Endocrine Abstracts

Introduction: Catecholamine-secreting tumours derived from the adrenal medulla and the nodes of the autonomic nervous system are known as pheochromocytomas or paragangliomas, respectively. Although their clinical manifestations are similar, their differentiation is important from an epidemiological and prognostic point of view and their possibility of being associated with genetic syndromes.

Case report: We present a case of a 16 year old patient, with no pathological history of interest, who was admitted to our centre after finding elevated levels of 24-hour urine fractionated metanephrine, as well as elevated dopamine, noradrenaline and methoxytyramine. A three-year history of evolution based on repeated cases of holocranial headache, diaphoresis, and skin pallor accompanied, especially in the last months before admission, by typical angina chest pain. In the month prior to admission, she had consulted her family doctor due to the increase in symptoms, and in medical consultation she noted high blood pressure. There was a family history of hypertension in a well-controlled maternal grandmother with 2 antihypertensive drugs.

The metanephrine elevation was confirmed after a second determination made during hospitalization. Likewise, imaging tests (abdominal MRI) were performed for tumour location, which showed the presence of a right para-aortic mass compatible in clinical and biochemical context with an abdominal paranganglioma. An extension study was performed with MIBG-123. The patient was operated by laparotomy after medical treatment with phenoxybenzamine and subsequent beta blocking with atenolol for 20 days prior to surgery. The results of the pathological anatomy were compatible with poorly differentiated paraganglioma with associated lymph node metastases. The levels of ki 67 in the sample was >3%. A genetic study was also requested which was positive for the SHDB enzyme mutation.

After the intervention the patient is asymptomatic. Post-surgical determination of fractionated metanephrines without elevation was requested demonstrating a decrease in levels of fractionated methanephrines and methoxytyramine, with persistence of dopamine elevation.

Conclusion: The study and management of catecholamine-secreting tumours is complex. It is well established that surgery is the treatment of choice but there are controversies about the pre and post-operative management of these patient. With this review we want to clarify the more complex data related to the management of these tumours that not only represent a diagnostic challenge but also a therapeutic challenge.