ACTH-Secreting Pheochromocytoma. Case report

Кузнецов, Н С; Марова, Е И; Latkina, N V; Dobreva, Ekaterina; Krylov, Vadim; Kats, L E; Remizov, O. V.; Voronkova, I A

doi:10.14341/2306-3513-2012-4-43-50

Cited by 5 publications

(3 citation statements)

References 7 publications

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“…Феохромоцитома (ФЕО) и параганглиома (ПГ) -опухоли хромаффинной ткани, продуцирующие катехоламины (адреналин, норадреналин, дофамин). В связи с нейроэндокринным происхождением данных неоплазий описаны крайне редкие случаи эктопической секреции и других пептидных гормонов, таких как вазоактивный интестинальный пептид, соматостатин, нейропептид Y, метэнкефалин, кальцитонин, АКТГ [7][8][9].…”

Section: Discussionunclassified

“…АКТГ-эктопический синдром -крайне редкое заболевание, ассоциированное с гиперпродукцией кортикотропина опухолями различной локализации, чаще всего раком легких или карциноидом бронхов. В литературе описано около 65 пациентов с АКТГ-секретирующей ФЕО [9,[13][14][15][16][17]. Клиническую картину больных с подобными опухолями объединяют жалобы на общую и мышечную слабость, снижение массы тела, повышенный уровень АД.…”

Section: Discussionunclassified

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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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Section: Discussionunclassified

Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

Rebrova,

Grigorova,

Vorokhobina

et al. 2023

Probl Endokrinol (Mosk)

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“…Выявленная в нашем клиническом примере двусторонняя гиперплазия мозгового слоя надпочечников и результаты иммуногистохимического исследования (около 30% клеток мозгового слоя надпочечников экспрессируют АКТГ) с большой долей вероятности позволяют рассматривать ее в качестве возможного очага АКТГ-эктопии с соответствующей диффузно-микронодулярной гиперплазией коры надпочечников, в основном за счет пучковой зоны. По данным литературы [9,11,12], на долю феохромоцитом действительно приходится по меньшей мере 2,3-5,6% всех случаев АКТГ-эктопий. Однако у данного пациента феохромоцитома была исключена на дооперационном этапе на основании нормального уровня метилированных катехоламинов в суточной моче и отсутствия очагов патологического накопления 18 F-фтордезоксиглюкозы при позитронно-эмиссионной томографии.…”

Section: Discussionunclassified

Syndrome of ectopic ACTH secretion in a patient without a verified tumor

et al. 2018

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Рrevalence of endogenous hypercortisilism is about 56 cases per 1 million people. ACTH-dependent hypercortico- sis in the general structure is about 80%. Among this cohort of patients in 7075% the cause of hypersecretion of ACTH is corticotrophinoma, about 10% are diagnosed with hyperplasia of corticotrophs, in the remaining 1520% neuroendocrine tumors of different locations, producing ACTH and / or corticoliberin. With ectopic ACTH syndrome the development of clinical manifestations is faster than with corticotropinoma, which determines the nature of the clinical picture. Difficulties in identifying the primary focus hypersecretion of ACTH may be due to its small size, absence or nonspecificity of the clinical picture from the primary focus, early metastasis in the case of malignant tumors. Besides, clinical manifestations of hyper- cortisolism can be considered and as independent nosological units, which creates certain difficulties at the stages of early diagnosis and timely treatment of ectopic ACTH syndrome.

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Ectopic ACTH- and/or CRH-Producing Pheochromocytomas

Elliott

Berhane

Ragnarsson

et al. 2020

The Journal of Clinical Endocrinology &Amp; Metabolism

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Context The characteristics of catecholamine-secreting pheochromocytomas have been well studied. However, less is known about the characteristics, management and outcome in patients with ectopic adrenocorticotropic hormone (ACTH) and/or corticotrophin releasing hormone (CRH)-secreting pheochromocytomas. Objective To review the characteristics and outcomes of ACTH- and/or CRH-secreting pheochromocytomas. Data Source A systematic search of PubMed/MEDLINE and Web of Science, identifying relevant reports published up to 10 February 2020. Study Selection Original articles, including case reports and case series, reporting individual patient data from patients with ACTH and/or CRH-secreting pheochromocytomas. Data extraction Information on sex, age, symptoms at presentation, comorbidities, biochemistry, imaging, histopathology, and outcomes was extracted. Data Synthesis We identified 91 articles reporting on 99 cases of ACTH and/or CRH-secreting pheochromocytomas (CRH-secreting n=4). Median age at diagnosis was 49 years (IQR 38, 59.5) with a 2:1 female to male ratio. Most patients presented with clinical Cushing syndrome (n = 79; 81%), hypertension (n = 87; 93%), and/or diabetes (n = 50; 54%). Blood pressure, glucose control, and biochemical parameters improved in the vast majority of patients postoperatively. Infections were the most common complication. Most cases (n=70, 88%) with reported long-term outcome survived to publication (median follow-up 6 months). Conclusion Ectopic ACTH- and/or CRH-secreting pheochromocytoma should be considered in patients presenting with ACTH-dependent Cushing syndrome and adrenal mass. Despite the challenge in diagnosis, patient outcomes appear favourable.

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ACTH-Secreting Pheochromocytoma. Case report

Cited by 5 publications

References 7 publications

Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

Corticotropin-producing pheochromocytoma in multiple endocrine neoplasia type 1

Syndrome of ectopic ACTH secretion in a patient without a verified tumor

Ectopic ACTH- and/or CRH-Producing Pheochromocytomas

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