Metastatic Malignant Glomus Jugulare Tumor: A Rare Case Report with Clinical Manifestations and Treatment Approach.

BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.

Revisiting the cytomorphological features of poorly differentiated thyroid carcinoma: a comparative analysis with indeterminate thyroid fine-needle aspiration samples.

INTRODUCTION: Poorly differentiated thyroid carcinoma (PDTC) is an uncommon high-grade carcinoma of follicular cell origin that is usually overlooked on preoperative fine-needle aspiration (FNA) due to its rarity and cytomorphological overlap with follicular-patterned neoplasms. Definitive diagnosis of PDTC usually requires histologic examination of the resected thyroid tumor. Herein, we describe the cytological and architectural findings of histologically confirmed PDTC cases. MATERIALS AND METHODS: A search for all thyroid FNAs with a corresponding surgical diagnosis of PDTC was performed. Surgical diagnoses were reviewed and confirmed using the Turin criteria. In addition, the control group consisted of indeterminate thyroid nodules (FLUS [follicular lesion of undetermined significance] and FN [follicular neoplasm]) that were either benign or well-differentiated thyroid tumors on resection. The PDTC and control groups were both subjected to cytological assessment using specific cytological and architectural parameters, which included cellularity, growth pattern, mitoses, necrosis, chromatin change, discohesion, and anisonucleosis. RESULTS: A total of 36 thyroid FNAs were included in the study. This consisted of 12 histologically confirmed PDTC FNAs and 24 indeterminate thyroid FNAs (FLUS and FN, 12 each). The most frequent findings among PDTC groups were hypercellularity (75%), trabecular/insular growth pattern (58%), branching capillaries (67%), and cellular discohesion (92%). Necrosis (25%), ≥3 mitoses (50%), and anisonucleaosis (42%) were less frequently observed. A peculiar finding was the presence of adenoid cystic carcinoma-like globules in 50% of PDTC cases. Certain findings such as colloid, necrosis, mitoses, and cellular discohesion were helpful in differentiating the two groups. CONCLUSIONS: Thyroid fine-needle aspiration remains an essential diagnostic/triage tool for most thyroid nodules/tumors. PDTC can be diagnosed or at least suspected preoperatively based on the demonstration of certain architectural and cytological alterations. Although mitoses and necroses are not always readily identified, an elevated Ki-67 labeling expression could provide additional clues to the diagnosis in some cases.

Sinonasal Renal Cell-Like Adenocarcinoma: A Report of a Rare Entity With Emphasis on Its Association With Von Hippel Lindau Syndrome.

Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare malignant sinonasal tumor with relatively indolent clinical course. Clinically, it could be asymptomatic or show non-specific symptoms such as epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires clinical, radiological, histopathological, and ancillary tests to characterize the lesion accurately. We herein report a case of a 41-year-old female with a nasal mass noted two years ago, which presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and inhibin, while they were negative for CK20, P63, CK 5/6, CD10, renal cell carcinoma (RCC), TTF1, PAX8, CEA, and GATA3. The proliferation index (KI67) was less than 5%. The diagnosis was consistent with SNRCLA. The patient has no recurrence and no symptoms after one year. Thus, our study reports a rare case of SNRCLA with a discussion of the histological features and its association with von Hippel Lindau syndrome.

SMARCB1-Deficient Sinonasal Carcinoma: Case Report and Review of the Literature.

BACKGROUND SMARCB1-deficient sinonasal carcinoma is a rare neoplasm with inactivation of the SWI/SNF complex, with an aggressive clinical course as most of the lesions present as advanced in pT3/T4 stages with frequent recurrence, and many patients succumb to the disease. Reported initially in 2014, the lesion has male predominance, with an age range of 19 to 89 years and predilection for the ethmoid sinus and nasal cavity. Histopathological findings show a proliferation of small- to medium-sized monomorphic basaloid cells with indistinctive cytoplasmic borders and round variably prominent nuclei with scattered cells that show rhabdoid morphology. Cytoplasmic vacuoles are common. It has similar morphological findings to a wide array of neoplasms in the sinonasal area. CASE REPORT We report a case of SMARCB1-deficient sinonasal carcinoma in a 30-year-old man referred to our hospital with a preliminary diagnosis of sinonasal adenocarcinoma, intestinal type. Computed tomography showed a huge destructive soft tissue mass in the left maxillary sinus, extended to involve the left nasal cavity with extension to the skull base and perineural spread along the foramen rotundum. Histological examination revealed a malignant basaloid neoplasm embedded in a myxoid stroma that showed loss of SMARCB1 stain. The patient was treated with induction chemotherapy using etoposide and cisplatin for disease control. CONCLUSIONS SMARCB1-deficient sinonasal carcinoma is a rare neoplasm with an aggressive clinical course and high-grade behavior despite having uniform cytological features. This poses complex diagnoses, especially in small biopsies. Incorporating morphological findings with ancillary tests is required to identify this high-grade malignancy.

Cytospin performance when using Paris system for reporting urinary cytology.

Objectives: The Paris System (TPS) for Reporting Urine Cytology has significantly improved the approach to evaluating urine cytology. TPS criteria were defined mainly according to ThinPrep and SurePath preparations, as they are widely utilized. The objective of this study is to validate urine cytology interpretation according to the TPS classification using cytospin technique in relation to the gold slandered histology. Material and Methods: This retrospective study examined and analyzed 316 urine specimens from King Abdulaziz University Hospital between 2015 and 2020. Cytospin technique is performed for all cases. Slides were recategorized using TPS criteria, then compared with the original histology diagnosis. Results: According to the TPS, 108 cases were classified as 101 AUC (32%), 95 NEG (30%), 59 HGUC (18.7%), 31 SHGUC (9.8%), and 30 (9.5%) others. The computed sensitivity of cytospin in urine cytology was 94.7%, with 73.9% specificity, a positive predictive value of 85.6%, a negative predictive value of 89.5%, and overall accuracy of 86.8%. Conclusion: Urine cytology testing is considered to be a non-invasive and sensitive method to screen for urothelial carcinoma. TPS defined standards are reliable on cytospin prepared slides for reporting urine cytology.

Myometrial myxoidosis in a patient with systemic lupus erythematosus.

Myometrium myxoidosis is new terminology to describe a non-neo-plastic process of extracellular mucinous accumulation in the myometrium wall of the uterus. We report a rare association of myometrial myxoidosis with lupus erythematosus in a 35-year old woman with a history of leiomyoma. At presentation, this case was diagnosed as a pelvic abscess and treated with specific antibiotherapy, and then discharged after clinical improvement. One week later, after recurrence of the symptoms, the patient underwent hysterectomy with bilateral salpingo-oophorectomy and appendectomy with anterior abdominal soft tissue part resection. Pathological analysis revealed diffuse hypo-cellular myxoid areas intersecting the smooth muscle layer of the uterus and cervix in addition to a focal area in the interstitium of smooth muscles of the appendix and soft tissue of the anterior abdominal wall. This case emphasizes the importance of distinguishing between myxoid neoplastic and non-neoplastic lesions with myxoid changes. SIMILAR CASES PUBLISHED: To our knowledge, there are only two similar cases reported.

Diagnosis of Hodgkin's Lymphoma Using Endobronchial Ultrasound-Guided Transbronchial Needle.

Endobronchial ultrasound-guided transbronchial biopsy has emerged as an excellent tool in diagnosing lung cancer. However, its use to diagnose lymphoma has been questioned, since the gold standard for diagnosing lymphomas is an excisional biopsy of involved lymph nodes. However, the procedure is sometimes risky or difficult. Recent studies have been showing great results using endobronchial ultrasound-guided transbronchial needle aspiration when accompanied by immunohistochemistry and cytology. Here, we present a case of Hodgkin's lymphoma patient that was accurately diagnosed using endobronchial ultrasound-guided transbronchial needle aspiration.

Clinicopathological Characteristics of Thyroid Cancer in a Saudi Academic Hospital.

Background The global incidence of thyroid cancer (TC) has increased significantly over the past decades. In Saudi Arabia, it is the third most common cancer among adults. This study aims to review the clinical and histopathological characteristics of TC in Saudi Arabia and analyze the size trend over the years. Methods We conducted a retrospective chart review of all differentiated and poorly differentiated TC patients following up at a tertiary care center in the Western region of Saudi Arabia. All patients 11 years and older, diagnosed between 2004 - 2018, and with sufficient histopathological data were included. Follicular and poorly differentiated TC were categorized and tumor stage was allocated. We performed descriptive and size trend analysis. Results We had a total of 285 patients who qualified for inclusion. The mean age at diagnosis was 40.6 years, and 81.05% of patients were females. Papillary TC comprised 88.07% of these neoplasms, and most patients (89.12%) were at Stage I. Only papillary TC showed a gender difference in the age of the diagnosis. In men, the mean age at diagnosis of papillary TC was 46.98 ± 15.4 years, while in female patients, it was 39.02 ± 12.8 years (p-value = 0.0001). We did not find a trend toward smaller tumours in the more recent years in comparison to the early years (r = -0.083, p-value = 0.168). Conclusions TC is diagnosed at a younger age and larger sizes in Saudi Arabia in comparison to other countries. A gender difference was only noted with papillary TC in regard to the age of diagnosis. There was no trend toward smaller sizes of TC over the years.

Quality assessment of systematic reviews and meta-analyses published in Saudi journals from 1997 to 2017.

OBJECTIVES: To assess the quality of the meta-analyses (MAs) and systematic reviews (SRs) in Saudi journals indexed in PubMed using 2 scales: A MeaSurement tool to assess systematic reviews (AMSTAR) and the overview quality assessment questionnaire (OQAQ). METHODS: This study focused on SRs/MAs published in 8 Saudi journals. We investigated, screened and extracted the data, which included recording the main topic of each SRs/MAs and the date of publication. Furthermore, we assessed the quality of each included SRs/MAs using the AMSTAR and the OQAQ. The reviews concluded in January 2018. Results: The search uncovered 201 unique articles; of these, the researchers screened 110 full texts and included 103 in this review. Most of the included studies were published in Saudi Medical Journal (50 articles, 48.5%), followed by Saudi Journal of Gastroenterology (21 articles, 20.4%), and Annals of Saudi Medicine (16 articles, 15.5%) . The main topics in these published articles were gastroenterology (20 articles, 19.5%), followed by oncology (14 articles, 13.7%), and pharmacology (9 articles, 8.7%). The AMSTAR and the OQAQ scales showed that most SRs/MAs were of medium quality. CONCLUSION: Quality of SRs and MAs published in Saudi journals was distributed in all categories (low, medium, and high) and it can be improved using critical evaluation by authors, journal editors, and readers. PROSPERO REG. NO. CRD: 42018102210.

P53 Gene Mutation Identified by Next Generation Sequencing in Poorly Differentiated Neuroendocrine Carcinoma of the Nasal Cavity.

Neuroendocrine carcinomas (NECs) are epithelial neoplasms showing morphologic, immunophenotypic or ultrastructural evidence of neuroendocrine differentiation. The 2017 WHO Classification of Head and Neck Tumours classifies NECs into well, moderately and poorly differentiated NECs according to light microscopic features, mitotic rate and presence of tumour necrosis. In this study, we performed next generation sequencing (NGS) using a targeted 161 cancer gene panel on a poorly differentiated NEC of the nasal cavity. The tumour was composed of large cells arranged in poorly formed glands and solid nests. The mitotic count rate was 30/10 HPFs and p53 protein was strongly expressed in all tumour cells. NGS identified a missense mutation, c.764T > G (p.Ile255Ser) in the TP53 gene with an allele frequency of 85%. This mutation results in an isoleucine to serine substitution and a non-functional protein. No other mutations were identified. These results suggest that TP53 mutations may drive oncogenesis in poorly differentiated NECs of the head and neck.