ECE2015 Eposter Presentations Adrenal cortex (94 abstracts)
A challenging case of paraneoplastic Cushing's syndrome
Adina Mazilu 1 , Mona Gheorghiu 2 , Madalina Musat 2 , Aurelian-Emil Ranetti 1, , Victor-Catalin Mazilu 1 , Narcis Tanase 1 , Razvan Petrescu 1 , Adrian Ciuche 1 , Augustin Tudose 1 , Florina Vasilescu 1 & Carmen Ardeleanu 3
1Central Military Emergency Hospital, Bucharest, Romania; 2National Institute of Endocrinology ‘C.I.Parhon’, Bucharest, Romania; 3Monza Hospital, Bucharest, Romania.
Introduction: Paraneoplastic Cushing’ syndrome (CS) represents 5–10% of all CS and has a severe prognosis due to severe metabolic imbalance, denutrition, associated infections, and progression of tumoural underlying pathology.
A 67 years old woman presented with mental confusion, progressive weight loss, severe oedema and kypokalaemia, without typical features of Cushing or hyperpigmentation. Investigations revealed paraneoplastic Cushing’s with ACTH 82.5 pg/ml, cortisol levels more than 63 μg/dl, probably produced by pulmonary tumour. Patient associated also empty sella syndrome with TSH and gonadotrophic insufficiency, left breast tumour, Helicobacter pylori gastritis, polinodular goitre, denutrition, and hepatic dysfunction. 99m-Tc tektrotyd scintigraphy was negative for pulmonary tumor, showing uptake in both adrenals and ileum. Patient needed 1200 mg/day ketoconazole and 200 mg/day of mifepristone in order to obtain normal cortisol levels and metabolic parameters. Owing to pulmonary sepsis and hepatic dysfunction we choose left adrenalectomy as first intervention, allowing reduction of ketoconazole to 600 mg/day and secondary left pulmonary lobe resection. Even if ketoconazole was stopped 1 day prior to adrenalectomy, patient developed adrenal insufficiency, needing noradrenaline support for 1 day and hydrocortisone replacement for 2 days. Histology exam showed benign bronchial carcinoid with ACTH staining and benign hyperplasia of left adrenal. Patient recovered almost completely. Mild hypokaliaemia and hypomagnesemia, even with oral supplementation, sartan therapy and normal levels of cortisol and ACTH persisted after surgery, probably due to severe deficit of intracellular compartment.
Conclusion: This case was difficult due to metabolic challenges, multiple associated pathology, lack of SSTR2 and SSTR5 receptors with negative scan, mild elevation of cromogranine A levels despite a typical bronchial carcinoid. Patient’s sister was operated for adrenal adenoma confirmed on histology exam, her daughter had papillary thyroid cancer, but no MEN association was proven in this family.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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