTION: Adenomatoid odontogenic tumor, initially referred to as adenoameloblastoma, is a rare and benign odontogenic tumor. No recurrence has been reported after enucleation-resection. CASE REPORTS: We report two cases of adenomatoid odontogenic tumor, which illustrate the radiological and anatomic features of this tumor. They occurred in two 21 and 14 years-old teenagers who presented gingival swelling. The panoramic radiographs showed a radiolucent lesion with an impacted tooth. Enucleation-resection was performed with good outcome. DISCUSSION: The clinical, radiological and histological features of adenomatoid odontogenic tumor are distinct from those of ameloblastoma. It affects most commonly patients in the second decade of life predominantly females. This tumor is frequently misdiagnosed as other odontogenic cysts or tumors. We discuss the anatomoclinical features and the histogenesis of this rare tumor.     

Malignant odontogenic tumors: a 22-year experience

OBJECTIVES/HYPOTHESIS: Malignant odontogenic tumors are exceedingly rare and arise from odontogenic epithelial residues and odontogenic cysts in the jaw bones. Odontogenic malignancies have various origins. Some develop directly from the remnants of odontogenic epithelium left after completion of dental development; others may result from malignant transformation of a benign odontogenic cyst or ameloblastoma. These lesions are usually locally aggressive with radical surgery being the primary mode of treatment. Because of their rarity, much of the existing information about malignant odontogenic tumors with regard to their origin, clinicopathological features, biological behavior, and therapeutics is derived from case reports or small series. The study represents one of the largest series of malignant odontogenic tumors compiled in a single institution. STUDY DESIGN: Retrospective 22-year review from an Academic Medical Center. METHODS: Twenty cases of reported malignant odontogenic tumors were diagnosed in the authors' institution between 1981 and 2002. All pathological slides were reviewed to reconfirm diagnosis. Malignancy was confirmed based on the following criteria: histological findings of infiltrative growth, atypical cytological features, and focal necrosis or clear evidence of distant metastatic spread. Patient age, race, sex, treatment and outcome were recorded on chart review. RESULTS: Of the twenty reported cases, only nine were actually found to be malignant tumors on re-evaluation. These consisted of four cases of malignant ameloblastomas, two cases of ameloblastic carcinoma, one case of malignant Pindborg tumor (calcifying epithelial odontogenic tumor), one case of odontogenic ghost cell carcinoma, and one case of squamous cell carcinoma arising in an odontogenic keratocyst. The racial demographics were six Caucasian patients, one African American patient, and two Asian patients; seven men and two women represented the gender distribution. Tumors of six cases were located in the mandible, and of three cases, in the maxilla. All cases were treated with radical surgical excision. Two patients died of their disease, three patients were alive and free of disease, and four patients were lost to follow-up. CONCLUSION: Malignant odontogenic tumors are rare. They require a multidisciplinary team to determine proper treatment. Long-term surveillance is mandatory and is accomplished by routine physical examinations, along with serial radiographic imaging.     

Odontogenic infection and antiresorptive agent-related osteonecrosis of the jaw with facial subcutaneous abscess formation: A retrospective clinical study of difficult-to-diagnose cases

ObjectiveThe objective of this study was to analyze difficult-to-diagnose cases of odontogenic infection and antiresorptive agent-related osteonecrosis of the jaw (ARONJ) with facial subcutaneous abscesses by otolaryngologists.MethodsThe study was conducted in the department of otolaryngology of a university hospital. Seven patients exhibiting odontogenic infection and ARONJ with facial subcutaneous abscesses diagnosed at the department of otolaryngology in our hospital from January 2008 to December 2018 were included in the study. We investigated the following information obtained from the patients: clinical department for initial treatment, sex, age, offending tooth, causative disease, site of the fistula, symptoms, methods of diagnoses, and therapy.ResultsOdontogenic infection with facial subcutaneous abscess formation can occur in individuals in a wide range of age groups; however, the pathological manifestations of ARONJ are often observed in older women, frequently at the chin and accompanied by nodules and pain in the adjacent teeth. Computed tomography and orthopantomography are useful for diagnosis and are especially indicated for patients under long-term follow-up or with frequent recurrences. Magnetic resonance imaging, cytodiagnosis, and histological analysis might be necessary to exclude the possibility of tumors. Management of such facial subcutaneous abscesses comprises dental treatment and infection control, and f surgical removal of the abscess is usually not required. Treatments such as sequestrectomy and sitafloxacin administration are useful for patients with ARONJ.ConclusionOur results demonstrated that for patients with facial subcutaneous abscesses, involvement of odontogenic infection and ARONJ should be considered.     

Odontogenic lesions in a pediatric population: Review of the literature and presentation of 745 cases

ObjectivesPediatric odontogenic lesions are rare clinical entities and are not well addressed in the otolaryngology literature. Knowledge of the biologic and clinical behavior of these lesions and their basic features such as location and age are key aspects for otolaryngologists in developing an early clinical differential diagnosis, such that they can provide adequate treatment.MethodsSix thousand histologically-diagnosed odontogenic lesions from a 40-year period were reviewed. Pediatric patients (745 cases, 12.7%) aged up to 17 years were selected and reevaluated. The patients were divided into three age groups according to dentition periods: primary dentition (0–5 years), mixed dentition (6–12 years), and permanent dentition (13–17 years). Clinical data, including age, sex, and location, were collected from pathology records.ResultsOf the total of 745 cases, 596 specimens (80%) were diagnosed as odontogenic cysts and 149 specimens (20%) were diagnosed as odontogenic tumors. The most frequent odontogenic cysts were radicular cysts (48.4%), followed by dentigerous cysts (16.7%). The most common odontogenic tumor was odontoma (8.05%).ConclusionThis study shows that all odontogenic lesions in the pediatric group are benign. Odontogenic tumors are relatively rare in this age group. Knowledge of the biological and histopathological behaviors of odontogenic lesions and their basic features such as location and age is a key aspect for developing an early clinical differential diagnosis and providing adequate treatment.     

Sclerosing odontogenic carcinoma — review of all published cases: is it a justifiable addition as a malignancy?

IntroductionSclerosing odontogenic carcinoma was a new addition to the list of head and neck tumors by World Health Organization in 2017. This lesion has scarcely been reported and a lack of pathognomonic markers for diagnosis exists.ObjectiveThe aim of the study was to summarize findings from the available literature to provide up-to-date information on sclerosing odontogenic carcinoma and to analyse clinical, radiological, and histopathological features to obtain information for and against as an odontogenic malignancy.MethodsWe conducted a comprehensive review of literature by searching Pubmed, EBSCO and Web of Science databases, according to PRISMA guidelines. All the cases reported as sclerosing odontogenic carcinoma in English were included. Data retrieved from the articles were gender, age, clinical features, site, relevant medical history, radiographical findings, histopathological findings, immunohistochemical findings, treatments provided and prognosis.ResultsMean age at diagnosis of sclerosing odontogenic carcinoma was 54.4 years with a very slight female predilection. Sclerosing odontogenic carcinoma was commonly reported in the mandible as an expansile swelling which can be asymptomatic or associated with pain or paraesthesia. They appeared radiolucent with cortical resorption in radiograph evaluation. Histologically, sclerosing odontogenic carcinoma was composed of epithelioid cells in dense, fibrous, or sclerotic stroma with equivocal perineural invasion. Mild cellular atypia and inconspicuous mitotic activity were observed. There is no specific immunohistochemical marker for sclerosing odontogenic carcinoma. AE1/AE3, CK 5/6, CK 14, CK19, p63 and E-cadherin were the widely expressed markers for sclerosing odontogenic carcinoma. Surgical resection was the main treatment provided with no recurrence in most cases. No cases of metastasis were reported.ConclusionFrom the literature available, sclerosing odontogenic carcinoma is justifiable as a malignant tumor with no or unknown metastatic potential which can be adequately treated with surgical resection. However, there is insufficient evidence for histological grading or degree of malignancy of this tumor.     

Ameloblastic fibroma of the maxillary sinus

Ameloblastic fibroma is a rare true mixed tumor of odontogenic origin with both mesenchymal and ectodermal components. It usually arises from the mandibular dentition and presents in the second decade of life. It is a benign slow growing tumor that is less infiltrative than an ameloblastoma but tends to expand bone. It infrequently involves the maxilla and hence the management of these tumors in that region is controversial. The goal of therapy is to avoid cosmetic deformity without compromising on tumor eradication. We present the tenth reported case of this type of tumor in the maxilla, and the second one in an infant.     

Pharyngocutaneous fistula following total laryngectomy

Pharyngocutaneous fistula (PCF) is the most common complication after total laryngectomy.ObjectivesTo establish the incidence of this complication and to analyze the predisposing factors.MethodThis is a cross-sectional study of a historical cohort including 94 patients who underwent total laryngectomy. The following aspects were correlated to the occurrence of PCF: gender, age, tumor site, TNM staging, type of neck dissection, previous radiation therapy, previous tracheotomy, and use of stapler for pharyngeal closure. The following were considered in PCF cases: the day into postoperative care when the fistula was diagnosed, duration of occurrence, and proposed treatment.ResultsTwenty (21.3%) patients had PCF. The incidence of PCF was statistically higher in T4 tumors when compared to T2 and T3 neoplasms (p = 0.03). The other analyzed correlations were not statistically significant. However, 40.9% of the patients submitted to tracheostomy previously had fistulae, against 21.1% of the patients not submitted to this procedure.ConclusionAdvanced primary tumor staging is correlated with higher incidences of PCF.     

Cone beam computed tomography evaluation of the relationship between atlantodental interval and skeletal facial morphology in adolescents

IntroductionIn the pediatric population, computed tomography examination of the upper cervical spine plays an important role in the diagnosis of neurological injuries involving that region. Due to the interconnected nature of the craniofacial structures, a structural change in one is expected to also cause changes in the other structures.ObjectiveThe aim of this study was to evaluate relationships between atlantodental interval, cervical vertebral morphology, and facial structure in healthy adolescents using cone beam computed tomography.MethodsThirty subjects aged 14–20 years (10 males, mean age: 17.2 years; 20 females, mean age: 17.9 years) were included in the study. The anterior, lateral and posterior atlantodental intervals, and vertical and anteroposterior dimensions of the first and second cervical vertebrae were evaluated from cone beam computed tomography images. Facial morphology was evaluated using 7 parameters on lateral cephalometric cone beam computed tomography images and 6 parameters on posteroanterior images. The Mann–Whitney U test and Wilcoxon test were used for statistical analyses.ResultsComparisons between males and females showed that most parameters were larger in males, with significant differences in vertical facial dimensions (anterior lower face height: p = 0.05; anterior upper face height: p = 0.001), (distance between the most internal point of the frontozygomatic suture and midsagittal reference plane; p = 0.01), (the distance between the deepest point of the right alveolar maxillar process and midsagittal reference plane; p = 0.001), and C2 vertebral dimensions. The anterior and lateral atlantodental interval values correlated with maxilla position relative to the mandible angle, and the anterior atlantodental interval correlated with lower anterior facial height (p = 0.05). Dimensional measurements of the C1 and C2 vertebrae were correlated with both anterior facial heights and some posteroanterior parameters.ConclusionSagittal, vertical, and transverse facial dimensions and positions were strongly associated with C1 and C2 vertebral dimensions, and the maxillomandibular relationship may affect atlantodental interval. Therefore, including craniofacial features in assessment of the atlantodental area and vertebral distances in adolescents may be beneficial.     

Clinicopathologic characteristics of laryngeal chondrosarcoma: An analysis of the National Cancer Database

ObjectivesLaryngeal Chondrosarcoma (LC) is a rare malignancy with limited studies documenting its clinicopathologic characteristics and treatment options. This study reports demographic and clinical determinants of outcomes for this rare tumor.MethodsThe National Cancer Database (NCDB) was queried for cases of LC reported from 2004–2016. 274 cases that met inclusion criteria were analyzed for demographic and clinicopathologic characteristics. Kaplan-Meier (KM) and Cox proportional hazard analyses were conducted to identify variables that impacted the overall survival of these patients.ResultsLC was found to be more common in males (74.8%). The mean age of patients was 61.8 years and 92.3% of the patients were white. 91.3% of patients were treated with only surgical resection, most commonly: partial laryngectomy (31.6%), total laryngectomy (25.7%), and local resection (22.4%). 98.8% of patients had no evidence of nodal disease and 99.6% of patients did not have distant metastasis at presentation. KM analysis revealed a 5-year overall survival (5YOS) of 89.0%. Age, insurance status, facility type, and surgery type were significant predictors of 5YOS (p<0.05). On Cox Proportional Hazard analysis, private insurance significantly improved survival (HR 0.21; p = 0.048) while increasing age was a poor prognostic indicator (HR 1.10; p = 0.004).ConclusionThe majority of LC patients present with no nodal involvement or distant metastasis at diagnosis, and overall this tumor has a favorable prognosis. Increasing age was found to be a poor prognostic factor while private insurance status was associated with improved survival.     

Structured histopathology and laboratory evidence in nasal polyposis with different pathogenesis

PurposeNon-steroidal anti-inflammatory drugs-exacerbated respiratory disease (NERD), intrinsic asthma, eosinophilic granulomatosis with polyangiitis (EGPA) and odontogenic sinusitis may be associated with nasal polyps. The aim of the study was to compare circulating inflammatory cells and structural histopathology of these groups of nasal polyposis.MethodsWe retrospectively evaluated 71 patients with nasal polyps stratified according to the above-mentioned pathogenesis. All patients underwent preoperative laboratory investigations and primary endoscopic sinus surgery. Surgical specimens were submitted to structured histopathological evaluation.ResultsThe median tissue eosinophil count (cells/HPF) was significantly different between the considered groups of nasal polyposis (p=0.0004). The median of NERD sub-cohort was significantly higher than intrinsic asthma (p=0.0030), odontogenic CRS (p=0.0001) and EGPA ones (p=0.0094). Eosinophilic aggregates positive rate was significantly higher in NERD sub-cohort than in odontogenic CRS (p=0.0072), EGPA (p=0.0497) and asthma (p=0.0188) ones. EGPA sub-cohort had a higher neutrophil infiltrate positive rate than NERD (p=0.0105) and intrinsic asthma ones (p=0.0040). Odontogenic CRS sub-cohort had a higher neutrophil infiltrate positive rate than NERD (p=0.0140) and asthma ones (p=0.0096). EGPA sub-cohort had a higher presence of fibrosis than NERD (p=0.0237) and odontogenic CRS sub-cohort (p=0.0107). Odontogenic sub-cohort had a lower sub-epithelial edema positive rate than NERD (p=0.0028) and asthma (p=0.0149) ones.ConclusionsStructural histopathology may identify nasal polyps histotypes with different morphological patterns. The identified histopathological features can facilitate the recognition of rational therapeutic and follow-up approaches that consider the tissue modifications associated with the response to drugs and surgery.     

Palatal growth in complete unilateral cleft lip and palate patients following neonatal cheiloplasty: Classic and geometric morphometric assessment

BackgroundA new method of early neonatal cheiloplasty has recently been employed on patients having complete unilateral cleft lip and palate (cUCLP). We aimed to investigate (1) their detailed palatal morphology before surgery and growth during the 10 months after neonatal cheiloplasty, (2) the growth of eight dimensions of the maxilla in these patients, (3) the development of these dimensions compared with published data on noncleft controls and on cUCLP patients operated using later operation protocol (LOP; 6 months of age).MethodsSixty-six virtual dental models of 33 longitudinally evaluated cUCLP patients were analysed using metric analysis, a dense correspondence model, and multivariate statistics. We compared the palatal surfaces before neonatal cheiloplasty (mean age, 4 days) and before palatoplasty (mean age, 10 months).ResultsThe palatal form variability of 10-month-old children was considerably reduced during the observed period thanks to their undisturbed growth, that is, the palate underwent the same growth changes following neonatal cheiloplasty. A detailed colour-coded map identified the most marked growth at the anterior and posterior ends of both segments. The maxilla of cUCLP patients after neonatal cheiloplasty had a growth tendency similar to noncleft controls (unlike LOP).ConclusionsBoth methodological approaches showed that early neonatal cheiloplasty in cUCLP patients did not prevent forward growth of the upper jaw segments and did not reduce either the length or width of the maxilla during the first 10 months of life.     

Treatment and outcomes of endoscopic surgery and traditional open resection in sinonasal mucosal melanoma

Objective: The optimal surgical approach for sinonasal mucosal melanoma (SNM) remains under debate. We aimed to compare the therapeutic efficacy of traditional open resection and endoscopic surgery for patients with SNM treated at a single center.

Methods: Thirty-three patients diagnosed with SNM who underwent surgery between January 1995 and June 2014 at a single institution were retrospectively analyzed; 18 patients were treated using an open resection approach and 15 using an endoscopic resection approach for the primary tumor. The associations between open resection and endoscopic resection with treatment-related survival outcomes were assessed using the Kaplan–Meier method and log-rank test.

Results: The most common presenting symptoms were epistaxis and abstraction; the nasal cavity was the most common anatomic location. The open resection and endoscopic resection groups did not have significantly different demographic or tumor characteristics. Overall survival (p?=?.66) and disease-free survival (p?=?.73) were modest and did not differ significantly between the open resection and endoscopic resection groups.

Conclusions: This retrospective study indicates that the endoscopic endonasal approach is an effective treatment for SNM in selected cases and, when performed by a skilled surgeon, can enable successful radical resection with a similar efficacy as traditional open resection.     

Seasonality of head and neck cancers

PurposeThe purpose of this study is to investigate if the season of diagnosis is associated with patient, tumor, and treatment characteristics within head and neck cancer.Materials and methods1406 patients with a diagnosis of head and neck cancer (HNC) were identified from a HNC database (1996–2019). Patients were classified as receiving a diagnosis in the winter, spring, summer, or fall by calendar definition. Proportions and chi-squared analysis compared patient, tumor, and treatment factors for all diagnoses. Data was subdivided and analyzed based on the primary site.ResultsFrom this cohort, 23 %, 27 %, 25 %, and 25 % of HNC patients were diagnosed in winter, spring, summer, and fall respectively with no statistically significant difference between seasons of diagnosis. When subdivided by primary site, oral cavity cancer was significantly more likely to be diagnosed in spring, salivary gland cancer was more likely to be diagnosed in winter and summer (p = 0.03 and p = 0.01 respectively). No other demographic, clinicopathologic, or management characteristics were associated with the season of diagnosis (p > 0.05 for all).ConclusionsDiagnosis of head and neck cancer does not follow a seasonal pattern. Diagnosis of oral cavity and salivary gland cancer showed a seasonal pattern. The majority of patient, tumor and management characteristics were not associated with the.     

Is the occurrence of spontaneous epistaxis related to climatic variables? A retrospective clinical,epidemiological and meteorological study

Conclusion: Epistaxis has a bimodal age distribution. Anterior epistaxis of mild severity is more common in children; severe epistaxis occurs more often in adults and elderly patients. The occurrence of spontaneous epistaxis was shown to be weakly-to-moderately and inversely correlated to the mean monthly temperature, relative humidity and total rainfall.

Objectives: To describe the clinical and epidemiological profiles of patients diagnosed with spontaneous epistaxis; to correlate its monthly occurrence with meteorological variables.

Methods: A retrospective cross-sectional study was performed in a referral ENT service in São Paulo, Brazil. The study assessed the clinical and epidemiological features of 508 patients with spontaneous epistaxis treated between February 2010 and January 2011. The occurrence of epistaxis was associated with weather variables for the same study period.

Results: Spontaneous epistaxis presented two age peaks of higher incidence (11–20 and 51–70 years). Children more frequently had localized bleeding (p?=?0.003), in the anterior region of the nasal cavity (p?=?0.0001), of absent-mild severity (p?=?0.0082). Diffuse bleeding (p?=?0.005), of moderate–severe intensity (p?=?0.003), was more common in adults and elderly patients. The total number of visits because of epistaxis was inversely correlated to mean temperature (R?=??0.489; p?=?0.011), mean relative humidity (R?=??0.364; p?=?0.038), and total rainfall (R?=??0.512; p?=?0.009) during each month of the analyzed period.     

72例咽旁隙肿瘤的临床诊治

目的 分析并总结咽旁隙肿瘤的临床特点、手术方法及预后情况。方法 回顾性分析南京医科大学第一附属医院2012年11月—2022年11月收治的咽旁隙肿瘤患者的临床资料,排除影像学资料不全及失访的患者共筛选到72例,其中男36例,年龄27~72岁,平均年龄（46.06±1.72）岁;女36例,年龄21~77岁,平均年龄（53.67±4.88）岁。所有患者均根据术前查体及影像学检查制定手术方案,经口径路27例,颈侧径路45例。术后随访2～135.9个月,中位随访时间54.38个月。统计分析临床、影像及手术资料。结果 72例患者术后病理为良性肿瘤63例,其中上皮源性肿瘤29例,神经源性肿瘤30例,其他组织来源良性肿瘤4例;4例复发,其中2例神经鞘瘤,2例多形性腺瘤,余59例恢复良好;恶性肿瘤9例,其中鳞状细胞癌5例,恶性梭形细胞瘤1例,恶性淋巴瘤1例,滑膜肉瘤1例;3例复发。尽管良性咽旁隙肿瘤经口内镜手术存在囊内分块切除的情况,但相比整块切除的患者并未增加复发风险（P=0.381）。结论 咽旁隙肿瘤位置深在,临床结构复杂,良性肿瘤预后可,恶性肿瘤易复发转移,预后较差。根据术前查体和影像学检查制定手术径路至关重要。手术以颈侧开放径路为主要方式,内镜辅助径路需严格把握适应证,肿物长径较大时在包膜完整的情况下作囊内分块切除是可行的。     

Stromal and epithelial syndecan-1 expression in benign and malignant salivary gland tumors: which is more reflective of behavior?

IntroductionSalivary gland tumors are a diverse group of lesions, with various origins and extremely different behaviors, leading to a variety of outcomes for patients. Therefore, the need to discover novel markers with the ability to predict the behavior of benign and malignant salivary gland neoplasms is crucial. Syndecan-1 is a cell-surface protein with significant roles in various aspects of tumor function. Its expression in salivary gland neoplasms, especially their stromal component, has not been investigated.ObjectivesWe aimed to assess the immunopositivity of syndecan-1 in epithelial and stromal components of salivary gland neoplasms and to compare it between benign and malignant subtypes in addition to evaluating its correlation with clinicopathologic parameters.Methods133 salivary gland tumors were immunohistochemically stained with syndecan-1 and the intensity and percentage of this protein was determined, compared between the tumors and correlated with clinicopathologic factors.ResultsStatistical analysis of lesions with a sufficient sample size showed significant differences in percentage and intensity between both epithelial and stromal components of all tumors (p < 0.05). Pairwise-comparisons demonstrated significantly higher staining-percentage of epithelial cells (p = 0.02) in Warthin’s tumor compared to pleomorphic adenoma and adenoid cystic carcinoma. Similarly, significantly higher staining intensities and/or percentages was observed in mucoepidermoid carcinoma and adenoid cystic carcinoma compared to pleomorphic adenoma and Warthin’s tumor (p < 0.05). Of the clinicopathologic factors, there was only a significant negative correlation between stromal percentage of mucoepidermoid carcinoma and age and a significant difference between stromal intensity+percentage of adenoid cystic carcinoma and gender (p < 0.05).ConclusionsAccording to our findings we postulate that stromal syndecan-1 correlates with the behavior of salivary gland tumors, with malignant neoplasms demonstrating a higher expression, indicating a role for syndecan-1 in invasion and metastasis.     

Recurrent odontogenic myxoma of maxilla: a diagnostic and operative dilemma

Odontogenic myxoma of maxilla is a very rare occurrence. It is a slow growing benign, locally malignant tumor notorious for recurrence. Hereby we present a case of recurrent odontogenic myxoma of the maxilla in a 32-year-old male managed surgically by partial maxillectomy. The diagnostic and operative dilemmas encountered while managing the case are discussed in detail.     

Treatment decision and estimation of growth of head and neck paragangliomas

PurposeHead and neck paragangliomas are slow growing tumors where observation has become more widely accepted. Tumor growth rate as well as predictors of increased tumor growth were analyzed with the goal to identify factors to better predict disease progression and counsel patients.Materials and methodsMulti-institutional retrospective cohort study from 2011 to 2020.Results130 head and neck paragangliomas in 125 patients were analyzed. 38 were observed (30.4%), 16 radiated (12.8%), and 71 underwent surgery (56.8%). Surgical patients were significantly younger (p = 0.038) and with more genetically mediated paragangliomas (p = 0.026). Significantly more patients were asymptomatic in the observation group (p = 0.005). Of the 39 observed tumors, 43.6% (n = 17) grew with a tumor doubling time of 5.67 years. More than half of the observed paragangliomas had no growth. When examining symptoms postoperatively and at follow-up, the surgical cohort had significantly more worsening symptoms (p = 0.007) and new cranial neuropathies (p = 0.031).ConclusionsHead and neck paragangliomas have slow growth rates if they grow at all. Patients in the surgical cohort had more clinical symptoms at presentation and worsening postoperative symptoms.     

Aktivierung des Sonic-hedgehog-Signalwegs in keratozystischen odontogenen Tumoren

Background

Keratocystic odontogenic tumors are benign neoplasms of the viscerocranium that occur sporadically as well as in association with Gorlin–Goltz syndrome. Multiple basal cell carcinomas of the skin are another typical feature of Gorlin–Goltz syndrome. Aberrant activation of sonic hedgehog signaling has been reported for sporadic and hereditary basal cell carcinoma caused by specific genetic mutations, but for keratocystic odontogenic tumors, the role of aberrant sonic hedgehog signaling has not yet been evaluated in detail.

Materials and methods

In the present study, 131 keratocystic odontogenic tumors were analyzed by immunohistochemistry for the expression of sonic hedgehog signaling proteins SHH, PTCH1, SMO, GLI1, and NMYC on tissue microarray sections.

Results

High expression of the analyzed proteins—between 67.3% (PTCH1) and 92.9% (SHH)—was found in the epithelial compartment of the keratocystic odontogenic tumors analyzed. In the stromal compartment of the tumors, high expression of the target proteins was found significantly less frequently (all p-values <0.001).

Conclusion

Aberrant sonic hedgehog signaling is critically involved in the molecular pathogenesis of keratocystic odontogenic tumors. This finding underlines the neoplastic character of this intraosseous lesion. Because of high recurrence rates after local excision, more radical surgical approaches are recommended for treating keratocystic odontogenic tumors.