Endocrine Abstracts

Introduction: Paraganglioma is a tumour derived from extra-adrenal chromaffin cells of the sympathetic paravertebral ganglia of the thorax, abdomen and pelvis. About 36 % to 60 % of the paragangliomas are functional secreting norepinephrine and normetanephrine, which cause hypertension. Non-functional paragangliomas can produce Chromogranin A. Paragangliomas also arise from the parasympathetic ganglia located along the glossopharyngeal and vagal nerves in the neck and at the base of the skull. Only about 5 % of these tumours secrete catecholamines.

Material and methods: The patient is a female, 51 years old. A few months before consulting the Endocrinology outpatient clinic, during an ultrasound examination of the abdomen, a tumorous lesion size 70 x 69 x 51 mm was detected - localized in front of the vena cava inferior. This finding was confirmed with Computed Tomography. She was not evaluated by an endocrinologist before the surgery. With a post-operative diagnosis of “St. post extirpationem TU v. cava inferior” and a histopathology report of paraganglioma, the patient consults the Endocrinology outpatient clinic: anamnestic without any symptoms both pre- and post-operatively and denial of past illnesses. From the outpatient laboratory analysis, metanephrine and vanillylmandelic acid in 24-hour urine were in the reference range and had an elevated value of Chromogranin A. A computed tomography scan of the abdomen and chest and a PET/CT scan with IV application of 18 F-FDG were performed, which did not reveal any local recurrence or distant metastases. During the follow-up, due to elevated chromogranin A values, a full body scan and SPECT/CT scan were performed after an i.v application of a somatostatin analogue. No pathological accumulations of a somatostatin analogue were registered.

Conclusion: Paragangliomas are rare neuroendocrine tumours. Malignancy is defined as the presence of metastasis at presentation or during follow-up, a metastasis is defined as the presence of chromaffin tissue in non-chromaffin organs. Paragangliomas commonly metastasize (30 %-50 %) to the liver, lungs, lymph nodes and bones. Metastases are usually functional, 80 % secrete norepinephrine and normetanephrine. It is essential that paragangliomas are acknowledged, because biochemical and imaging pre-operative evaluation is necessary, which is significant for perioperative and postoperative follow-up.