Endocrine Abstracts

Introduction: Pheochromocytomas (adrenal and extra- adrenal/Paragangliomas) are rare catecholamine producing tumors and required complex dedicated MDTintervention. We preliminarily reported our service in a tertiary referral centre over 10 years (2008–2018).

Method: A retrospectively review of confirmed phaeochromocytoma were carried out by reviewing clinical correspondences and ICE investigation-result system (laboratory, radiology and histology.)

Results: Phaeochromocytoma was confirmed in 51 cases (30 female −59%) with mean age 54.5 years(range 20–85), 4(7.8%) metastasis, 4(7.8%) extra-adrenal phaeochromocytoma. At presentation, 21(41%) had hypertension, 20(39%) had paroxysmal symptoms. 34 (67%) presented as incidentaloma in which only 14 (41%) were truly asymptomatic but 9(26%) hypertension, 7(21%) paroxysmal symptoms, 4(12%) both symptoms and 4(12%) had phenotype of phaeochromocytoma syndromes. In term of treatment, 94% received alpha-blocker, remainder 6% where diagnosis was made histologically at post-op period (4% non-secretary at pre-op assessment). 46(90%) underwent surgery, 1(2%) move away before surgery, 4(8%) were unfit for surgery and managed conservatively. There was no mortality at immediate post op for those who underwent surgery. 43 (84%) is currently cured (normal urine/plasma metanephrine on last measurement), 2 (4%) patients awaiting follow up. 4 (8%) patients have died at the time of review (1 died from unrelated condition, 2 was treated conservatively and 1 had rapid progressive metastasis post surgery.)

Discussion: Number of Phaeochromocytoma referral to our centre has increased recently due to increased referral from other endocrine centres and increasing incident of adrenal incidentaloma. In the later cases, being major presenting feature in our series, most symptoms were missed, and diagnosis was delayed until their presentation as incidentaloma. It highlights the diagnosis challenges. No peri-operative mortality suggested our cohort have received optimal pre, during and post-op cares. Further review is underway for detail of morbidity, histology and genetic testing.