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.

Laryngotracheoesophageal Cleft Type IV in a Preterm Neonate. A Case Report and Literature Review.

We present a case of a preterm neonate with a type IV laryngo-tracheo-oesophageal cleft, an uncommon congenital malformation, resulting from the failure of separation of the trachea and the oesophagus during fetal development, often associated with other deformities as well. Data in the literature shows that the long-term morbidity from the entity has declined over the last decades, even though prognosis remains unfavourable for types III and IV. This report emphasizes the complex issues neonatologists are faced with, when treating neonates with this rare disorder in the first days of life, what will raise suspicion of this rare medical entity, and that direct laryngoscopy/bronchoscopy finally depicts the exact extension of the medical condition. At the same time extensive evaluation for coexisting congenital anomalies should be performed. For all the above reasons, these neonates should be treated in specialized tertiary pediatric centers for multidisciplinary prompt management, which may improve, the outcome.

Revisiting the Concept of Non- and Minimally Invasive Interventions in Early Glottic Cancer - Part II: Single Therapy Should be Favored over the Combination of Transoral Laser Microsurgery and Radiotherapy, Regarding the Postinterventional Voice Quality

Abstract Introduction Early glottic cancer (EGC) is associated with a high cure rate. Hence, patients and physicians also focus on the impact of the proposed treatment on the speaking function of the preserved larynx. Objectives The present study assessed the impact of single-modality treatment (transoral laser microsurgery [TLM], or radiotherapy) or combination therapy for EGC on post-interventional voicing and explored factors which might explain the related perceptions. Methods A total of 108 patients filled in the voice handicap index 10 questionnaire, 1 and 2 years postinterventionally. Non-parametric tests were used for the respective statistical analyses. Results Sixty-four patients were treated with TLM, 15 with radiotherapy, and 29 with both modalities. Transoral microsurgery and radiotherapy were associated with post-interventional dysphonia, which attenuated between the first and second postinter-vention year (p = 0.000). No association between sociodemographic parameters and the attenuation of postinterventional dysphonia was identified for either treatment modality. Transoral microsurgery and radiotherapy resulted in comparable postin-terventional voicing, in the first (p = 0.940) and second (p = 0.196) postintervention years. The addition of TLM to radiotherapy resulted in worse voice quality in the second, compared with the first postintervention year (p = 0.000), demonstrating a detrimental effect on speech intelligibility in noise (p = 0.000). Conclusion Single therapy should be favored over the combination of TLM and radiotherapy for EGC in terms of retaining better postinterventional voice quality. Postinterventional dysphonia should be taken into account, during preinterventional counseling, as it may exert leverage on the quality of patients' lives. Patients and physicians should acknowledge the optimal time of voice function return, which seems to be extending up to two years posttreatment.