Head-and-neck paragangliomas are associated with sleep-related complaints, especially in the presence of carotid body tumors.

OBJECTIVES: The carotid body functions as a chemoreceptor. We hypothesized that head-and-neck paragangliomas (HNP) may disturb the function of these peripheral chemoreceptors and play a role in sleep-disordered breathing. DESIGN: This is a case-control study. SETTING: This study was conducted in a tertiary referral center. PARTICIPANTS AND MAIN OUTCOME MEASURES: We assessed fatigue, sleep, and exercise capacity in 74 HNP patients using three questionnaires (Epworth Sleepiness Scale, St. George Respiratory Questionnaire, and a standard clinical sleep assessment questionnaire). Outcomes were compared to those of age- and sex-matched controls. RESULTS AND CONCLUSIONS: Activity, disturbance of psychosocial function, and total score were worse compared to controls (15.4 ± 18.5 vs. 7.2 ± 9.9, P = 0.007; 5.3 ± 10.5 vs. 1.2 ± 2.6, P = 0.008; and 10.4 ± 12.9 vs. 5.0 ± 4.8, P = 0.006, respectively). Patients reported more daytime fatigue, concentration difficulties, and depression (51% vs. 24%, P = 0.006; 31% vs. 10%, P = 0.010; and 19% vs. 2%, P = 0.012). Waking up was reported to be less refreshing in HNP patients (53% vs. 73%, P = 0.038). Dysphonia was a predictor of symptoms, activity, disturbance of psychosocial function, and total scores. Remarkably, the presence of a carotid body tumor was an independent predictor of increased daytime sleepiness (ß = 0.287, P = 0.029). In conclusion, patients with HNP have remarkable sleep-related complaints. Especially the presence of carotid body tumors appears to be associated with increased daytime somnolence.

Plasma chromogranin A levels are increased in a small portion of patients with hereditary head and neck paragangliomas.

CONTEXT: The majority of patients with head and neck paragangliomas (HNPGL) have biochemically silent tumours. Chromogranin A (CgA) is a tumour marker for neuroendocrine tumours. OBJECTIVE: To assess the role of CgA as a tumour marker in patients with hereditary HNPGL. PATIENTS AND METHODS: We included 95 consecutive patients with hereditary HNPGL for screening of plasma CgA levels and catecholamine excess by measurement of 24-h urinary excretion of (nor)metanephrine, (nor)adrenaline, VMA, dopamine and 3-methoxytyramine. In all patients with catecholamine excess, abdominal/intrathoracic paragangliomas were excluded by (123) I-MIBG scintigraphy, MRI and/or CT. RESULTS: Plasma CgA levels were increased in only 15 of 95 patients (16%). Thirty-three of the 95 patients (35%) had increased urinary excretion rates of catecholamines. Six of these 33 patients (18%) had increased plasma CgA levels. Nine of the 62 patients (15%) with a biochemically silent tumour, i.e. no increased urinary excretion of catecholamines or their metabolites, had increased CgA levels. Increased plasma CgA levels were positively correlated with urinary excretion rates of noradrenaline (r = 0·68, P = 0·005) and normetanephrine (r = 0·68, P = 0·005). There was a positive correlation between maximal HNPGL diameter and plasma CgA levels in the 57 patients with a single HNPGL (r = 0·57, P = 0·001). CONCLUSIONS: Plasma CgA levels are increased in only a small portion of patients with hereditary HNPGL and have limited additional value to the combination of radiological and routine biochemical assessment of patients with HNPGL. Increased plasma CgA levels are associated with increased noradrenergic activity and tumour size in patients with a single HNPGL.

Effects of octreotide therapy in progressive head and neck paragangliomas: case series.

BACKGROUND: Octreotide, a somatostatin analog, may be beneficial in the treatment of head and neck paragangliomas (HNPGLs). METHODS: We conducted a nonblinded, prospective intervention study. During 1 year, patients received a monthly intramuscular injection of 30 mg octreotide. Pretreatment and posttreatment tumor volumes were assessed by MRI, urinary catecholamine secretion was measured, and HNPGL-related signs and symptoms were recorded. RESULTS: In 1 of 4 included patients with HNPGL, a stabilization of tumor growth was observed after octreotide therapy. In 1 patient, octreotide therapy was discontinued before the end of the study because of potential side effects. No improvements in HNPGL-related signs and symptoms were observed. CONCLUSION: In 1 of 4 patients, HNPGL tumor growth velocity was reduced after octreotide therapy. Research assessing the effects of somatostatin analogues targeting different somatostatin receptor subtypes or combined with other therapies may offer new possibilities for the treatment of HNPGLs.

Urinary 5-HIAA excretion is not increased in patients with head and neck paragangliomas.

BACKGROUND: Case reports have documented carcinoid-like features in head and neck paragangliomas (HNPGLs), which, in addition to catecholamine storing granules, may also contain granules with serotonin. Serotonin is metabolized to 5-hydroxyindoleacetic acid (5-HIAA). 
 AIM: To assess the urinary excretion rates of 5-HIAA and catecholamines in HNPGL patients.
 METHODS: In 114 consecutive HNPGL patients, normetanephrine, metanephrine, norepinephrine, epinephrine, VMA, dopamine, 3-methoxytyramine and 5-HIAA excretion rates were measured in two 24-hour urinary samples. Increased excretion rates were defined as an increase of the average hormone excretion rate of 2 urine samples above the reference range. In all patients with catecholamine excess, intrathoracic and abdominal paragangliomas were excluded by 123I-MIBG scintigraphy, MRI and/or CT. Genetic screening for mutations in genes of the succinate dehydrogenase (SDH) family was performed. 
 RESULTS: Mean urinary 5-HIAA excretion rate was 14±9 µmol/24 hours (reference range 10-44 µmol/24 hours). Urinary 5-HIAA excretion was slightly increased in only 1 patient (48 µmol/24 hours). None of the 50 patients (44%) with increased urinary excretion rates of catecholamines and/or their metabolites had elevated 5-HIAA excretion.
 CONCLUSION: Urinary 5-HIAA excretion is within the normal reference range in almost all HNPGL patients. Therefore, this parameter has no clinical relevance in the routine clinical assessment of HNPGL patients.