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Introduction <p>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.</p> Objectives <p>To evaluate the clinicopathological features of paragangliomas diagnosed over a 5-year period at a single tertiary care centre.</p> Methods <p>Twenty histologically confirmed cases of paraganglioma resected between 2018 and 2023 were retrospectively reviewed. Hematoxylin and eosin (H&amp;E) and immunohistochemistry (IHC) slides were re-evaluated, and relevant clinical and radiological data were extracted from hospital records.</p> Results <p>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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年2月13日

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  • 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

摘要

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.