A Rare Case of Bilateral Malignant Paragangliomas

Paragangliomas are neuroendocrine neoplasms that arise from the extra-adrenal paraganglia of the autonomic nervous system. Approximately 3% of all paragangliomas occur in the head and neck region. Most reported cases are benign and unilateral. We present a rare case of bilateral malignant paragangliomas.

A 28-year-old woman presented with a 10-year history of bilateral neck swelling. Physical examination showed bilateral neck masses with obvious pulsation. Enhanced computed tomography revealed 2 irregular solid nodules, located in the left and right carotid artery bifurcation, respectively. Carotid artery angiography showed compression of the internal and external carotid arteries by the tumors on both sides. On diagnosis of the bilateral carotid body tumors, preoperative embolization was performed. The left-side lesion and lymph nodes were resected and a diagnosis of malignant paraganglioma with lymph node metastasis was made. She was treated with 50-Gy radiotherapy, and computed tomography performed 2 years later showed that the right-side lesion was unchanged. She was symptom-free as of the last follow-up.

The rarity of bilateral malignant paragangliomas makes their management clinically challenging. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative radiation is beneficial in slowing the progression of residual disease. Genetic studies have shown that familial paragangliomas are associated with germline mutation of SDHD on 11q23.