PubMed1993
This is a revision of our experience between 1975 and 1992 over a total of 35 patients who underwent surgery in 40 occasions for pheochromocytoma. If we dismiss one case of pheochromocytoma with early metastatic malignant presentation and which was not removed, a total of 6 patients with abdominal paraganglioma (17%) have undergone surgery in 10 occasions. Three of them (50%) were multiple and recurrent, an one (16%) become malignant after multiple recurrences. Average age was 34.6 years with balanced sex distribution. No patient had neurofibromatosis, Von Hippel Lindau disease, MEN syndromes or Carney's triad and only one case, which corresponded to the malignant pheochromocytoma, showed direct familial background. All patients were hypertensive. Four cases (66%) presented with catecholamine crisis, one case was an incidentaloma that at the 24-hour monitoring presented hypertensive crisis during the sleep, and the last one was diagnosed while investigating a case of sustained HTA in a young female. All patients had high urinary catecholamine excretion. To establish location of the tumour MIBG scanning (90% sensitivity) was used in first place followed by guided CT (80% sensitivity) since both techniques have a good correlation in order to design the surgical approach. Angiography was reserved for cases where the other two techniques were inconclusive or when it was necessary to obtain a better profile of the surgical anatomy. A total of 15 paraganglioma were removed, the most frequent location being renal parahilar (26%) followed by preaortic in Zuckerkandl's organ (20%) and vesical (20%). The most relevant intraoperative complications were HTA crisis related to anaesthetics manoeuvres and tumoral handling (90%), and hypotension following tumour exercises (10%). During the postoperative period, three patients required blood transfusion and one of them had to be re-intervened for bleeding caused by unnoticed damage to the right renal vein. Currently, three patients (50%) are disease free, one with residual mild HTA controlled with diuretics. Two patients (33%) are awaiting for re-intervention due to recurrence (one with multiple extra-abdominal recurrence) after one and two prior interventions respectively. The last one (16%) is the malignant pheochromocytoma, operated four times for recurrence which currently shows lung metastasis with adequate drug control of clinical manifestations, after polychemotherapy failure and 27-months follow-up since metastasis has been detected. Revision of existing literature and discussion of issues related to signs and symptoms, diagnosis, surgical preparation and approach, as well as prognostic implications related to paraganglioma as compared with adrenal-located pheochromocytoma.