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.

The Youngest Case of Metachronous Bilateral Acinic Cell Carcinoma of the Parotid Gland: A Case Report and Literature Review.

Introduction: Acinic cell carcinoma (ACC) is a low-grade malignant salivary neoplasm that represents 17% of all salivary gland malignancies. It has a tendency to affect young individuals, especially females. ACC mainly originates in the parotid gland and has a potential for recurrence and metastases. Rarely, ACC can affect both parotid glands in a single individual. A bilateral ACC of the parotid gland could either present as a synchronous or a metachronous tumor. Case Report. Our patient is a 19-year-old female known case of ACC of the right parotid gland. The tumor was resected in December 2017. After 3 years, she presented with a left parotid pain and swelling, which raised the suspicion of a contralateral metachronous tumor of the left parotid gland. In September 30, 2020 we proceeded with ultrasound-guided fine needle aspiration of the left intraparotid lesion, and the results turned out to be consistent with ACC. Here, we report a case of a 19-year-old female presenting with metachronous bilateral ACC of the parotid gland with an interval of 3 years, which is the 6th of its kind in the literature and the youngest amongst them. Conclusion: Despite the rareness of metachronous occurrence of bilateral ACC of the parotid gland, it is still encountered in the medical practice. Here, we are highlighting the importance of follow-up with a periodic clinical and radiological examinations, bearing in mind the contralateral nonaffected parotid gland.

Unilateral Exserohilum Allergic Fungal Sinusitis in a Pediatric Host: Case Report.

INTRODUCTION: Allergic fungal sinusitis (AFS) is a result of an inflammatory reaction to fungi in the nasal and paranasal sinuses. Although the causative agents of AFS vary, Exserohilum species are among the rare ones, as only a few cases have ever been reported a few times in the literature. The objective of this report is to highlight this unusual fungal type causing unilateral AFS in Saudi Arabia. PRESENTATION OF THE CASE: We present a case of AFS who initially presented at the age of 15 years, and was operated on in 2015 by another health care provider. He presented again in 2019 complaining of intermittent loss of smell and greenish nasal discharge, mainly from the right side for 3 months, which was associated with right-sided nasal obstruction. Based on the history and physical examination, a diagnosis of refractory chronic rhinosinusitis was made. The patient underwent functional endoscopic sinus surgery (FESS) of the paranasal sinuses and polypectomy. Histopathology of the samples taken during the surgery showed Exserohilum specie. The diagnosis of AFS was confirmed using Bent and Kuhn's criteria. DISCUSSION: Allergic fungal sinusitis is believed to be an allergic reaction caused by a broad spectrum of species, the majority being Aspergillus in India and Saudi Arabia. Exserohilum species are one of the rare organisms causing AFS in our region. CONCLUSION: Most cases of AFS in our region have been reported to be caused by Aspergillus. Here we report a case of unilateral Exserohilum AFS in an immunocompetent pediatric patient.

Ganglioneuroma of the External Auditory Canal and Middle Ear.

OBJECTIVE: We report an extremely rare case of ganglioneuroma involving the external auditory canal and middle ear. CASE REPORT: Ganglioneuromas are rare benign mature tumors thought to originate from sympathetic ganglions, with the highest incidence in the retroperitoneum, adrenal medulla, and posterior mediastinum. We present a case of ganglioneuroma of the external auditory canal and middle ear. At the age of 12 months, the patient was diagnosed with neuroblastoma stage IV with metastasis to the squamous temporal bone, bone marrow, and skull base. He received a high-risk protocol regimen resulting in complete remission. The patient later presented with recurrent right ear discharge at the age of six years and was diagnosed with ganglioneuroma of external auditory canal and middle ear after appropriate investigations. We report in this article the clinical presentation, investigations, surgical intervention, and follow-up. CONCLUSION: After the literature review and to our knowledge, this is the first reported case of its kind. Ganglioneuroma maturing from neuroblastoma is one of the theories describing pathophysiology of the disease. Ganglioneuroma should be considered in the differential diagnosis of patients presenting with recurrent ear discharge and decreased hearing in treated cases of neuroblastoma with metastases to temporal bone.

Etiology of optic atrophy: a prospective observational study from Saudi Arabia.

BACKGROUND: Optic atrophy (OA) represents permanent retinal ganglion cell loss warranting study to establish etiology. OBJECTIVES: To describe neurogenic causes of OA. DESIGN: Prospective, observational. SETTING: Tertiary care center, Riyadh, Saudi Arabia. PATIENTS AND METHODS: We included consecutive patients of all ages with OA caused by lesions affecting the visual pathways who were referred over a 9-month period (November 2013 to July 2014). Diagnosis was based on visual acuity, ophthalmoscopic features and ancillary tests. Patient demographics, results of a clinical examination, test data and etiology were recorded. For each cause of OA, both gender and age group were analyzed as potential risk factors using simple univariate logistic regression. OA associated with glaucoma and retinal diseases was excluded. MAIN OUTCOME MEASURE: Description of causes of OA. RESULTS: Two hundred and four patients and 353 eyes met inclusion criteria. The median age was 27 years (range 3 months-77 years; interquartile range, 27 years) among 111(54.4%) females and 93(45.6%) males, with no statistically significant difference in age of presentation between the genders. The majority of lesions were bilateral (n=151, 74%). Tumors were the most common cause, accounting for 127 (62.2%) cases. These occurred mostly in adults (72.4%) compared to the pediatric group (OR=3.3, 95% CI: 1.79-6.03; P < .001). Hereditary neoplasia (OR=5.55; 95% CI: 1.67-18.42; P=.005) and metabolic diseases (OR=17.57; 95% CI: 2.15-143.62; P=.007) were more common causes in the pediatric group. There were no significant associations between gender or visual acuity and etiology of OA. In developed nations, OA is frequently the result of ischemia and neuritis. We found many other causes, especially orbital and intracranial tumors. CONCLUSIONS: The frequency of tumors as the cause of OA may represent a higher incidence of aggressive tumors coupled with poor recognition/acknowledgement of symptoms and limited access, resulting in late presentations. LIMITATIONS: These findings may reflect bias from selective referrals to a tertiary center and may not represent all of Saudi Arabia.

Hepatocellular carcinoma first presenting as a tumor of the oral cavity.

Hepatocellular carcinoma (HCC) is the sixth most common neoplasm worldwide; HCC metastasis is common affecting 50% of cases. However, metastasis to the oral cavity is extremely infrequent. We present a case of hepatocellular cancer first presenting as a mass lesion at the upper alveolus and review metastatic hepatocellular carcinoma to the oral cavity in 73-year-old male patient.