Endocrine Abstracts

Introduction: We present cases of metastatic pheochromocytoma highlighting diagnostic, management, and therapeutic challenges.

Case-1: 69 Female, presented with breathlessness, palpitations, and sweating of 4 months. Panic attacks of 10 years. Imaging confirmed left-sided pheochromocytoma, bone, liver, and pulmonary metastases. Plasma metadrenaline (MT) 4471 pmol/l (0-510), normetadrenaline (NMT) >25000 pmol/l (0-1180) LVEF 30%, global LV hypokinesia. Decompensated heart failure, LVEF 5-10%, non-sustained VT despite blockade. Following MDT discussion, left adrenalectomy, splenectomy, and liver resection with Impella (extracorporeal LVAD) support completed, with subsequent gradual cardiac improvement. Large, right humeral metastasis required resection, without mechanical cardiac support for symptom management. Awaiting MIBG-therapy. Genetics negative.

Case-2: 69 Male, b/g Parkinson’s disease, resistant hypertension, CKD 4. Admitted with acute STEMI, and significant blood pressure fluctuations. Plasma metanephrines were raised given caveats of co-beneldopa and acute MI repeat confirmed plasma NMT >25000. Imaging confirmed left phaeochromocytoma (100x115x80mm) with liver and bilateral lung metastases. Detailed pre-operative assessment and MDT discussions agreed that major debulking surgery, chemotherapy or MIBG therapy were not in patients’ best interest. Currently awaiting SSTR PET CT to assess suitability of Somatostatin analogue (SSA) therapy. Genetics pending.

Case-3: 63 Female, right pheochromocytoma (MIBG avid) 2014, presented with collapse, multi-organ dysfunction, Tukotsubo-cardiomyopathy, LVEF 8% requiring ECMO. Underwent right adrenalectomy (2015). Genetics negative. Recurrent symptoms and raised metanephrines in 2019; ⁶⁸Ga-DOTATATE scan confirmed kidney, liver, pelvis, spine, and lung metastasis. SSA therapy commenced 2022. Ga-DOTATATE updated scan 2023 (UK) confirmed avid progressive bone metastases with reduction of soft tissue disease. Plasma MT >25000, NMT 18500. Oral prednisolone 20 mg daily for ITP treatment precipitated pheochromocytoma-crisis with, with severe constipation (extreme faecal loading, dilated small and large bowel). Admitted to ITU for stabilisation. Patient awaiting MIBG therapy.

Conclusion: Management of metastatic pheochromocytoma in dedicated centres with multidisciplinary team, tailoring care to patients is required.