Endocrine Abstracts

Case history: We present a case of 36Y old male who presented with worsening leg edema, leg ulceration and poor mobility leading to admission in intensive care due to sepsis and hemodynamic instability. Found to have metabolic alkalosis with hypokalaemia. Clinically, moon-like facies, low proximal muscle mass, skin hypopigmentation (mainly hands), broken skin fingers and legs, abdominal fat distribution. Investigations:Random cortisol 2348nmol/l, post 1mg dexamethasone cortisol 2886nmol/l, ACTH 443 ng/L, testosterone 4.0nmol/. Plasma metadrenalines and normetadrenalines were >25000 pmol/l. CT CAP/Adrenals – Left 7.3 cm adrenal mass consistent with ACC with multiple lung metastases lung lesions were investigated and found to be inflammatory. MRI Pituitary normal. FDG PET showed metabolically active left adrenal malignancy. The new bilateral pulmonary changes are likely inflammatory/infective. The pulmonary nodules which were seen on CT CAP were thought to be likely metastasis. MIBG was consistent with a diagnosis of Phaeochoromocytoma. Treatment: Was initiated on metyrapone and alpha blockade. He improved markedly after starting metyrapone. Underwent left open adrenalectomy. Histology was in keeping with Pheochromocytoma with capsular irregularities. Ki67=7.4%. Capsular and lymphovascular invasion. PASS score was 10. Immunohistochemistry SDHB preserved. Post- operatively results showed normal ONDST and plasma metanephrines. SST demonstrated adrenal insufficiency and remains on hydrocortisone. Genetic testing did not reveal a genetic cause of phaeochromocytoma.

Conclusion and points of discussions: ACTH secreting phaeochromocytoma is extremely rare but should be considered in the differential diagnosis.References1. Clinical, biochemical, and tumour characteristics in patients with ectopic ACTH.2. The utilisation of different imaging modalities.3. Role of drugs such as Metyrapone, alpha blockade pre-operatively.4. Review of literature