Benign Paragangliomas: Clinical Presentation and Treatment Outcomes in 236 Patients

Abstract

Paragangliomas are rare tumors that arise from extraadrenal chromaffin cells. We examined the clinical characteristics, location, treatment, and outcome of 236 patients (141 females, 60%) with 297 benign paragangliomas evaluated at the Mayo Clinic during 1978-1998. The mean age (+/-SD) at diagnosis was 47 +/- 16 yr. Of the 297 paragangliomas, 205 were in the head and neck region, and 92 were below the neck. Paragangliomas were discovered and diagnosed incidentally on imaging studies in 9% of patients. Biochemical screening was performed in 128 patients; 40 patients (17% of the total and 31% of those screened) had hyperfunctional tumors. Of the 40 patients with tumoral catecholamine excess, 38 had documented hypertension. In patients identified with catecholamine-secreting paragangliomas, the sensitivities achieved by measurements in the 24-h urine collection were 74% for total metanephrines, 84% for norepinephrine, 18% for dopamine, and 14% for epinephrine. Multiple imaging modalities were used for tumor localization. The false negative rates were 0% for magnetic resonance imaging, 5.8% for computed tomography, 3.4% for angiography, 10.7% for ultrasonography, and 39% for radioactive iodine-labeled metaiodobenzylguanidine scintigraphy. Of 192 patients (81.4%) with follow-up data (mean, 43.9 months; range, 0.5-240), operative cure was achieved in 133 (69%). Of the 59 patients without cure, 23 had persistent disease, 5 had recurrent disease, 16 had multiple persistent synchronous tumors, and 15 subsequently developed metachronous tumors. In conclusion, most paragangliomas are nonhypersecretory and located in the head and neck region. Magnetic resonance imaging was associated with the lowest false negative rate, and metaiodobenzylguanidine was the least sensitive imaging study. A significant proportion of patients (31%) has persistent or recurrent disease, and long-term follow-up is important.

Cardiac Paraganglioma—A Rare Subset of a Rare Tumor

“…Their neu- person years, with a markedly higher incidence after autopsy, as a not insignificant number remains asymptomatic [3] [4]. The mean age at diagnosis of benign paragangliomas is around 47 years [5], varying depending on tumor localization [6] and genetic background (sporadic vs. hereditary) [7]. The latter is also influencing the gender-dependent incidence.…”

15 YEARS OF PARAGANGLIOMA: Genetics and mechanism of pheochromocytoma–paraganglioma syndromes characterized by germline SDHB and SDHD mutations

“…Thus, succinate and ROS are considered as the signaling molecules that ultimately trigger tumor formation upon SDH mutations. Since discovery of the first mutations in familial PPGLs in -2001(Baysal et al 2000, Niemann & Muller 2000, Astuti et al 2001b), alternative models for tumor development have been advanced using different observations and experimental models that studied the consequences of SDH genetic loss. These models can be broadly classified as constitutive hypoxic drive, inhibition of developmental neuronal culling and histone/genome hypermethylation (Fig.…”

“…Thus they are functionally and histologically similar to pheochromocytomas (in older literature they were often described as extra-adrenal pheochromocytomas). Occasionally HNPGL can also secrete catecholamines (Erickson et al 2001).…”

Nationwide study of patients with head and neck paragangliomas carrying SDHB germline mutations

“…These extra‐adrenal PGLs and phaeochromocytomas usually present with signs and symptoms of catecholamine excess3. Generally HNPGLs are parasympathetic in origin, and symptoms depend on the localization, tumour size, compression of surrounding structures and associated cranial nerve deficits.…”