Endocrine Abstracts

Objective: To audit the benefits of MIBG and familial screening in patients with a phaeochromocytoma or paraganglionoma.

Results: 77 patients (male:female 34:43) have been seen within our hospitals since 1985. Further information has been located on 75. Patients had a median age of 44.3 years (range 19.4–81.2) and a median follow up of 12.0 months postoperatively (range 0–12 yrs). The adenoma was unilateral in 65 cases (86%, left 25, right 40) and bilateral in five cases (7% 2 Von Hippel-Lindau disease (VHL) 1 Multiple Endocrine Neoplasia Type 2A (MEN2A) 2 Sporadic). Five cases (7%) were paraganglionomas. 16 cases (21%) were familial (4 MEN2A, 7 Neurofibromatosis type 1 (NF1), 5 VHL). In all cases the familial syndrome had been identified prior to the diagnosis of a phaeochromocytoma. Of the 59 sporadic cases, 51 were screened for MEN2A and 33 were screened for VHL.

MIBG confirmed the lesion shown on MRI\CT in 32 out of 45 cases. On one occasion MIBG identified a lesion not detected on CT. The false negative rate was 29%. The result only effected management on one occasion.

69 patients have had an operation (35 open, 34 laparoscopic). 55 normalised their urinary metadrenalines and six were clinically cured. 8 patients were returned to their referring physician for postoperative follow up. Recurrence was <3%.

Conclusion: Routine MIBG and screening for familial syndromes cannot be recommended but MIBG may have a role if the lesion is not identified on CT\MRI scanning. Genetic screening may be more useful and enables testing for SDHD, SDHC and SDHB as well.