Endocrine Abstracts

Introduction

Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare neuroendocrine tumors of the autonomic nervous system, originating from neural crest. Despite the same embryological origin, there are some differences between them.

Aim

We searched for differences in method of discovery, clinical and biochemical phenotype, of sporadic vs hereditary PHEO/PGL in a cohort of Flemish patients.

Material and methods

A retrospective analysis of electronic medical records of sixty-seven consecutively registered patients diagnosed or treated with hereditary or sporadic PHEO/PGL in a tertiary care center from Belgium between 2002–2020, was performed. Patients were divided according to anatomic location (PHEO vs. PGL) and according to presenting with sporadic or hereditary PHEO/PGL.

Results

We identified 38 women and 29 men, aged 50 ± 19 years (range 13–85). At diagnosis, 42(63%) had PHEO, 25(37%) had PGL. Patients with PHEO presented more frequently with sporadic than hereditary disease [34 (81%) vs. 8 (19%)] than patients with PGL [9(36%) vs.16 (64%)] respectively. Mean age at diagnosis was significantly higher in PHEO patients (55 ± 17 years in PHEO vs.40 ± 18 years in PGL, p = 0.001). The diagnosis was done either due to PHEO/PGL related symptoms [PHEO:21(50%);PGL:13(52%)], discovered incidentally (27) or due to genetic screening (6) [PHEO: 21(50%); PGL: 12(48%)]. In our patient cohort, following CV events were known at diagnosis: myocardial infarction(MI)[PHEO: 8(19%); PGL:1(4%)], Takotsubo cardiomyopathy [PHEO:2; PGL:2], arrhythmias [PHEO:3; PGL:0] and stroke [PHEO:1; PGL:0]. All PHEO and about two-thirds of PGL were hormonally functional. Metanephrine levels were higher in PHEOs than in PGLs (P < 0.02). Tumor diameter was 44 (17–200) mm in PHEO vs. 30 (11–110) mm in PGL. Metanephrine levels were correlated with tumor diameter in PHEOs vs. PGLs. (P < 0.02).An interesting aspect is related by the differences in tumor localization of sporadic vs hereditary PPGLs. 61.7 % of sporadic PHEOs and 77.7% of sporadic PGLs and had right lateralization while 62.5% of hereditary PHEOs and 50% of hereditary PGLs had left lateralization. PGLs were mostly HNPGLs (12), followed by abdominal(16), pelvic(16) and thoracic(1) PGLs.

Conclusion

PGLs have a higher hereditary predisposition, therefore are diagnosed at younger age than PHEOs. Although both PHEO and PGL in about half of cases were diagnosed due to related symptoms, patients with PHEO more often presented with cardiovascular co-morbidities which is presumably related to their hormonal activity.