Natural History of Jugular Paragangliomas: A Review of 16 Patients Managed with Conservative Observation

Abstract

Objectives:

Characterize radiologic growth and clinical disease progression in a cohort of observed, previously untreated, jugular paraganglioma tumors (JPT).

Methods:

Retrospective review evaluating all patients with primary JPTs that were observed with serial clinical examination and imaging between 1993 and 2013. Primary outcome measures included radiographic growth and progression of cranial neuropathy.

Results:

A total of 16 patients (75% female, median age 65.2 years) met inclusion criteria. One JPT was stage I, 7 stage II, 7 stage III, and 1 stage IV. Primary indications for observation included advanced age, minimal symptoms, contralateral paraganglioma(s), and patient preference. The most common presenting symptoms were hearing loss and pulsatile tinnitus; 6 patients were found to have lower cranial neuropathy at time of diagnosis (2 CN 9, 6 CN 10, 2 CN 11, 1 CN 12). At a mean clinical follow-up of 67 months, 6 patients endured progressive cranial nerve deficits and 4 tumors demonstrated unequivocal growth; the average rate of tumor growth was 1.2 mm/year. Six patients underwent vocal cord medialization procedures for dysphonia. Notably, no patients required feeding tube placement or tracheostomy and there were no deaths attributable to tumor progression.

Conclusions:

In the absence of brainstem compression or concern of malignancy, initial observation of JPTs should be considered. A significant number of tumors do not grow after time of diagnosis, and symptoms frequently remain stable for many years. Even with disease progression, most JPTs exhibit indolent growth with slowly progressive cranial neuropathy affording satisfactory physiologic compensation in most patients.