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.

Fiberoptic Endoscopic Evaluation of Swallowing Findings in Children With Rett Syndrome.

Feeding abnormalities, swallowing dysfunction, and gastrointestinal issues cause poor weight gain, oral motor dysfunction, and air swallowing in children with Rett syndrome (RTT). Pneumonia is the leading cause of death. Our study describes fiberoptic endoscopic swallowing findings in 11 female RTT children. Each patient was evaluated using the 8-point Penetration/Aspiration Scale (PAS). The average age was 7 years. All patients had tongue dyskinesis and prolonged oral stage. Eight girls exhibited liquid entering the airway without coughing, whereas 6 did well with pureed meal. Three girls had pneumonia. Age was not correlated with pneumonia episodes (P = .18). Pureed material was related with pneumonia (P = .006), whereas liquids were not. Pureed PAS was positively correlated with Liquid PAS (P = .008) and age (P = .004). All aspiration/penetration incidents occurred before the pharyngeal phase. No patient under 7 years experienced pneumonia episodes. Silent aspiration can occur early in infancy, although pneumonia episodes can occur later.

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.