Endocrine Abstracts

Phaeochromocytomas and paragangliomas account for about 0.1% of cases of persistent hypertension. Only 50% of these are being diagnosed as symptoms are often paroxysmal.

We report a retrospective data analysis on 16 random patients (age 24–71 years, mean 51) diagnosed and treated for phaeochromocytoma and paraganglioma in our centre over the past 20 years. Symptom duration was 6 weeks to 15 years with female preponderance 2:1 (69 vs 31% in males). Sympathetic symptoms were present at diagnosis in 50% (with hypertension), 30% (without hypertension) and as an incidental finding in 20%.

12 patients had phaeochromocytoma; all unilateral and predominantly right sided (58%). It was malignant in one patient with metastasis to liver and pelvic bone. Initial size on CT ranged from 0.3 to 10 cm (mean 5 cm). One familial case was identified with neurofibromatosis type 1 (8%), the remaining 92% being sporadic. In four patients with paraganglioma, three were malignant. The secretory pattern in phaeochromocytomas was predominantly adrenaline (67%) with combination of both adrenaline and noradrenaline in 58%. The inverse was true for paragangliomas with 100% elevated urinary noradrenaline. 24-h urine dopamine was elevated in malignancy in both conditions. 63% of total cases were MIBG avid with no uptake in six patients – benign phaeochromocytomas (3) and paragangliomas (1 benign and 2 malignant). Phenoxybenzamine was commenced during first consultation (80%) and discontinued following surgery in all patients with phaeochromocytomas. Laparoscopic adrenalectomy was performed in 67 and 33% had laparotomy. All but one patient from both groups under the age of 45 were referred for mutation analysis and none were identified. A low threshold of suspicion for the above conditions is required to initiate early treatment and avoid long-term complications and sudden death. The poor specificity of MIBG indicates need for other functional modalities at an earlier stage to diagnose, predict and influence prognosis in potentially malignant paragangliomas given the poor prognosis and uncertainty in available treatment modalities.