Abstract
Objectives: Often confused with other primary and secondary laryngeal neoplasms, paragangliomas of the larynx are exceptionally rare lesions. The authors describe the case of a tracheal paraganglioma that recurred following misdiagnosis and treatment as medullary thyroid carcinoma and review the existing literature.
Methods: A retrospective review of a case of invasive tracheal paraganglioma recurring 1 year after near-total thyroidectomy for “medullary thyroid carcinoma.”
Results: A 50-year-old female presented with a prior histopathological diagnosis of medullary thyroid carcinoma following near-total thyroidectomy and radioactive iodine ablation therapy for a palpable left thyroid nodule. She presented to the emergency department 1 year postoperatively with hemoptysis and in acute respiratory distress resulting in emergent tracheotomy. Direct laryngoscopy revealed a pulsatile mass filling the tracheal lumen and biopsy was deferred. Review of the original specimen with immunohistochemical analysis concluded a diagnosis of tracheal paraganglioma. Subsequently, a mass invading through the tracheal cartilage was excised after preoperative embolization.
Conclusion: Tracheal paragangliomas occur infrequently and are exceedingly rare in the proximal trachea. They are atypical lesions often misinterpreted as medullary thyroid carcinoma as well as other neoplasms. Imperative to accurate delineation is proper microscopic and immunohistochemical analysis. In this case, appropriate diagnosis avoided potential life-threatening airway hemorrhage that has been described after biopsy. Clinical photographs, radiographs, and histopathology are presented and discussed.