ACTH ectopic syndrome caused by corticomedullary mixed tumor

Ивановна, Марова Евгения; Сергеевич, Кузнецов Николай; Яковлевна, Рожинская Людмила; Валерьевич, Ремизов Олег; Вадимовна, Латкина Нонна; Владимировна, Тихонова Ольга; Ефимовна, Кац Любовь; Александровна, Воронкова Ия; Михайловна, Лапшина Анастасия; Сергеевна, Колесникова Галина

doi:10.14341/probl201662150-54

Probl Endokrinol (Mosk)

2016

DOI: 10.14341/probl201662150-54

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ACTH ectopic syndrome caused by corticomedullary mixed tumor

Марова Евгения Ивановна¹,

Кузнецов Николай Сергеевич²,

Рожинская Людмила Яковлевна³

et al.

Abstract: We report the case of a 61 year old woman with a typical clinical picture of ACTH dependent Cushing’s syndrome. Diagnostic tests confirmed ACTH dependent Cushing’s syndrome, which is caused by corticomedullary mixed tumor. ACTH-secreting pheochromocytoma are rarely described in accessible literature, there is no one case report about ACTH-secreting corticomedullary mixed tumor. Due to the rarity of the disease, we believe it appropriate to share our experience and consider all variants of interaction in hypoth… Show more

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2023

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Cited by 1 publication

References 11 publications

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Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

Rebrova,

Grigorova,

Vorokhobina

et al. 2023

Probl Endokrinol (Mosk)

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A clinical case of a man 66 y.o. who was diagnosed with hormone-inactive pituitary macroadenoma complicated by corneal erosion and partial atrophy of the optic nerve of the left eye due to exophthalmos. The increase in prolactin level was regarded due to a «stalk-effect». The patient underwent a transnasal pituitary adenomectomy with subsequent regression of symptoms. After 4 years, against the background of a new coronavirus infection, increasing general weakness, headaches, a crisis increase in blood pressure and tachycardia attacks appeared. Computed tomography (CT) accidentally revealed an adrenal incidentaloma, in laboratory tests — hypercortisolism, elevated ACTH levels, hypokalemia, hyperglycemia, increased levels of metanephrine and normetanephrine. The patient developed acute steroid psychosis, after which an adrenalectomy with a tumor was performed, a pheochromocytoma was histologically confirmed. After surgery, there was a regression of symptoms, the development of adrenal insufficiency with reduced levels of ACTH and cortisol. Upon further examination, a polynodose euthyroid goiter was established, the biopsy of the nodes — Hashimoto’s thyroiditis (Bethesda II). Meanwhile, primary hyperparathyroidism was detected. According to ultrasound, scintigraphy with Ts99m-Technetril and CT revealed an increase of left parathyroid gland. A bilateral revision of the neck, removal of the right upper and left upper parathyroid adenomas were performed. In the postoperative period, the levels of calcium and parathyroid hormone were normalized. Given the presence of a combination of multiple tumors of the endocrine system (primary hyperparathyroidism, corticotropin-producing pheochromocytoma, hormone-inactive pituitary macroadenoma, polynodose euthyroid goiter), the MEN1 syndrome was clinically established. The study of 2 and 10 exons of the MEN1 gene revealed no mutations, which does not exclude the presence of a hereditary syndrome. The patient continues observation. In the available literature in Russian and English languages the case of ACTH pheochromocytoma as part of the MEN type 1 syndrome have not been found. Therefore, we consider the presented case to be the first one.

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Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

Rebrova,

Grigorova,

Vorokhobina

et al. 2023

Probl Endokrinol (Mosk)

View full text Add to dashboard Cite

show abstract

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ACTH ectopic syndrome caused by corticomedullary mixed tumor

Cited by 1 publication

References 11 publications

Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

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