Introduction:
Paragangliomas are neuroendocrine tumors arising from chromaffin cells located in sympathetic paraganglia. Mediastinal paragangliomas are extremely rare and can be classified as functional or non-functional according to their ability for secreting catecholamines. Patients can be asymptomatic and the diagnosis is usually incidental. Complete surgical resection remains the standard of care for paragangliomas.
Presentation of case:
We present a 44-year-old woman with a functional mediastinal paraganglioma incidentally found during the perioperative imaging workup for a diagnosed breast carcinoma. Chest radiograph and computed tomography (CT) showed a well-defined lesion in the posterior mediastinum suspicious for an esophageal malignancy. Endoscopic and CT-guided biopsies were performed confirming the diagnosis of a neuroendocrine tumor. Laboratory studies showed elevated catecholamines and chromogranin A levels, consistent with a paraganglioma. Appropriate pre-operative management was done and successful surgical resection without catecholamine related complications was achieved.
Discussion:
The workup and treatment of incidentally discovered adrenal and extra-adrenal lesions are controversial. Because of the absence of symptoms and the wider differential diagnosis of extra-adrenal lesions, an attempt for biopsying and surgically remove these lesions prior to biochemical testing is not an uncommon scenario, although this could be potentially harmful. Surgeons should have an index of suspicion for catecholamine-secreting tumors and hormonal levels should be assessed prior to biopsy or surgical resection.
Conclusion:
Surgeons should consider paragangliomas as a differential diagnosis for extra-adrenal lesions. Biochemical testing with catecholamines and chromogranin A levels should be performed prior to biopsy or surgical removal in order to avoid catastrophic complications.