High-Grade Sinonasal Small Cell Neuroendocrine Carcinoma in a Patient With Xeroderma Pigmentosum: A Case Report.

Neuroendocrine tumors are rare and highly heterogeneous neoplasms with a wide spectrum of histological differentiation, functional, and biological features. Small cell neuroendocrine carcinomas (SNECs) of the sinonasal tract are particularly rare. Pathological features of SNECs of the nasal cavity and paranasal sinuses, which are aggressive tumors, are similar to those of anaplastic small cell carcinomas of the lung. We present a case of high-grade sinonasal SNECs in a patient with Xeroderma pigmentosum complementation group C (XPC) gene-positive xeroderma, which, to our knowledge, is the first case reported worldwide.

Efficacy of gelfoam middle ear packing in type-1 tympanoplasty: systematic review and meta-analysis.

PURPOSE: To conduct a systematic review and meta-analysis of randomized controlled trials (RCTs) that evaluated the surgical outcomes of type-1 tympanoplasty with and without gelfoam middle ear packing. METHODS: PubMed, CENTRAL, Scopus, Web of Science, and Google Scholar databases were screened from inception until October 2022. The included RCTs were evaluated for risk of bias, and the quality of each outcome was assessed according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. RESULTS: Nine RCTs with 773 participants (gelfoam = 381 and non-gelfoam = 392) were analyzed. The overall study quality varied: low risk (n = 4 RCTs), some concerns (n = 3 RCTs), and high risk (n = 2 RCTs). There were no significant differences between both arms regarding the graft uptake rate (moderate certainty), hearing improvement rate (moderate certainty), type of impedance audiometry (moderate certainty), and ear discharge (low certainty). The mean change in air-bone gap was significantly higher in the non-gelfoam arm compared with the gelfoam arm (low certainty). However, the non-gelfoam group had superior hearing improvement in only the early postoperative period (i.e., one month); however, after two, three, and six months, there were no significant differences between both arms. The rate of ear fullness was significantly higher in the gelfoam arm compared with the non-gelfoam arm (moderate certainty). CONCLUSION: Among patients undergoing type-1 tympanoplasty, the surgical outcomes did not significantly differ between both arms. The practice of middle ear packing with gelfoam needs to be standardized.

Isolated Congenital Anosmia: Case Report and Literature Review.

Isolated congenital anosmia (ICA) is a rare entity worldwide with poorly understood genetic variation. The diagnosis of ICA is made by exclusion of acquired causes of anosmia. Additionally, magnetic resonance imaging in ICA is essential for diagnosis, as it shows reduced or absent development of olfactory bulbs and shallow olfactory sulci. Here, we present the case of a 21-year-old man who presented to our clinic with complete anosmia since birth. The patient's history was negative for acquired causes of anosmia, and the physical examinations of the ears, nose, throat, head, and neck were all not remarkable. Smell testing revealed complete anosmia. The CT imaging was unremarkable; however, magnetic resonance imaging of the anterior brain and olfactory region showed bilaterally absent olfactory bulbs and olfactory tracts, with a shallow olfactory groove. The patient was then subjected to whole exome sequencing. Bioinformatics analysis was performed on the 37 genes associated with olfactory dysfunction, in which a missense variant was identified in the HS6ST1(NM_004807.3) gene was identified, which insilico tools predicted to be likely pathogenic. The results of this patient's genetic analysis add to the possible genetic culprits reported in ICA cases. Additional genetic analyses are required to validate mutations and understand the heterogeneity of disease representation.

Molecular Targeted Therapy in the Management of Recurrent and Metastatic Nasopharyngeal Carcinoma: A Comprehensive Literature Review.

Nasopharyngeal carcinoma (NPC) is the most frequent malignancy arising in the nasopharynx. NPC, to a larger degree, substantially differs from the other malignancies of the head and neck, in terms of incidence, etiology, risk factors, molecular pathogenesis, clinical behavior, management and prognosis. Fundamentally, the management of NPC is entirely guided by the disease stage. Favorably, patients with early- stage disease have encouraging survival outcomes with stand-alone radiation therapy (RT), specifically following the emergence of intensity-modulated RT (IMRT). The reported five-year local control rates are outstanding, and they range from 70% to 90%. Unfortunately, around one-third (30%) of patients presents with loco-regional or distant recurrences, despite rigorous curative treatment in the intermediate (stage II) and advanced (stage III-IVB) NPC disease. At the present time, the management of recurrent and metastatic NPC is largely discouraging and presents significant challenges to the treating physicians. Broadly speaking, there are three management schemes utilized in the management of recurrent and metastatic NPC, namely: (i) palliative systemic chemotherapy, (ii) molecular targeted therapy, and (iii) immunotherapy. The goal of this study is to holistically review the existing body of literature on the utility and safety of molecular targeted therapy in the management of patients with recurrent and metastatic NPC, with a special focus on vascular endothelial growth factor (VEGF) and epidermal growth factor receptor (EGFR) targets.

Prevalence of C-shaped canal system in mandibular first and second molars in a Saudi population assessed via cone beam computed tomography: a retrospective study.

OBJECTIVES: The purpose of this study was to determine the prevalence of the C-shaped root canal configuration, location of the longitudinal groove, sex differences, and unilateral/bilateral presence in mandibular first and second molars in a Saudi population using cone beam computed tomography (CBCT). MATERIALS AND METHODS: CBCT images for the mandibular first and second molars of 487 patients (a total of 529 first molars and 681 s molars) were evaluated. The teeth were assessed for the presence of C-shaped root canals according to Fan criteria. Subdivisions were also made according to sex, direction of the longitudinal groove, and unilateral/bilateral presence. RESULTS: Only one C-shaped mandibular first molar was observed (0.19%), whereas 62 second molars (9.1%) exhibited C-shaped anatomy. Unilateral presence of the C-shaped root canal system was more common (53.85%). Female patients had a higher prevalence than males. Longitudinal grooves were most commonly found on the root lingual surface (58.1%). CONCLUSIONS: The prevalence of the C-shaped canal configuration in a Saudi Arabian population was 0.19% in the mandibular first molar and 9.1% in the mandibular second molar. Longitudinal groove prevalence was highest on the lingual surface. Women had a significantly higher prevalence of the C-shaped canal configuration than men. Patients with unilateral presence of the C-shaped canal configuration were more common than those with bilateral presence. CLINICAL RELEVANCE: Tooth type, patient sex, and ethnicity can help clinicians predict the prevalence of the C-shaped canal system in mandibular molars.

Double cross finger flaps from the middle to the index or ring finger: A case series of 4 patients with an emphasis on donor finger morbidity.

INTRODUCTION: The use of two cross finger flaps from one digit has not been previously reported and the technique raises concerns regarding donor finger morbidity. In this paper, the authors report on a case series of double cross fingers flaps harvested from the middle finger to reconstruct large defects in the adjacent index or ring finger; with an emphasis on documenting morbidity in the donor middle finger. METHODS: A total of four cases of double cross finger flaps were retrospectively reviewed. Demographic data, surgery, and postoperative complications were documented. Donor middle finger morbidity (stiffness, painful neuromas, skin graft instability, cold intolerance and cosmetic concerns) were also documented. RESULTS: All patients were young male industrial workers. Two patients underwent reconstruction with de-epithelialized cross finger flaps and the other two patients had classic cross finger flaps. No postoperative complications were noted. Mild stiffness at the distal interphalangeal joints were noted in all patients. There were no painful neuromas and one donor site had occasional blistering at the site of the skin graft. Mild cold intolerance was seen in the two patients with electric burns. Hyperpigmentation of the skin grafts was noted in all patients. CONCLUSIONS: Our paper introduces to the literature the technique of utilizing two cross finger flaps from the middle finger to reconstruct large dorsal or volar defects of the adjacent index or ring finger. The study shows that the technique is feasible and is easily executed. The results document an acceptable donor finger morbidity.