Incidental posterior mediastinal paraganglioma: The safe approach to management, case report

Abstract

HighlightsMediastinal paragangliomas are extremely rare and their diagnosis and management can be challenging.These tumors are classified as functional or non-functional according to their ability to produce and release catecholamines.Appropriate laboratory studies should be done prior to biopsy or surgical resection to avoid complications.Complete surgical resection continues to be the standard of care for patients diagnosed with mediastinal paraganglioma.Surgeons must consider catecholamine-secreting tumors as a differential diagnosis of mediastinal lesions.

Surgical management of mediastinal paraganglioma: All hands on deck!

“…Differential diagnosis of tumors of the middle (also known as the visceral compartment) of the mediastinum include cystic lesions, most commonly, foregut duplication cysts or pericardial cyst, neoplasms (leiomyoma or carcinoma) of the esophagus, lymphadenopathy of various etiologies, and rarely, as mentioned in the case report by Shah et al, 1 solid complex highly vascular intrapericardial paragangliomas. The radiographic investigations for mediastinal tumors include computed tomography (CT) scan and/or magnetic resonance imaging (MRI) of the chest with intravenous contrast, positron‐emitting tomography/computed tomography (PET/CT) scan, occasional endoscopic ultrasound via upper gastrointestinal endoscopy or bronchoscopy, specialized nuclear imaging such as 123 I‐metaiodobenzylguanidine (I‐IMBG) scintigraphy for paragangliomas and octreotide scan or 68 Ga‐DOTATATE (radioconjugate used as a somatostatin receptor‐imaging agent to detect receptor‐expressing neuroendocrine tumors) PET/CT scan for neuroendocrine tumors 2,3 . The surgical management of these mediastinal tumors, except mediastinal paragangliomas, is frequently straightforward.…”

“…Neuroendocrine tumors arising from chromaffin cells of the sympathetic system are termed pheochromocytoma if originating in the adrenal medulla (90% of cases) and called paraganglioma if located in the extra‐adrenal sympathetic ganglia (10%) 3 . The majority of paragangliomas are found in the abdomen (80%‐95%), and less commonly found elsewhere such as in the neck (4%) and in the thorax (about 2%).…”

“…The majority of paragangliomas are found in the abdomen (80%‐95%), and less commonly found elsewhere such as in the neck (4%) and in the thorax (about 2%). Mediastinal paragangliomas account for less than 0.3% of all mediastinal tumors 3‐5 . Similar to pheochromocytomas, some paragangliomas can synthesize and secrete catecholamines to the circulation, however, most of these tumors are nonfunctional.…”

“…Mediastinal paragangliomas are very uncommon. Most published literature about this tumor is in the form of single case reports 3‐6,8‐10 or case series of fewer than 15 cases each accumulated over a very long period of time at tertiary academic centers 11,12 . Brown et al 11 from Mayo Clinic in Rochester, MN, provided an excellent review of this topic.…”

Surgical resection of a middle mediastinal paraganglioma that caused diabetes

“…Ninety percent of adrenergic tumors originate in the adrenal medulla and are known as pheochromocytomas; the remaining 10% are extra-adrenal and are called paragangliomas [ 1 ]. Paragangliomas originating from the mediastinum account for 1–2% of all paragangliomas and less than 0.3% of all mediastinal tumors [ 2 – 4 ]. Mediastinum paragangliomas commonly originate from the posterior mediastinum, while those originating from the middle mediastinum are quite rare [ 1 ].…”