Paraganglioma of the mediastinum: challenges in diagnosis and surgical management

Abstract

Mediastinal paraganglioms are rare, highly vascularized tumors arising from chromaffin tissue located in the para-aortic ganglia. Tumors tend to invade bordering structures and may also form metastasis. Up to 50% of patients are asymptomatic and diagnosis is incidental. Presenting symptoms are related to catecholamine hypersecretion or to a mass effect. Complete surgical resection remains the standard of care due to malignant potential of the tumor and poor response to chemotherapy or radiation. Strategic location of the tumor in proximity to great vessels, trachea, and recurrent laryngeal nerve poses challenge for the surgeon. We report a case of a 59-year old asymptomatic female who was incidentally diagnosed with a middle mediastinal mass on a positron-emission tomography (PET-CT) scan performed as part of breast cancer surveillance. Complete resection of the tumor was achieved using cardiopulmonary bypass. The patient recovered uneventfully and in a ten-month follow up there is no evidence of recurrence.

Paraganglioma of Urinary Bladder Presenting as An Early Preeclampsia with Successful Perinatal Outcome After Surgery: A Case Report and Review of Literature

“…On review of the literature it was found that many centres believe in terminating the pregnancy electively before proceeding with partial cystectomy in such circumstances. A good outcome with vaginal delivery has been described in only few selected cases [8].…”

“…Surgery is the definitive treatment of choice for these tumours, with appropriate alpha-adrenergic and if needed, subsequent beta-adrenergic blockade to prevent a hypertensive crisis. In both pregnant and non-pregnant individuals with catecholamine secreting tumours Phenoxybenzamine is the alpha blocker of choice [8]. Radical cystectomy, partial cystectomy and transurethral resection are the reported treatment options for localized or locally advanced disease.…”

Paraganglioma não funcional de mediastino médio: diagnóstico e manejo cirúrgico

“…(4) Mediastinal paragangliomas are derived from the para-aortic and paravertebral ganglion chain. (3) Multicentric tumors are observed in 23% of cases, and there seems to be no specific distribution in the remaining cases. (5) Similar to pheochromocytomas, paragangliomas can secrete catecholamines, although most are nonfunctional.…”

“…(5) Similar to pheochromocytomas, paragangliomas can secrete catecholamines, although most are nonfunctional. (3,6) Functional mediastinal paragangliomas are discovered as a result of the effects of catecholamine hypersecretion (1)(2)(3) or of compression, which leads to hoarseness, dysphagia, dyspnea, and chest pain. (7) The diagnosis is incidental in more than 50% of all cases.…”

“…Distant metastases are typically to the lung and bones. (2) The treatment of choice is complete surgical resection, (2,3,8) which is difficult because of tumor hypervascularization and anatomical juxtaposition.…”