Endocrine Abstracts

We present a 41 years old lady who presented with non specifc symptoms- lethargy, general myalgia, memory impairment. She also complained of hot flushes, sweats and had a brief episode of panic attacks hence was commence on Sertraline by the GP. She has a past medical history of appendicectomy. Her mother had lymphoma and mother died from lung cancer. She is a smoker, however is teetotal. She has 3 children and works in a laundry. She was initially referred to the haematologist for investigation of lymphocytosis and neutrophiliawho subsequently discovered a 51mm left adrenal mass ( not typical of an adenoma) raising the possibility of a phaeochromocytoma or adrenal cortical carcinoma through a CT scan subsequently characterised byfurther MRI scans. On clinical examination, her blood pressure was 126/73 sitting and 125/84 standing with a regular pulse of 82bpm. She has no cushingoid features and no proximal myopathy. Her investigations showed a normal cortisol 18 nmol/l post overnight dexamethasone suppression test. Raised Metanephrines 6.8 umol/24 h (0-3 umol/24 hr), Raised Normetanepherine 7.35 umol/24 h (0-1.14 umol/24 h), 3-methoxytyramine 2.11 umol/24 h Normal. Aldosterone renin ratio, DHEA 3.70 umol/l normal, Aldosterone renin ratio 25.3 pmol/mU Normal She was alpha and beta blocked prior to surgery. She had a left laparoscopic adrenalectomy where her histology results showed a mixed composite of phaeochromcytoma/ paraganglioma with neuroblastic elements. Immunohistochemistry showed some areas staining strongly with chromogranin and synaptophysin while other areas are weaker. The Ki67 proliferation index is <1%. Post operatively, she had a 123I-mIBG scintigraphy scan which show physiological tracer distribution with no abnormal focal mIBG avidity. She consented for genetic testing and undergone the pheochromocytoma and paraganglioma gene paneltesting (FH, MAX, RET exons 10&11, SDHA, SDHAF1, SDHB, SDHC, SDHD, TMEM127, VHL) with no alteration identified. Her RET gene testing (exons 5, 8, 10, 11, 13, 14, 15 and 16) is also normal. She was offered entry into the molecular pathology of human genetic diseasese Post operatively, she recovered well, her subsequent 24 hour metanephrines normalised. Serial CT scans show no disease recurrence so far. Adjuvant chemotherapy was also discussed with the oncology team. However, this wasn’t deemed necessary and surveillance was advocated. This is a very rare tumour with few case series reported. This case adds to the sparse literature on this type of tumour and uncertainties regarding longterm management.