Endocrine Abstracts

We present the case of a 36-year-old man presenting with rapid onset of symptoms of Cushing’s syndrome, including proximal myopathy, weight gain, new onset diabetes and hypertension, striae and bruising. He had elevated random cortisol of 1123 nmol/l, Midnight Salivary cortisol 30.1ng/ml (ref range <2.1 ng/ml) and Urine free cortisol 10,000nmol/ 24 hrs (ref range 0-486), ACTH 326ng/l (0-46), conforming ACTH dependent Cushing’s syndrome. Pituitary MRI was normal. Inferior Petrosal sinus sampling ruled out pituitary cause for Cushing’s. CT and FDG PET scan showed Bilateral adrenal hyperplasia and widespread pulmonary lymphadenopathy with fissural nodules, suggestive of sarcoidosis. No primary source was identified for ectopic Cushing’s, and he underwent bilateral adrenalectomy due to progressive symptoms. He developed erythema nodosum and arthritis and was started on Mycophenolate mofetil. Ga 68-dotatate scan revealed RT-perihilar avid focus, presumed to be ACTH-secreting primary pulmonary tumour, with a hepatic focus suspicious for metastasis. Transbronchial biopsy and liver biopsy were nondiagnostic. Primary lung surgery and radiotherapy were felt to be high-risk with sarcoidosis. Imaging surveillance continued and patient started on Somatostatin analogue therapy. Following bilateral adrenalectomy, he developed worsening of sarcoidosis (assumed due to removal of endogenous steroids,). He was treated with prednisolone, and azathioprine initially but the sarcoidosis is progressing and infliximab is being planned. Repeat Ga 68-dotatate scan demonstrated liver metastasis progression, with ACTH 8180ng/l (0-46). One enlarging liver lesion was targeted for biopsy, which confirmed a well-differentiated (G2) Ki 15% NET, with possible GI-tract origin on immunoprofiling (positive for serotonin, negative for CDX2,TTF-1 and PAX80) In view of progression on SSA-therapy, PRRT has been considered, though a worsening renal function, precludes this treatment. Systemic therapy is being considered at present. This case illustrates challenging treatment of two conditions with, at times, opposing treatment needs. Uncontrolled Cushing’s syndrome poses a significant risk to life and necessitated bilateral adrenalectomy, which in turn worsened the outlook from sarcoidosis. Immunosuppressive therapy for Sarcoidosis has the potential to impact treatment of the neuroendocrine tumour. The concomitant presence of both conditions poses challenges for treatment optimisation and prioritisation and necessitates multimodal and multidisciplinary treatment plans.