Background: Phaeochromocytomas (PHAEO) and paragangliomas (PGGL) are rare neuroendocrine tumours. The traditional 10% teaching mnemonic has recently been challenged.
Methods: Clinical and biochemical/pathological data were collected prospectively. A 24-h urine sample for metanephrine assay was used for postoperative biochemical follow-up.
Results: Between Jan 89 and June 10 110 patients were operated for PHAEO or PGGL. Thirty-four patients were lost to follow-up and further analysis was made on 76 patients (35 M:41 F, age 587 years, median 48 years) with PGGL (n=2) and unilateral (n=67) or bilateral (n=7) PHAEOs (n=74). One malignant metastatic PHAEO was diagnosed on presentation. Five PHAEOs presented in childhood (ages 517 years), of which two recurred 16 years later.
Syndromic disease was present in 18 patients with MEN-2A (n=5), VHL (n=7) or neurofibromatosis 1 (n=6). A further 11 patients (2974 years) demonstrated no mutation (n=9) or mutations of unknown clinical significance (n=2). Forty-seven patients (2187 years) either declined genetic testing or presented over 50 years.
Patients underwent laparoscopic (n=49), open (n=13) or open-on-bypass (n=2) adrenalectomy (operation details were missing in 12 patients). During the follow-up period (range 1210 months, median 48 months) eight patients died of unrelated malignant conditions (n=6) or unknown cause (n=2).
Recurrent disease (n=8) of local abdominal PHAEO (n=7, with two occurring contralaterally) and PGGL (n=1) was diagnosed at 24210 months (median 108 months) after initial laparoscopic (n=3), open (n=4) or open bilateral (n=1) adrenalectomy. These patients (4 M:4 F, age 572 years, median 57 years) presented with familial VHL (n=2) or sporadic malignant tumours (n=6).
Conclusion: This study demonstrates the importance of life-long follow-up of PHAEOs, particularly for those who presented in childhood. It questions the original 10% mnemonic, showing that, in this surgical series, 24% of PHAEOs are familial, 7% occur in childhood, 11% recur, 3% are extra adrenal, 9% are bilateral and 1% are metastatic malignant on initial presentation.