Fig. 5.1
Serial axial and coronal gadolinium enhanced T1-weighted MRI sequences of a right-sided JP demonstrating stable tumor size over the course of 10 years of observation
The primary outcomes of interest were disease progression and treatment. Tumor volume was analyzed using three perpendicular axes using the ellipsoid volume calculation [24]. Growth was defined as a minimum 20% volume increase on consecutive imaging [24, 26].
Overall, the median age at diagnosis was 70 years (38–80 years) and 12 of 15 patients were women (Table 5.1). Hearing loss (12; 75%) and pulsatile tinnitus (11; 69%) were the most common symptoms, while vagal (6; 38%), accessory (3; 19%), and hypoglossal (2; 12%) paralyses were less common at diagnosis (Tables 5.2 and 5.3). The median duration between symptom onset and diagnosis was 31 months (range 0–144 months).
Table 5.1
Baseline features, interventions, and follow-up on 16 jugular paraganglioma tumors, 15 patients, initially managed with conservative observation
Baseline features | Intervention(s) | Clinical follow-up | |||||
|---|---|---|---|---|---|---|---|
Age | Sex | Multiple tumorsa | Resection | Radiation | Vocal cord procedure | Duration | Status and age |
69.6 years | F, 12 (80%) | Y, 3 (20%) | Y, 1 (6%) | Y, 1 (6%) | Y, 5 (31%) | 7.2 years | AWD, 13 (87%), 69.7 |
(Range, 38–80) | M, 3 (20%) | N, 12 (80%) | N, 15 (94%) | N, 15 (94%) | N, 11 (69%) | (Range, 2.0–13.2) | DUC, 2 (13%), 80.1 |
Table 5.2
Baseline and progression of symptoms during course of observation in 16 jugular paraganglioma tumors, 15 patients, initially managed with conservative observation
Status | Hearing loss | Pulsatile tinnitus | Bloody otorrhea | Vertigo | Dysphonia | Dysphagia |
|---|---|---|---|---|---|---|
Never present | 4 (25%) | 5 (31%) | 14 (88%) | 12 (75%) | 9 (56%) | 10 (63%) |
Stable | 6 (38%) | 11 (69%) | 0 (0%) | 2 (13%) | 4 (25%) | 4 (25%) |
Progressed | 6 (38%) | 0 (0%) | 0 (0%) | 1 (6%) | 2 (13%) | 1 (6%) |
New deficit | 0 (0%) | 0 (0%) | 2 (13%) | 1 (6%) | 1 (6%) | 1 (6%) |
Table 5.3
Baseline and progression of cranial neuropathy during course of observation
Status | Carlson et al. 2015 | Prasad et al. 2014 | |||||
|---|---|---|---|---|---|---|---|
CN 7 | CN 10 | CN 11 | CN 12 | CNs 10, 11, 12 combined | CN 7 | CNs 10, 11, 12 combined | |
Never present | 15 (94%) | 8 (50%) | 11 (69%) | 11 (69%) | 8 (50%) | 21 (91%) | 13 (57%) |
Stable | 0 (0%) | 4 (25%) | 2 (13%) | 1 (6%) | 2 (12.5%) | 2 (9%) | 2 (9%) |
Progressed | 0 (0%) | 2 (13%)a,b | 1 (6%) | 1 (6%) | 2 (12.5%)a | 0% | 1 (5%) |
New deficit | 1 (6%) | 2 (13%) | 2 (13%) | 3 (19%) | 4 (25%) | 0% | 7 (30%) |
Six (38%) JP had growth through the medial wall of the jugular bulb with intracranial extension at the time of diagnosis (Table 5.4). The median tumor volume was 2.7 cm3 (range 0.9–19.9 cm3) and the median linear size was 2.0 cm (1.4–3.9 cm). There was no statistically significant correlation between age and tumor size at diagnosis (p = 0.53). Over a median imaging follow-up of 58 months (range 24–144 months), seven (58%) tumors were stable, while five (42%) enlarged with a median growth rate of 0.8 mm/year (range 0.6–1.6 mm/year) or 0.44 cm3/year (0.14–0.87 cm3/year). There was no age difference between the cohort of patients with growing and stable tumors (median 67 vs. 69, p = 0.27). However, patients with growing JP had a longer duration of follow-up (median 87 vs. 44 months; p = 0.07).
Table 5.4
Literature review on the natural history of jugular paragangliomas
Study | Number of cases | Median age, year | Stage at diagnosis (Fisch type) | Volume at diagnosis, cm3 | Max linear growth rate, mm/year (range) | Volumetric growth rate, cm3/year (range) | Stable or regression at follow-up | Progression at follow-up | Mean follow-up, years (range) |
|---|---|---|---|---|---|---|---|---|---|
Carlson et al. 2015 | 16 | 69.6 | C: 10 (62%) D: 6 (38%) | 2.7 | 0.8 (0.6–1.6) | 0.4 (0.1–0.9) | 7 (58%)e | 5 (42%)e | 4.8 (2.0–12.0)a |
Prasad et al. 2014b | 23 | 69 | C: 15 (65%) D: 8 (35%) | NA | NAc | NA | 15 (65%) | 8 (35%) | 5.1 |
Jansen et al. 2000d | 11 | NA | NA | 0.8 | 0.79 | NAe | 5 (45%) | 6 (55%) | 3.8 |
At a median clinical follow-up of 86 months (24–158 months), 6 (38%) patients experienced progressive hearing loss, 2 (12.5%) developed bloody otorrhea, pulsatile tinnitus remained stable in all patients, 8 (50%) maintained normal vagal function, and 11 (69%) maintained normal accessory and hypoglossal function. New or progressive lower cranial nerve paralysis developed in less than a third of cases, and only one (6%) experienced partial facial paresis. Four (25%) patients underwent type 1 thyroplasty with arytenoid adduction, and one (6%) received injection laryngoplasty. No patients required feeding tube, tracheostomy, or ventriculoperitoneal shunt placement. At last follow-up, none of the 15 patients experienced death from disease.
Gruppo Otologico, Piacenza Italy
In 2014, Prasad et al. reviewed 47 patients with JP (Fisch type C and D) who were managed conservatively and analyzed for tumor response and facial and lower cranial nerve status on follow-up [23]. Of t hese, 32 (68.1%) were older than 65 years. Tumor volume was measured by its diameter in two perpendicular planes; however, these values were not reported. Tumor growth was determined by the increase in the maximum linear dimension on follow-up and stratified into two groups: slow-growing (<3 mm/year) and fast-growing (>3 mm/year) tumors.
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