Endocrine Abstracts

Phaeochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumours arising from chromaffine cells either from adrenal medulla (phaeochromocytoma) or extra-adrenal paraganglia (paraganglioma). Phaeochromocytomas and sympathetic paragangliomas (arising from thoracic, abdominal, or pelvic sympathetic ganglia) may secrete catecholamines which are associated with the most clinical symptoms and signs whereas parasympathetic paragangliomas (arising from parasympathetic ganglia of the head and neck region) are secretory inactive. PPGL may develop metastases, in particular in those patients harbouring mutation in the SDHB gene. Around two thirds of phaeochromocytomas are diagnosed incidentally during morphological examinations from other reasons and only one third after targeted biochemical screening in symptomatic subjects. Symptomatology of PPGL varies from mild symptoms on one side such as headache, palpitations, and sweating (reported in around 50 - 60% of patients) to life-threatening complications such as circulatory shock, takotsubo cardiomyopathy, arrhythmias on the other side. Unique combination of signs in PPGL is the association of hypertension with orthostatic hypotension, in some cases seriously limiting quality of life. Once is PPGL diagnosed (nowadays using metanephrines either in plasma or in urine), patients are scheduled for tumour removal and prepared with a-adrenergic receptor blockers. Experienced surgeons and anaesthesiologist are required for successful tumour removal due to hemodynamic instability during and after operation. Due to the risk of recurrence or metastases (may be present also at initial presentation), patients with PPGL should be followed-up for at least 10 after the surgery.

Conclusion

Awareness of PPGL among different medical specialties such as anaesthesiology, cardiology or internal medicine may contribute to early diagnosis of PPGL which is the best prevention of potentially lethal cardiovascular complications or development of metastases.