# Anterosuperior mediastinal paraganglioma in a 42-Year-old woman: a diagnostic and therapeutic challenge—a case report

**Authors:** Zheng Wang, Wenkang Zong, Shuo Liang, Fang Zhou, Xike Lu, Daqiang Sun

PMC · DOI: 10.1186/s13019-024-03283-9 · Journal of Cardiothoracic Surgery · 2025-04-07

## TL;DR

A rare case of a mediastinal paraganglioma in a 42-year-old woman was successfully treated with surgery and careful planning.

## Contribution

This case report presents a rare extra-adrenal paraganglioma and emphasizes the importance of imaging and surgical expertise.

## Key findings

- The tumor was successfully removed despite significant intraoperative blood loss.
- Histopathology and immunohistochemistry confirmed the diagnosis of paraganglioma.
- Comprehensive imaging and interdisciplinary collaboration are crucial for managing such cases.

## Abstract

Paragangliomas are rare neuroendocrine tumors predominantly located within the adrenal gland. Extra-adrenal paragangliomas, particularly those in the anterosuperior mediastinum, are exceedingly rare and pose significant diagnostic and therapeutic challenges due to their complex anatomical location.

A 42-year-old woman was found to have an anterosuperior mediastinal mass during a routine health screening. Enhanced chest computed tomography (CT) revealed an ovoid, low-density mass intricately associated with major vascular structures including the superior vena cava, brachiocephalic trunk, left common carotid artery, aortic arch, right anonymous vein, and right subclavian artery. Despite significant intraoperative blood loss of 2000 ml, the mass was successfully excised with meticulous surgical technique and effective hemostasis. Histopathological examination showed a classic Zellballen pattern with chief cells and sustentacular cells embedded in a vascular-rich stroma. Immunohistochemistry confirmed the tumor’s chromaffin nature, with chief cells testing positive for CD56, Synaptophysin, and Chromogranin A, and sustentacular cells positive for S100 protein, consistent with a diagnosis of paraganglioma. The patient’s postoperative recovery was uneventful, and she was discharged one week after surgery.

This case highlights the essential role of comprehensive preoperative imaging and the necessity for interdisciplinary surgical expertise in managing complex mediastinal paragangliomas. Advanced surgical techniques and careful intraoperative management are paramount to achieving successful outcomes. Appropriate imaging modalities and auxiliary laboratory tests are crucial for early detection of recurrences in these rare tumors.

Key findings

What is known and what is new?

What is the implication, and what should change now?

• Report here about key findings of the study.

• Report here about what is known.

• Report here about what does this manuscript adds.

• Report here about implications and actions needed.

## Linked entities

- **Proteins:** NCAM1 (neural cell adhesion molecule 1)
- **Diseases:** paraganglioma (MONDO:0000448)

## Full-text entities

- **Genes:** CHGA (chromogranin A) [NCBI Gene 1113] {aka CGA, PHE5, PHES}, SYP (synaptophysin) [NCBI Gene 6855] {aka MRX96, MRXSYP, XLID96}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, NCAM1 (neural cell adhesion molecule 1) [NCBI Gene 4684] {aka CD56, MSK39, NCAM}
- **Diseases:** neuroendocrine tumors (MESH:D018358), Paragangliomas (MESH:D010235), mediastinal paraganglioma (MESH:D008480), tumor (MESH:D009369), Extra-adrenal paragangliomas (MESH:D010236), blood (MESH:D006402)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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Source: https://tomesphere.com/paper/PMC11974066