"Clinicopathological profile of paragangliomas: A 5-Year retrospective analysis from a single tertiary Centre".

IF 3.5 3区 医学 Q2 PATHOLOGY
Geetha V, Megha Murali, Bhavna Nayal, Deepak Nayak, Vidya Monappa, Shreya Garg, P S Priya, Girish Menon, Raghavendra Nayak, Kailesh Pujary, R Balakrishnan, Suresh Pillai
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引用次数: 0

Abstract

INTRODUCTION: Paragangliomas are rare neuroendocrine tumours arising from neural crest–derived paraganglionic cells and form part of the pheochromocytoma–paraganglioma (PPGL) spectrum. The World Health Organization (WHO) 5th edition classification emphasizes autonomic origin, distinguishing sympathetic from parasympathetic paragangliomas, and recognizes that all paragangliomas possess metastatic potential. OBJECTIVES: To evaluate the clinicopathological features of paragangliomas diagnosed over a 5-year period at a single tertiary care centre. METHODS: Twenty histologically confirmed cases of paraganglioma resected between 2018 and 2023 were retrospectively reviewed. Hematoxylin and eosin (H&E) and immunohistochemistry (IHC) slides were re-evaluated, and relevant clinical and radiological data were extracted from hospital records. RESULTS: The cohort showed a female predominance (male-to-female ratio 1:1.8) with a median age of 49 years. The most common tumour site was the jugulotympanic region (30%), followed by intradural extramedullary (IDEM) locations (15%) and the retroperitoneum (15%). Two patients (10%) had metastatic disease involving the spine. Radiological findings were concordant with histology in 50% of cases. IHC was performed in 75% of cases and aided differentiation from histologic mimics. The Ki-67 proliferation index ranged from 1 to 2% in most cases, with one exception (4%). Follow-up data were available for 13 patients; one case showed local recurrence, and no syndromic associations were identified. CONCLUSION: Histological features alone are insufficient to predict tumour behaviour. Metastasis remains the only definitive criterion for malignancy, particularly when identified at sites lacking normal chromaffin tissue. Comprehensive clinicopathological assessment, incorporating radiology and immunohistochemistry, is essential for accurate diagnosis and management, and integration of molecular markers may further refine risk stratification in future studies.

副神经节瘤的临床病理特征:来自单一三级中心的5年回顾性分析。
副神经节瘤是由神经嵴源性副神经节细胞引起的罕见神经内分泌肿瘤,是嗜铬细胞瘤-副神经节瘤(PPGL)谱系的一部分。世界卫生组织(WHO)第5版分类强调自主起源,区分交感神经副神经节瘤和副交感神经副神经节瘤,并认识到所有副神经节瘤具有转移潜力。目的:评估在单一三级保健中心诊断的副神经节瘤的5年期间的临床病理特征。方法:回顾性分析2018年至2023年间20例经组织学证实的副神经节瘤切除病例。重新评估苏木精和伊红(H&E)和免疫组织化学(IHC)切片,并从医院记录中提取相关的临床和放射学数据。结果:该队列显示女性优势(男女比例为1:8 .8),中位年龄为49岁。最常见的肿瘤部位是颈鼓膜区(30%),其次是硬膜内髓外(IDEM)部位(15%)和腹膜后(15%)。2例患者(10%)有累及脊柱的转移性疾病。50%的病例放射学表现与组织学一致。在75%的病例中进行了免疫组化,并帮助从组织学模拟物中进行区分。多数病例Ki-67增殖指数为1% ~ 2%,有一例例外(4%)。13例患者有随访数据;1例局部复发,无综合征相关性。结论:仅凭组织学特征不足以预测肿瘤行为。转移仍然是恶性肿瘤的唯一明确标准,特别是在缺乏正常染色质组织的部位。综合临床病理评估,包括放射学和免疫组织化学,是准确诊断和管理的必要条件,而分子标记的整合可以在未来的研究中进一步完善风险分层。
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来源期刊
Diagnostic Pathology
Diagnostic Pathology 医学-病理学
CiteScore
4.60
自引率
0.00%
发文量
93
审稿时长
1 months
期刊介绍: Diagnostic Pathology is an open access, peer-reviewed, online journal that considers research in surgical and clinical pathology, immunology, and biology, with a special focus on cutting-edge approaches in diagnostic pathology and tissue-based therapy. The journal covers all aspects of surgical pathology, including classic diagnostic pathology, prognosis-related diagnosis (tumor stages, prognosis markers, such as MIB-percentage, hormone receptors, etc.), and therapy-related findings. The journal also focuses on the technological aspects of pathology, including molecular biology techniques, morphometry aspects (stereology, DNA analysis, syntactic structure analysis), communication aspects (telecommunication, virtual microscopy, virtual pathology institutions, etc.), and electronic education and quality assurance (for example interactive publication, on-line references with automated updating, etc.).
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