Twee patiënten met een retroperitoneaal paraganglioom

- A 35-year-old woman and a 36-year-old man presented with abdominal complaints which, in both cases, appeared to be due to an unusual retroperitoneal tumour, namely a paraganglioma. Three years after radical excision the women had no complaints, while the male patient had developed metastatic disease; he was still alive 6 years after excision. Paragangliomas are sporadic tumours. Although well defined, these tumours are not well known, mainly due to the numerous different names used to describe them in the literature. These neuro-endocrine tumours arise from hyperplastic paraganglionic cells and occur in or near the ganglia of the autonomic nervous system. Paragangliomas may produce catecholamines and, on the basis of this, are classed as either functional or non-functional. In the absence of metastases, there are no definitive clinical, histopathological or molecular-biological parameters to predict whether a tumour is benign or malignant. Macroscopic radical surgical excision is therefore the treatment of choice. Five- and ten-year survival rates of a radically excised paraganglioma are 75 and 45, respectively. In the case of metastatic disease, half the patients will die within 3 years. If a paraganglioma is found, genetic screening for familial neoplastic syndromes is advised.