Endocrine Abstracts

Fifty patients (male:female 23:27) with a phaeochromocytoma or paraganglionoma have been seen within the Oxford hospitals since 1985. Further information has been located on 48. Patients had a median age of 42.6 years (range 19.4-74.6) and a median follow up of 13.9 months postoperatively (range 0.6-146.3). The adenoma was unilateral in 44 cases (88%, left 20, right 22) and bilateral in 4 cases (8% 2 Von Hippel-Lindau disease (VHL) 1 Neurofibromatosis type 1 (NF1) 1 Sporadic). Two cases (4%) were paraganglionomas. 12 cases (25%) were familial (3 Multiple endocrine neoplasia type 2 (MEN2), 4 NF1, 5 VHL) but in all cases the familial syndrome had been identified prior to the diagnosis of a phaeochromocytoma. Of the 36 sporadic cases, 29 were screened for MEN2 (7 calcium only). 18 were screened for VHL (4 MRI cerebellum and abdomen only). One individual with bilateral phaeochromocytomas and a renal cell carcinoma was thought to have VHL but was negative on genetic screening. No new familial cases were detected.
MIBG scanning was performed in 32 cases (28 sporadic 4 familial). MIBG confirmed the lesion shown on MRI\CT in 21 cases. On one occasion in 1985 MIBG identified a lesion not detected on CT. Sensitivity was therefore 66% and the result only effected management on one occasion.
43 patients had an operation (29 open, 14 laparoscopic). 35 normalised their urinary metadrenalines and six were cured on clinical grounds. One patient with a malignant metastatic phaeochromocytoma died after 21 months. Another patient normalised his urinary metanepharines post-operatively but these have slowly increased and he is being investigated for a possible recurrence. Patients were returned to their referring physicians for long-term follow up and would have been re-referred should a recurrence have been identified. Recurrence therefore is <2%.
Routine MIBG and screening for familial syndromes on all cases cannot therefore be recommended but MIBG may have a role if the lesion is not identified on CT\MRI scanning.