Primary Intracranial Adenoid Cystic Carcinoma: Report of Three Cases.

Objective Nasal cavity and paranasal sinuses host a variety of malignant tumors with adenoid cystic carcinoma (ACC) being the most frequent cancer of salivary gland origin. The histological origin of such tumors virtually precludes primarily intracranial localization. The aim of this study is to report cases of primarily intracranial ACC without evidence of other primary lesions at the end of an exhaustive diagnostic workup. Methods An electronic medical record search complemented by manual searching was conducted to identify prospective and retrospective cases of intracranial ACCs treated in Endoscopic Skull Base Centre Athens at the Hygeia Hospital, Athens from 2010 until 2021 with a mean follow-up time of at least 3 years. Patients were included if after complete diagnostic workup there was no evidence of a nasal or paranasal sinus primary lesion and extension of the ACC. All patients were treated with a combination of endoscopic surgeries performed by the senior author followed by radiotherapy (RT) and/or chemotherapy. Results Three unique illustrative cases (ACC involving the clivus, cavernous sinus and pterygopalatine fossa, one orbital ACC with pterygopalatine fossa and cavernous sinus involvement and one involving cavernous sinus, and Meckel's cave with extension to the foramen rotundum) were identified. All patients underwent subsequently proton or carbon-ion beam radiation therapy. Conclusions Primary intracranial ACCs constitute an extremely rare clinical entity with atypical presentation, challenging diagnostic workup and management. The design of an international web-based database with a detailed report of these tumors would be extremely helpful.

Eight Month-Old-Infant Impaled by Toothbrush Through the Hard Palate.

Impalement of children with foreign bodies is not uncommon. A variety of foreign bodies and mechanisms of injuries have been described. Infant tissues are more vulnerable to injuries and often penetrating traumas are very dangerous. An 8-month-old infant was referred to our department with a major impalement injury of his hard palate by a toothbrush. The integrity of the orbit was in question due to the high proximity of the foreign body to the lamina papyracea. A well-structured algorithm was followed to secure the best interests of the child. Our approach minimizes the risk of complications by taking into account every single parameter. After the removal of the foreign body, a careful reconstruction with meticulous nasendoscopy was held to restore the hard palate integrity and to check the orbit inner wall. According to the literature, toothbrushes seem to be the most common medium of impalement trauma. Parents should be educated that their offspring are at great risk when holding or sucking objects while playing.

Quality of life after mastoid surgery. Is there a real benefit to preserving the posterior wall of the external auditory canal? A systematic review and meta-analysis.

OBJECTIVE: Canal wall up (CWU) and canal wall down (CWD) mastoidectomies represent the most common cholesteatoma surgical techniques. In this meta-analysis, we compare the postoperative quality of life (QoL) in patients treated with either CWU or CWD mastoidectomy. METHODS: A literature search was conducted in the following three electronic databases: Medline/PubMed, ScienceDirect, and Google Scholar. Articles were assessed for eligibility in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement and data were extracted independently by two authors. Biases assessment was conducted for each study according to the Methodological Items for Non-Randomized Studies (MINORS) tool. Meta-analysis was performed for postoperative QoL following CWU versus CWD mastoidectomy. RESULTS: Our systematic review included four studies that met the inclusion criteria, three prospective cohort studies, and one retrospective cohort study. The meta-analysis did not favor treatment with one of the two surgical techniques. Postoperative QoL did not show a statistically significant difference between CWU and CWD mastoidectomies (p>0.05). CONCLUSION: Our systematic review and meta-analysis results indicate that QoL is not statistically significantly better among patients who underwent CWU mastoidectomies when compared with CWD. The trend of selecting CWU over CWD mastoidectomies in selected cases - for QoL purposes - is not always based on evidence-based data. The statistically insignificant difference between the two surgical techniques suggests that an initial more radical approach might prevent patients from further surgeries, without affecting postoperative QoL.

Early Internal Branch of Superior Laryngeal Nerve Bifurcation Passes Through Double Thyroid Foramen.

Internal branch of superior laryngeal nerve (ibSLN) provides sensory innervation mostly to the supraglottic part of the larynx and thus prevents aspiration during ingestion. Normally, it is distributed to the larynx after piercing the thyrohyoid membrane above the superior laryngeal artery. Multiple anatomical variations in the course of ibSLN have been reported. An early ibSLN bifurcation and course through double thyroid foramen constitutes an interesting anatomical variation that may easily lead to an injury during procedures in the thyroid gland and the larynx. Knowledge of the anatomical variability is essential in order to prevent surgical complications that could potentially impact the patient's quality of life.

Sigmoid Sinus Thrombosis as a Complication of Acute Otitis Media in a 6-Year-Old Male.

Sigmoid sinus thrombosis (SST) is a potentially life-threatening complication of otitis media which is nowadays rare due to the widespread use of antibiotics. A high index of suspicion is necessary to allow for a timely diagnostic and therapeutic intervention. Intravenous wide-spectrum antibiotics and a cortical mastoidectomy are the mainstay of treatment. There is no consensus regarding the necessity of anticoagulants in pediatric patients. We present a 6-year-old boy who presented with an SST as a result of acute otitis media.

Clival Mucocele: A Rare Yet not Forgotten Pathology.

Primary clival mucoceles are a rare clinical entity that usually represents an incidental finding on computed tomography or magnetic resonance imaging scanning. There are only a few reports in the literature of patients who presented with vague symptoms such as headaches, facial paresthesia, and numbness. Clival mucoceles can also be secondary, by extension of a sphenoid mucocele to the clivus. We present a case of primary clival mucocele, aiming to highlight the importance of a multidisciplinary approach.

A Rare Case of Hemoptysis Caused by a Laryngeal Leech.

The larynx is an uncommon location for live foreign bodies. The leech can reach the glottis during consumption of contaminated water but is usually expelled by an effective cough reflex. Patients present with hoarseness and dysphagia and occasionally with dyspnea or hemoptysis. Endoscopically, a mobile mass is usually noted in the supraglottic area. We present a rare case of a laryngeal leech in a 62-year-old-male farmer who lives in a rural area. The leech was removed successfully with direct laryngoscopy under general anesthesia.

A Case Report of Glomus Tympanicum Complicated With Facial Nerve Palsy.

Introduction: Generally, glomus tumors are considered tumors of the autonomic system arising from chromaffin cells of the parasympathetic paraganglia of the skull base and neck. Glomus tympanicum is the most common primary tumor of the middle ear cavity and it arises from the paraganglia of the middle ear. Case Report: We present a case of glomus tympanicum presented in a 70-year-old woman, complicated with facial nerve palsy which at first sight was misdiagnosed as cholesteatoma. Patient presented in our clinic because of otorrhea, pulsatile tinnitus and hearing loss in the right ear. However, facial nerve function was good in the first examination (40 days before the surgery). Eventually, she treated successfully with a canal wall down mastoidectomy. Technique had been chosen because of the mass size and the involvement of external auditory canal, after a discussion with the patient. Conclusions: Although histologically benign, glomus tympanicum is slow growing and destructs adjacent tissues potentially. The two most common complaints are hearing loss (conductive) and pulsatile tinnitus. These neoplasms are more common in women and they can be diagnosed by CT or MRI scan. It is of high importance physicians suspect a glomus tumor when patient 's clinical findings are hearing loss and pulsatile tinnitus and use an intravascular agent in imaging so that the differential diagnosis will be supported.

Late Transformation of a Dermatofibrosarcoma Protuberans Neck Sarcoma to a Neoplasm With Fibrosarcomatous Elements: Lessons Learned.

Dermatofibrosarcoma protuberans is a low-grade soft tissue sarcoma not often related to the head and neck area. Distant metastases are not often recorded. Wide surgical excision is considered the most appropriate treatment modality. In cases of insufficient or positive surgical margins, the role of adjuvant radiation therapy or targeted therapy might be beneficial. Dermatofibrosarcoma protuberans transformation to a neoplasm with fibrosarcomatous elements is extremely rare. The presence of fibrosarcomatous elements is associated with more aggressive behavior of the tumor and increases significantly its metastatic potential. We present a rare case of head and neck dermatofibrosarcoma protuberans with multiple local recurrences 13 years after the initial diagnosis.

Adenoviral Infections in Neonates: A Case-Based Literature Review.

Adenoviral infections in neonates are associated with high rates of mortality due to the lack of humoral immunity. A comprehensive search of published literature in PubMed, Google Scholar, and Science Direct electronic databases was conducted for case reports published between the years 1990 and 2021. The aim of our study is to investigate the risk factors, clinical manifestations, treatment, and outcomes of adenoviral infections in neonates. In our study, 36 cases were included. The most common type of infection was disseminated one (14/36, 38.8%), followed by adenoviral pneumonia (13/36, 36.1%). Cidofovir was administered in seven cases (19.4%), and death was reported in six of them. One preterm low birthweight neonate with disseminated adenoviral infection was treated with a combination of cidofovir, intravenous immune globulin, and haploidentical virus-specific T lymphocytes (VSTs) and survived. In this review, we found a statistically significant difference in the outcome based on the type of adenoviral infection (p=0.001). Disseminated infection and pneumonia are associated with the worst prognosis. In addition, mortality was observed to be higher in neonates with disseminated disease in contrast to neonates with localized infection (p=0.002). However, the antiviral treatment had no statistically significant effect on the mortality rate (p=0.137). There is a necessity for further investigation and randomized studies to validate the results of the present study.

Does the orientation of the styloid process cause Eagle Syndrome? An anatomical study of the styloid process in 72 Greek skulls.

<b>Introduction:</b> Patients often suffer from an elongated styloid process. This clinical entity is well known and is described as Eagle syndrome. The presence of this anatomical variation is not always associated with symptoms. However, there is a strong correlation between the elongated styloid process and Eagle syndrome </br></br> <b>Aim:</b> This study aims to calculate the incidence of elongated styloid processes in the Greek population, to analyze the morphometric characteristics of styloid processes, and to compare these among sexes. </br></br> <b>Material and methods:</b> This is a single-center retrospective study. All skulls were donated to the Department of Anatomy, the Ari-stotle University of Thessaloniki from January 2013 to May 2019 for research purposes. A styloid process longer than 30 mm was considered elongated. We used Welch's t-test for the statistical analysis of the data. </br></br> <b>Results:</b> The prevalence of elongated styloid processes was 35%. The average diameter was 3.32 mm and the average total length was 25.02 mm. There was no statistically significant difference between male and female skulls, though the female styloid processes were slightly longer and the male ones were slightly wider. Symmetry was recorded in 31% of skulls. Average axial angle was 650 but in 4% of cases it was less than 20⁰. </br></br> <b>Conclusions:</b> The elongated styloid process is not a rare entity. Eagle syndrome should always be considered in the differential diagnosis when patients report chronic sore throat. Our anatomical findings add a new dimension to the etiopathogenesis of Eagle syndrome.

Cross-Cultural Adaptation and Validation of the Greek SPOT-25 Quality of Life Questionnaire in Patients with Otosclerosis.

Objective: To translate, adapt and validate in Greek the stapesplasty outcome test (SPOT)-25 quality of life questionnaire for patients with otosclerosis. Materials and methods:SPOT-25 was translated to Greek and completed by otosclerosis patients on the day of diagnosis, the day before surgery and three months postoperatively. Fifty controls without any otological history, symptom or finding also completed the questionnaire. Pure-tone average was obtained both preoperatively and three months postoperatively. Results:Test-retest evaluation on 56 patients was accepted. The Greek-SPOT-25 had an excellent internal consistency. All its items and subscales were significantly correlated between test and retest evaluation. Controls had significant lower SPOT-25 scores, and the postoperative scores were significantly lower than preoperative ones. Pure-tone average of four frequencies (PTA4) was significantly correlated to preoperative SPOT-25 total and subscales scores (P<0.001) before surgery and significantly correlated only with the "hearing function" subscale (p<0.05) postoperatively.

Epigenetic editing and tumor-dependent immunosuppressive signaling in head and neck malignancies.

Head and neck cancer (HNC) comprises a heterogeneous variety of malignant tumors, characterized by a relatively high tumor mutation burden. Previous data have revealed that immune system dysfunction appears to serve a key role in the development and progression of HNC and established immunosuppression is vital for evading the host immune response. Despite progress in chemotherapy and radiotherapy, the survival rate of patients with HNC is still low. Therefore, the present review discusses the development of novel immunotherapy approaches based on the various immune cell signaling routes that trigger drug resistance and immunosuppression. Additionally, the present review discusses the epigenetic alterations, including DNA methylation, histone modifications, chromatin remodeling and non-coding RNAs that drive and support HNC progression. Furthermore, the role of cancer-associated fibroblasts, tumor macrophages and myeloid cells in tumor-related immunosuppression are considered. Specifically, the molecular immune-related mechanisms in the tumor microenvironment, which lead to decreased drug sensitivity and tumor relapse, and strategies for reversing drug resistance and targeting immunosuppressive tumor networks are discussed. Deciphering these molecular mechanisms is essential for preclinical and clinical investigations in order to enhance therapeutic efficacy. Furthermore, an improved understanding of these immune cell signaling pathways that drive immune surveillance, immune-driven inflammation and tumor-related immunosuppression is necessary for future personalized HNC-based therapeutic approaches.

Swallowing disorders after thyroidectomy: a systematic review and meta-analysis.

PURPOSE: Swallowing disorders following thyroidectomy are common, even after surgery without confirmed complications. The purpose of the current systematic review is to investigate the prevalence of dysphagia at various time points after thyroidectomy, at the whole spectrum of it (total/partial, open/endoscopic, for benign/malignant disease). METHODS: The literature available at PubMed, SciELO and Cochrane Library databases was reviewed, according to PRISMA guidelines, using the terms "dysphagia", "swallowing disorder", "deglutition disorder", "thyroidectomy" and "thyroid surgery" in the appropriate combinations. A quantitative synthesis of the results followed. RESULTS: The systematic review of the literature resulted in 35 articles, which met the inclusion criteria and were analyzed regarding their type, sample, follow-up and results regarding post-thyroidectomy dysphagia in multiple follow-up times. A significant increase of swallowing impairment compared to baseline was recorded shortly after surgery. Dysphagia reverted to pre-operative levels 2-3 months later. Dysphagia continued to be reported in a significantly lower proportion of patients, even 1 year after surgery. No significant difference was noticed between open and endoscopic thyroid surgery at 2-3 months post-surgery. CONCLUSIONS: The swallowing disorders reported after thyroidectomy should be expected, but are not always detectable through objective methods. This should not lead to underestimation of symptoms, since the patients' quality of life is negatively affected by the symptomatology.