ECE2022 Eposter Presentations Pituitary and Neuroendocrinology (211 abstracts)
Cushing’s disease in MEN 4 syndrome
Dina Rebrova 1 , Ekaterina Zgoda 1 , Leonid Krasnov 1 , Vladimir Rusakov 1 , Ilya Sleptsov 1 , Roman Chernikov 1 , Arseny Semenov 1 , 2 , Elisey Fedorov 1 , Igor Chinchuk 1 , Ilya Sablin 1 , Shamil Shihmagomedov 1 & Ilya Shcherbakov 1
1Saint Petersburg State University, Saint Petersburg State University Hospital, St. Petersburg, Russian Federation; 2Saint Petersburg State University, Medical Faculty, Saint-Petersburg, Russian Federation
In 2000 female, 28 y.o., admitted endocrinologist with headache, high blood pressure, dark skin and muscle weakness. Lab data showed high levels of ACTH and cortisol, low potassium level. According to MRI scans, pituitary gland seemed normal. On CT in right adrenal was found 32 mm mass with the native density of 20 HU. Diagnosed ACTH-dependent Cushing syndrome (CS). As Mitotane was not available in Russia at that time, she received ketokonazole due to progression of CS but it was canceled later due to intolerance. So, right adrenalectomy was performed. Histology: adrenal adenoma with adrenal hyperplasia. The cortisol level decreased and symptoms diminished. In 2002 pituitary adenoma of 6 mm appeared on MRI. It was resected endoscopically by transsphenoidal approach. Histology: chromophobe adenoma. However, the hypercortisolism and high level of ACTH remained. Later in 2002 she underwent the proton beam surgery with following disease remission. At 2012 the patient had noticed the deterioration of health: skin darkening, high levels of blood pressure, hyperglycaemia. In 2015 the relapse of hypercortisolism was confirmed. According to MRI there were signs of pituitary adenoma relapse (partial empty sella). CT scan showed two adrenal masses up to 23 mm and 10 mm in the left adrenal. Second transsphenoidal partial removal of pituitary adenoma was performed, complicated by liquorrhea, purulent meningitis, secondary hypothyroidism. Second relapse of hypercortisolism with high level of ACTH with no signs of pituitary regrowth on MRI scan occurred in 2018. Ketoconazole therapy was tried once again with bad tolerance and no effect. Right adrenalectomy was performed. The patient is prescribed with adrenal replacement therapy (hydrocortisone 45 mg and fludrocortisone 0,1 mg daily), levothyroxine 100 mkg daily. Levels of ACTH are still persistently high. In 2020 the patient was diagnosed with papillary thyroid cancer and primary hyperparathyroidism. She received right hemithyroidectomy with central neck lymph node dissection, selective parathyroidectomy (lower right and higher left glands). No mutations in MEN1 gene were found. It is known that patient’s mother was operated on thyroid gland due to papillary cancer. MEN4 syndrome was described in 2010. We present the patient with 3 MEN4 tumors in absence of MEN1 gene mutations, burdened family history.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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