Metachronous and Sporadic Bilateral Carotid Body Paraganglioma

The Authors' goal is to highlight the importance of early diagnosis and management of metachronous carotid body paraganglioma (CBP), analysing and discussing the main features of this rare tumor.

This study review the literature on a very particular topic, reporting the case of a patient with the rare evidence of a metachronous CBP in the left side of the neck, which onset and was surgically treated 26 years after the first contralateral presentation.

After excision, the diagnosis of CBP was confirmed by histological and immunohistochemical examination of the surgical specimen. Transient dysphonia and dysphagia were observed after surgery, both recovered after 1 years. No cardiac anomalies were detected.

Paragangliomas are rare neuroendocrine neoplasms with an incidence of 2-8 X 1,000,000. Paragangliomas of the head and neck (HNP) originate mainly from the carotid body (60%) and in a few cases from the jugular bulb, the medial promontory of the middle ear (30%), the vagal nerve and the nodose ganglion (5-10 %). Bilateral CBP represents an extremely rare entity, accounting for approximately 5% of patients with CBP. Diagnostic imaging of CBP includes ultrasound, Computed Tomography (CT), Magnetic Resonance Images (MRI) and Angiography. The treatment of choice for CBP is its complete surgical removal. Early detection of symptoms referable to a new CBP is very important, because of the possibility, even after many years, of contralateral presentation of the same tumor. This, if operated at an early stage, decrease the risk of neurovascular complications.