Neuroendocrine and undifferentiated sinonasal and skull base tumors: An up-to-date narrative review.

Tumors located in the nasal cavity, paranasal sinuses and the skull base comprise a wide range of histologic subtypes. Among them, neuroendocrine and undifferentiated tumors are rare but noteworthy, because of their distinctive features, aggressive nature, and diagnostic complexities. A literature search was conducted in the PubMed/MEDLINE and the Scopus databases from 2019 until inception. The keywords "neuroendocrine", "undifferentiated", "nose", "sinonasal", "paranasal", "skull base" were used. Thirty-eight articles referring to neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base were finally included and analyzed. Neuroendocrine and undifferentiated tumors of the nose, paranasal sinuses and the skull base are infrequent malignancies, most commonly affecting middle-aged men. They usually present with non-specific symptoms, even though ocular or neurologic manifestations may occur. Prognosis is generally poor; however, novel targeted and immunological therapies have shown promising results. Sinonasal Neuroendocrine Carcinomas (SNECs) carry distinct histological and immunohistochemical features. Management consists of surgical resection coupled with systematic therapy. Sinonasal Undifferentiated Carcinomas (SNUCs) lack specific squamous or glandular features. They typically stain positive for pancytokeratin and INI1 antibody. Treatment includes induction chemotherapy, followed by a combination of chemotherapy and radiotherapy. Olfactory neuroblastomas (ONBs) have neuroepithelial or neuroblastic features. They show diffuse positivity for various markers, including synaptophysin, chromogranin, and neuron-specific enolase (NSE). Surgical resection plus radiotherapy is considered the treatment of choice. In conclusion, neuroendocrine and undifferentiated tumors arising from the nose, paranasal sinuses and the skull base represent a unique group of malignancies. A thorough understanding of their clinical features, molecular changes, diagnostic approaches, treatment modalities, and prognostic factors is critical for providing optimal patient care. Still, continued research efforts and multidisciplinary collaboration are warranted, in order to improve outcomes for patients diagnosed with these rare and aggressive tumors.

Holmium:YAG laser-assisted intraductal sialendoscopic lithotripsy under local anesthesia.

OBJECTIVES: This retrospective multi-institutional chart review study aims to present results from patients treated with Holmium:YAG laser-assisted sialendoscopic intraductal lithotripsy under local anesthesia. METHODS: Preoperative ultrasound and/or CT were used for location and measurement of the stones. Local anesthesia was applied in all patients, Marchal all-in- one 1.3 mm and Erlangen 1.6 sialendoscopes were used. The laser was used with power settings limited by discomfort or pain. Inclusion criteria were stone size (all >5 mm) and fixed stones. RESULTS: 42 patients, 48 stones were treated (30 males/12 females), 21 submandibular and 21 parotid. Ages ranged from 20 to 70 years (95 % CI: 43.37-51.58). Mean size was 6.2 mm (95 % CI: 5.71-6.72). Complete fragmentation was achieved in 66.7 % and incomplete in 33.3 %. Out of the incomplete fragmented 14 cases, seven (50 %) remained symptom free on follow-up. Maximum duration was 120 min. Minimum power settings of the laser was 4.8 Watts and maximum 18 Watts. Six patients expressed pain or discomfort that limited power increase and duration of the procedure. Total follow up time was 10 years. There were no major complications such as hemorrhage, nerve paresis or skin ulceration. CONCLUSION: The procedure is safe under local anesthesia, well tolerated by most patients and should be used in cases of small fixed and "intermediate-sized" stones as a single modality. Discomfort may limit power settings and duration. The later is the major disadvantage of the method.

Sinonasal Neuroendocrine Carcinoma in Adult Proteus Syndrome.

Introduction: Proteus syndrome (PS) is a rare genetic disorder usually caused by mutations in AKT1 or PTEN genes, characterized by multiple, asymmetric tissue overgrowth with high clinical variability. Sinonasal neuroendocrine carcinomas (SNEC) are exceptionally rare tumors encountered in the ethmoid sinus, nasal cavity, or maxillary sinus. Case Report: We report a 35-year-old patient with PS, who underwent successful surgical removal of a well-differentiated SNEC obstructing his nasal cavity and highlight the role of the otolaryngologist for safe airway management, minimal surgical intervention and coordination of the multidisciplinary care. Histologically, focally hyperplastic mucosal epithelium of respiratory type of the nasal chamber was noticed along with seromucinous glands and capillary congestion of the subepithelial fibrovascular tissue. The limited presence of neoplastic tissue with histomorphological and immunophenotypic features of a neuroendocrine neoplasm was focally observed. Tumor cells grow in the form of islets within a vascular stroma; these neoplastic cells are immunohistochemically positive for synaptophysin, CD56, EMA, Ki67 (low expression, cell proliferation rate: 2%), CD31, chromogranin and pancytokeratin AE1 / AE3 as well as for S-100 protein (weak intensity). Conclusions: This first description of a SNEC in a PS patient, might hint towards a common basis between the two conditions, due to the mosaic AKT1 variant and an activated AKT/PIK3CA/PTEN pathway.

A Systematic Review of Intracranial Complications in Adults with Pott Puffy Tumor over Four Decades.

The purpose of this study is to investigate the risk factors of intracranial complications in adult patients with Pott Puffy Tumor (PPT). A systematic review was conducted of clinical studies from January 1983 to December 2022 that reported on PPT adult patients. The full-text articles were reviewed for the patients' ages, sex, cultured organisms, surgical procedures, clinical sequalae, and underlying diseases that may affect the onset of intracranial complications in PPT adult patients. A total of 106 studies were included. Medical data were reviewed for 125 patients (94 males, 31 females). The median age was 45 years. A total of 52% had comorbidities, mostly head trauma (24.5%), sinus/neurosurgical operations (22.4%), immunosuppression conditions (13.3%), diabetes mellitus (9.1%), cocaine use (7.1%), or dental infections (6.1%). A total of 28 cultures revealed Streptococcus (22.4%), 24 contained staphylococci (19.2%), and 22 cultures contained other pathogens (17.6%). An amount of 30.4% developed intracranial complications, with the most common being epidural abscesses or empyemas (55.3%), as well as subdural (15.7%) and extradural lesions (13.2%). Age, DM, and immunosuppression conditions are significantly associated with intracranial complications (p < 0.001, p = 0.018 and p = 0.022, respectively). Streptococcus infection is associated with intracranial complications (p = 0.001), although Staphylococcus and other microorganisms are not. Surgical intervention, mainly ESS, and broad-spectrum antibiotics remain the cornerstones of treatment.

Silicone Cervical Lymphadenopathy: A Rare Complication After Breast Augmentation.

A 66-year-old female patient, who had undergone breast augmentation 10 years ago, presented with unilateral neck pain, dysphagia, and hoarseness. Subsequent imaging revealed right-sided supraclavicular and axillary lymphadenopathy and a breast implant rupture on the same side. A lymph node core biopsy under sonographic guidance revealed silicone lymphadenopathy. Implant extirpation was offered to the patient. Cervical lymphadenopathy warrants an initial workup to exclude sinister chronic inflammatory or malignant conditions. Nonetheless, in cases of diagnostic uncertainty and a history of breast augmentation, the otolaryngologist should be cognizant of distal silicone lymphadenopathy. This condition is associated with silicone leakage and lymphatic dissemination of silicone particles. Even though silicone-related granuloma formation is a rare entity, its incidence is slowly rising as the population that has undergone breast augmentation grows older.

Fourteen cases of acute invasive fungal rhinosinusitis: is there a place for less aggressive surgical treatment?

PURPOSE: Acute invasive fungal rhinosinusitis (AIFRS) is a rare, fulminant, angio-invasive infection with high mortality rate. Treatment of AIFRS includes aggressive surgical debridement combined with antifungal agents and treatment of the underlying disease. The aim of this study is to report our experience with this often fatal disease and estimate the long-term survival of AIFRS patients with more conservative surgery. METHODS: A retrospective study was performed on immunocompromised adults with AIFRS from January 2011 to December 2020. The diagnosis was histologically confirmed by tissue fungus invasion. We analyze epidemiological and microbiological findings, the underlying disease and discuss our patients' treatment plan and long-term survival. RESULTS: Fourteen patients were included in the study. Mean age was 53.8 ± 18.9 years. The underlying disease was hematologic malignancy in nine patients, diabetes mellitus in three, aplastic anemia in one, and renal/liver failure in one. Leukopenia was not significantly correlated with outcome or survival. Histological and culture findings revealed that Mucor/Rhizopus were the causative organisms in nine cases. All patients received systemic antifungal therapy. Surgery was performed in nine patients (endoscopic for all patients, combined in three with an external approach). Overall survival was 35.7% as five patients survived, two who underwent surgery, and three who were treated non-surgically. Nine patients died, two from AIFRS after central nervous system involvement and seven from their primary disease, free of fungi. Older individuals and patients with hematologic malignancies had a worse outcome. CONCLUSION: Early surgical debridement, antifungal agents, and treatment of the underlying disease remain the cornerstones of AIFRS management. Prognosis is overall defined by the underlying disease and in some selected cases, medical treatment alone could be a reasonable option.

The long-term need for calcium supplementation after incidental parathyroidectomy.

Objectives: Hypocalcaemia and hypoparathyroidism are the most frequent complications of total thyroidectomy that may result in the need for lifelong calcium supplementation. This study aims to investigate the impact of incidental parathyroidectomy on short- and long-term hypocalcaemia. Methods: This retrospective study was conducted on patients who underwent total thyroidectomy with or without central neck dissection between March 2016 to May 2018. All procedures were performed by three surgeons with similar experience levels. Histopathology reports were reviewed, the number of resected parathyroid glands and the underlying pathology reports were recorded, and the patients were divided into two groups; groups A and B comprised those with and without parathyroid glands respectively. The incidence rates of short- and long-term hypocalcaemia were recorded. The former was obtained through blood tests during hospitalisation and the latter via phone calls to patients 3, 6, and 12 months postoperatively. Results: A total of 116 patients participated, 18 (15.5%) in group A and 98 (84.5%) in group B. No statistical significance was detected between the two groups regarding early postoperative (p = 0.109) or long-term hypocalcaemia (p = 0.48). These results were similar in patients with cancer (n = 40) (p = 0.855, p = 0.08). Conclusion: Incidental parathyroidectomy of one parathyroid gland was not correlated with a higher incidence of hypocalcaemia, even in cases with thyroid cancer.

Variation in volumes and characteristics of ENT emergency visits during COVID-19 pandemic. Where are the patients?

The aim of this study is to analyze patterns and volumes of patients visiting ENT emergency departments during periods of community lockdowns given the newness of this situation and to assess the variation in numbers of visits and examine whether their reduction equally affects all categories of visit causation or whether it is limited to diseases not considered as emergencies. A retrospective descriptive study was conducted on prospectively collected registry data on patients of all age groups visited the emergency ENT department during the period of nationwide lockdown. Patients were grouped in 9 categories, according to the cause of visit the ENT emergency department. A total of 201 patients visited the ENT emergency department. The retrospective analysis of the data during the same period in the years 2013-2019 showed a reduction of 73% in total visits (mean total visits number between 2013 and 2019 was 745.29±20.0143). A statistically significant reduction in visitation was found in all categories studied with the exception of foreign body ingestion - aspiration. Patients with General ENT symptoms, mild epistaxis cases, otology cases, vertigo cases, uncomplicated infectious cases showed a statistically significant reduction in numbers and were treated empirically. Swallowing foreign body cases did not show statistically significant reduction. Limitation of movement and the lockdown itself, led to reduction of trauma cases. Surprisingly, there was also a reduction in oncology cases and an increase in numbers of these cases can be expected in the near future.

A Different Era for Malignant Otitis Externa: The Non-Diabetic and Non-Immunocompromised Patients.

BACKGROUND: Τo investigate the differences in regard to the clinical, laboratory, and imaging findings as well as the treatment course between diabetic and non-diabetic, non-immunocompromised patients with malignant otitis externa. METHODS: A total of 36 hospitalized patients diagnosed with malignant otitis externa between January 2011 and December 2020 were divided into 2 groups according to their medical history, blood glucose, and glycated hemoglobin levels. RESULTS: Thirty-two patients were diabetic (group A) and 4 were non-diabetic, non-immunocompromised (group B). Otalgia was present in all patients (100%), followed by otorrhoea (67%) and edema (64%). Polyps were present in 18 patients (50%). Pseudomonas aeruginosa was isolated in 16 out of 25 positive cultures (64%). Four patients of group A and none of group B underwent surgery. Five patients of group A and none of group B had at least 1 cranial nerve involvement. The mean age was 77.22 ± 8.17 for group A and 47.25 ± 3.59 for group B (P < .001). No statistical significance was observed in regards to major symptoms, inflammatory markers (white blood cell, C-reactive protein, and erythrocyte sedimentation rate), positive imaging, and microbiological findings between the 2 groups. The average days of hospitalization were 42.41 ± 31.06 for group A and 10.25 ± 2.63 for group B (P < .049). Four diabetic patients died. CONCLUSION: Non-diabetic, non-immunocompromised adult patients with malignant otitis externa had a better response to antibiotic therapy and a shorter length of hospitalization. A high clinical suspicion for malignant otitis externa should always raise in cases of otitis externa that fail to respond in a topic and/or oral antibiotic treatment for more than a week.

Clinical and Microbiological Factors Associated With Abscess Formation in Adult Acute Epiglottitis.

OBJECTIVES: To evaluate clinical and microbiological findings that are correlated with abscess formation in adult acute epiglottitis (AE). METHODS: We reviewed 140 cases of adult AE. Demographic, clinical, imaging, and microbiological findings are analyzed for all patients with AE in comparison to those with epiglottic abscess (EA). RESULTS: A total of 113 patients presented with AE and 27 presented or progressed to EA (19.3%). Age, sex, seasonality, smoking, body mass index (BMI), and comorbidities were statistically insignificant between the 2 groups. Muffled voice (P < .013), respiratory distress (P < .001), and pre-existence of epiglottic cyst (P < .001) are symptoms and signs connected with abscess formation. A total of 120 patients were treated conservatively. Surgical treatment was performed on 20 patients with EA. About 72 out of 80 cultures revealed monomicrobial infection. Mixed flora was isolated in 8 patients with EA. Streptococcus was isolated in 51 out of 80 positive cultures (64%). Haemophilus Influenza (Hib) was not isolated in any sample. EA and mixed flora relates to a higher rate of airway intervention (P < .001). CONCLUSION: A high level of suspicion for abscess formation is required if clinical examination reveals dyspnea, muffled voice, or an epiglottic cyst in adult with AE. The existence of EA doubles the duration of hospitalization. EA is typically found on the lingual surface of the epiglottis. Supraglottic or deep neck space expansion should be treated surgically. EA is associated with a mixed flora and a higher rate of airway obstruction. Streptococcus is discovered to be the most common pathogen.

Variation in volumes and characteristics of ENT emergency visits during COVID-19 pandemic. Where are the patients?

The aim of this study is to analyze patterns and volumes of patients visiting ENT emergency departments during periods of community lockdowns given the newness of this situation and to assess the variation in numbers of visits and examine whether their reduction equally affects all categories of visit causation or whether it is limited to diseases not considered as emergencies. A retrospective descriptive study was conducted on prospectively collected registry data on patients of all age groups visited the emergency ENT department during the period of nationwide lockdown. Patients were grouped in 9 categories, according to the cause of visit the ENT emergency department. A total of 201 patients visited the ENT emergency department. The retrospective analysis of the data during the same period in the years 2013-2019 showed a reduction of 73% in total visits (mean total visits number between 2013 and 2019 was 745.29±20.0143). A statistically significant reduction in visitation was found in all categories studied with the exception of foreign body ingestion - aspiration. Patients with General ENT symptoms, mild epistaxis cases, otology cases, vertigo cases, uncomplicated infectious cases showed a statistically significant reduction in numbers and were treated empirically. Swallowing foreign body cases did not show statistically significant reduction. Limitation of movement and the lockdown itself, led to reduction of trauma cases. Surprisingly, there was also a reduction in oncology cases and an increase in numbers of these cases can be expected in the near future.

Early detecting cervical necrotizing fasciitis from deep neck infections: a study of 550 patients.

PURPOSE: The aim of this retrospective review study is to evaluate Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score as an indicative parameter in early detecting cervical necrotizing fasciitis (CNF) from deep neck infections (DNI). METHODS: We reviewed 12 cases of CNF and 538 cases of non-necrotizing deep neck infection hospitalized in our hospital over the last decade. Cervical necrotizing fasciitis was histologically confirmed. RESULTS: Using an LRINEC score of 6 as a cutoff sensitivity was calculated at 100% (95% CI 99.9-100) and specificity 72.5% (95% CI 72.4-72.6). Negative predicted value (NPV) was 100% and positive predicted value (PPV) was 7.5%. C-reactive protein (CRP), white blood count (WBC), and glucose (Glu) levels have a higher correlation. Haemoglobin (Hb), sodium (Na), and creatinine (Cr) do not seem to have a big impact in our study. CONCLUSION: LRINEC score proves to be a useful "rule-out" tool that works on the safe side with high sensitivity and poor specificity. WBC, CRP, and Glu seem to be the most significant variables of the LRINEC score. Hb, Na, and Cr make the score safer. Decision for surgery must be based on medical history, clinical symptoms and signs, imaging findings, and laboratory tests and not according to the LRINEC score itself.

Paraglottic and Pre-epiglottic Space Abscess in Adults: Report of Two Cases.

INTRODUCTION: Acute epiglottitis or supraglottitis is a rapidly progressing upper respiratory tract infection that can often threaten the airway patency. Epiglottic abscess that expands to the paraglottic (PGS) or preepiglottic (PES) space and acute airway obstruction constitute rare complications, exclusively presented in adults. CASE REPORT: We report two cases. In the first case flexible fiberoptic Rhino-Pharyngo-Laryngoscopy showed epiglottitis that was obstructing the airway and abscesses on the lingual surface of the epiglottis. Abscesses were opened using laser CO2. In the following days flexible fiberoptic endoscopy revealed persisting protrusion of the left hemilarynx. A CT scan was performed showing an abscess in the paraglottic space. Under direct laryngoscopy the abscess was drained. In the second case endoscopic examination revealed epiglottitis that did not cause airway obstruction. The patient was admitted for follow-up and treated with intravenous antibiotics. On the 5th day showed an exacerbation of her symptoms. A CT scan was performed that showed the existence of an abscess in the preepiglottic space. She was taken to surgery and the abscesses were drained through a cervical- U shaped- incission. CONCLUSION: Existance of an abscess means, by default, an adequate surgical treatment to ensure the airway, and immediate drainage under direct laryngoscopy or through an external approach. Diagnosis is based exclusively on medical history and clinical examination. CT scan is necessary to reveal "secret" abscesses and "silent" extension of the infection inside pre-epiglottic and paraglottic space even if epiglottitis is mild. Postoperative management includes proper care of the surgical wound and antibiotics.

Cervical necrotizing fasciitis affects only immunocompromized patients? Diagnostic challenges, treatment outcomes and clinical management of eleven immunocompetent adult patients with a still fatal disease.

PURPOSE: Cervical Necrotizing fasciitis (CNF) has been reported as an unusual rapidly progressive, life-threatening, inflammatory disease that is known to affect predominately immunocompromised patients. Few case reports have been sporadically published involving immunocompetent adults. This is the first study presents a series of immunocompetent CNF patients. MATERIALS AND METHODS: A retrospective chart review study was performed on immunocompetent adults with CNF from January 2003 to December 2019. The diagnosis was histologically confirmed. We analyzed epidemiological, clinical, laboratory and imaging findings and discussed them along with the treatment plan. RESULTS: A total of eleven adult patients were included in the study. The average age was 43 years, ranging from 17 to 62 years. The average hospitalization time was 28 days, ranging from 10 to 129 days. All patients presented with cervical erythema and odynophagia. The causative factors are known in nine patients. Tissue cultures demonstrated mixed flora in six patients. Seven underwent a temporary tracheostomy. Wide neck exploration incisions were performed in all patients as the diagnosis was established after imaging findings. In nine cases the anterior-upper mediastinum was affected but only in two patients extension of the surgical planes to the mediastinum was performed. Four patients developed polyserositis. Nine patients survived and two developed multiorgan failure and died. CONCLUSION: There seem to be no differences between immunocompetent and immunocompromised CNF patients, in regards to clinical, imaging and laboratory findings as well as their treatment plan.

Pott Puffy Tumor in Adults: The Τiming of Surgical Ιntervention.

Pott's puffy tumor (PPT) represents a rare complication of frontal sinusitis, and it is considered as a subperiosteal abscess of the frontal bone based on osteomyelitis. We report two adult PPT patients and discuss the treatment plan as well as the correct timing of surgical intervention. Clinical examination revealed sinusitis with puss, and imaging findings showed bony erosion of the dorsal wall of the frontal sinus in both patients. In case 1, a "wait and see" approach was followed with remission of the patient's symptoms, and a Draf IIb type was performed 21 days after discharge. In case 2, worsening of symptoms led to surgical drainage through a Lynch incision followed by 20 days of intravenous antibiotic treatment. Then a Draf type IIa was performed. Both patients received antibiotic therapy over the course of six weeks and had full recovery. We highlight the importance of the correct timing of surgical intervention as it is depended on the clinical and radiological findings. The timing to performing radical drainage surgery including external or endoscopic frontal sinus surgery is not determined in the literature. Worsening of common symptoms and neurological signs in adult PPT patients means by default an immediate surgical intervention. Reduction of symptoms and antibiotic treatment response means that surgery should be delayed and performed in a surgical field free of inflammation.