Pheochromocytoma and Paraganglioma Presentation Differs Between Sexes

Women with pheochromocytomas and paragangliomas (PPGLs) are more likely than men to present with headaches and adrenergic symptoms at diagnosis, according to study findings published in the Journal of the Endocrine Society. 

Biological sex can influence the physiology and severity of some diseases. However, data regarding sex-related differences in PPGL presentation are scarce.

To evaluate the sex-related differences in the presentation of a variety of PPGLs, researchers conducted a retrospective, single center study at the Centre Hospitalier de l’Université de Montréal between January 1995 and January 2022. Participants had a diagnosis of either a secretory or nonfunctional PPGL.

In their primary analysis, the researchers described adrenergic symptoms including hypertension, headaches, diaphoresis, palpitations, tremor, and presence of diabetes according to biological sex and tumor location. The researchers also stratified catecholamine profiles into 3 subgroups of (1) epinephrine/metanephrine, (2) norepinephrine/normetanephrine, and (3) metanephrine/normetanephrine.

The study included a total of 385 patients (mean age, 49.8 years), of whom 60.8% were women, 30.6% had head and neck paragangliomas, 15.1% had thoracoabdominal paragangliomas, and 54.3% had pheochromocytomas. 

Headaches were more common among women compared with men (47.5% vs 32.4%; P =.007), whereas diabetes prevalence was more common among men compared with women (21.1% vs 12.5%; P =.039). The researchers identified no statistically significant between-sex differences regarding other adrenergic symptoms.

Pheochromocytomas were diagnosed more often among women vs men (58.4% vs 41.6%), but men were more likely than women to have diabetes at diagnosis (28.2% vs 11.2%; P =.0038). 

Compared with men, women also had a higher prevalence of head and neck paragangliomas (69.5% vs 30.5%) and thoracoabdominal paragangliomas (51.7% vs 48.3%).

Among the patients with head and neck paragangliomas, women were more likely than men to present with headaches (31.0% vs 11.4%; P =.0499) and palpitations (16.9% vs 0.0%; P =.0079). Similar trends were observed among patients with TAPGLs. 

Regarding secretory tumors, more women than men presented with headaches (58.9% vs 42.7%; P =.0282) and more men than women had diabetes at diagnosis (29.3% vs 12.5%; P =.0035). These trends were consistent across all catecholamine profiles and secretory statuses.

Among patients with non-secretory tumors, women vs men reported higher rates of headaches (46.9% vs 3.6%; P =.0002), diaphoresis (16.3% vs 0.0%; P =.0454), and palpitations (22.4% vs 0.0%; P =.0057). 

In genetic counseling and analysis, the researchers identified a higher prevalence of SDHD mutations among women compared with men (3.7% vs 0%). 

Study limitations include its retrospective nature, the fact that most symptoms were qualitative, and limited generalizability due to missing data.  

“These findings are most likely multifactorial and can be explained by differences in adrenergic receptor sensitivity, self-reported symptoms, and possibly other vasoactive peptides and sex-hormone status,” the researchers concluded. 

This article originally appeared on Endocrinology Advisor