Posterior mediastinal paragangliomas: A report of three patients with peculiar tumours

Abstract

Paragangliomas of the mediastinum are rare neoplasms, representing less than 0.3% of mediastinal tumours and less than 2% of all paragangliomas. To date, no definitive morphologic criteria exist that correlate with the clinical outcome of these tumours. This report describes three patients with peculiar primary paragangliomas of the posterior mediastinum: gangliocytic, dumbbell and functional paraganglioma. The clinicopathological characteristics of these patients are presented and the literature reviewed.

Metastatic Malignant Paraganglioma: A Case Report and Review of Literature

“…Mediastinal paragangliomas are estimated to make up 0.3% of all mediastinal tumors and less than 2% of all paragangliomas [ 8 ]. To date, the only reliable criterion for malignancy is the presence of metastatic spread of chromaffin cells in tissues that normally do not contain such cells [ 9 ].…”

“…Histological studies are required for definitive diagnosis. Previous studies described a Zellballen appearance in which chief cell nests are surrounded by sustentacular supporting cells [ 9 ]. Tumor markers include neuron-specific enolase (92.1% sensitivity), chromogranin (84.2%), and met-enkephalin (73%) [ 10 ].…”

“…Tumor markers include neuron-specific enolase (92.1% sensitivity), chromogranin (84.2%), and met-enkephalin (73%) [ 10 ]. Histological studies have also denoted a negative keratin marker and the appearance of S-100 and glial fibrillary acid protein (GFAP) in the surrounding sustentacular cells [ 9 , 10 ].…”

Dumbbell-shaped neurofibroma over the external ear

“…Dumbbell-shaped tumors have been commonly described in the spine, thorax, intracranial region, pelvis, and hand. [ 5 6 7 8 9 ] However, dumbbell-shaped tumors in the periphery are rarely described in the literature.…”

“…Histologically, dumbbell-shaped tumors can be neurofibromas, neurilemmomas, lipomas, or paragangliomas. [ 5 6 7 8 9 ] Dumbbell-shaped neurilemmomas have been described in the hand and are characterized by mild, localized pain, and paresthesia resulting from pressure on the nerve of origin and histologically they are well-encapsulated in contrast to neurofibromas that are usually painless and do not have a well-defined capsule.…”