Pheochromocytoma

Бельцевич, Д. Г.; Трошина, Е. А.; Юкина, М. Ю.

doi:10.14341/probl201056163-71

Cited by 8 publications

(6 citation statements)

References 13 publications

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“…Феохромоцитома может возникнуть в любом возрасте, но наиболее часто -между 20 и 40 годами. Частота развития ФХЦ у взрослых мужчин и женщин одинакова [19]. При анализе данных литературы, посвященных ФХЦ с АКТГ-ЭС, мы получили, что данная патология чаще встречается у женщин, соотношение Ж/М=11/4 (всего 15 случаев) в возрасте 45,5±12,5 лет (самой младшей было 15 лет, а старшей 62 года) (табл.…”

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“…Данный симптом может быть отнесен как к проявлению АКТГ-ЭС, так и к ФХЦ. Артериальная гипертензия (АГ) встречается в 90% случаев при АКТГ-ЭС, а постоянная форма повышения АД при ФХЦ -в 10-50% случаев [19,22] (табл. 1).…”

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“…У одной из больных была зафиксирована тахикардия. Данный симптом также может быть отнесен к проявлению обоих заболеваний, но более характерен для ФХЦ и связан с воздействием норадреналина (НА) на β 1 -рецепторы сердца, избыточно продуцируемого тканью ФХЦ [19]. Эта же пациентка предъявила жалобы на учащенное мочеиспускание, что более свойственно проявлению ФХЦ (табл.…”

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“…2). Уровень МПК представляет собой интегративный показатель опухолевой активности за 24 ч. Указанный метод обладает высокой чувствительностью (93%) и специфичностью (75%) [19]. По результатам исследований у 3 женщин было обнаружено повышение метанефринов (1553, 1481 и 638 мкг/сут при норме до 312 мкг /сут) и норметанефринов (640, 830 и 545,6 мкг/сут при норме до 445 мкг/сут).…”

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“…Прогноз у описываемых пациенток относительно благоприятный, поскольку частота выявления метастатического поражения при ФХЦ колеблется от 3 до 36%, а маленькие ФХЦ (диаметр менее 5 см) редко метастазируют [19]. К тому же, наличие в данных удаленных новообразованиях рецепторов к соматостатину позволяет обсуждать, при необходимости, возможность применения биотерапии для данной группы пациентов.…”

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Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

et al. 2015

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Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS), accounts for about 10–20% of Cushing’s syndrome (CS). Ectopic hormone-secreting pheochromocytomas (Pheo) are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome.We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHC)of these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5) was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.

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Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

et al. 2015

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Metastatic risk factors in pheochromocytoma/paraganglioma

Rebrova,

Loginova,

Vorobyev

et al. 2023

Probl Endokrinol (Mosk)

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Currently, all pheochromocytoma/paraganglioma (PPGLs) are considered malignant due to metastatic potential. Consequently, PPGLs are divided into «metastatic» and «non-metastatic». Metastatic PPGLs can be with synchronous metastasis (metastases appear simultaneously with the identified primary tumor) or metachronous (metastases develop after removal of the primary tumor). The term metastatic PPGLs is not used in the presence of tumor invasion into surrounding organs and tissues, without the presence of distant metastases of lymphogenic or hematogenic origin. It is generally believed that about 10% of pheochromocytomas and about 40% of sympathetic paragangliomas have metastatic potential. On average, the prevalence of PPGLs with the presence of metastases is 15–20%. Risk factors for metastatic PPGLs are widely discussed in the literature, the most significant of which are groups of clinical, morphological and genetic characteristics. The review presents a discussion of such risk factors for metastatic PPGLs as age, localization and type of hormonal secretion of the tumor, the size and growth pattern of the adrenal lesion, the presence of necrosis and invasion into the vessels, the tumor capsule surrounding adipose tissue, high cellular and mitotic activity, Ki-67 index, expression of chromogranin B and S100 protein, the presence of genetic mutations of three main clusters (pseudohypoxia, kinase signaling and Wnt signaling).Over the past two decades, a number of authors have proposed various predictor factors and scales for assessing a probability of metastatic PPGLs. The review contains detailed description and comparison of sensitivity and specificity of such predictor scales as PASS, GAPP, M-GAPP, ASES and COPPS.

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Atypical and typical course of neurofibromatosis type 1 in combination with pheochromocytoma

et al. 2022

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Neurofibromatosis type 1 is a hereditary disease that has a multisystem character of organism damage, a wide variability of clinical manifestations, up to the almost complete absence of typical symptoms. Phenotypic manifestations, their expressiveness and heaviness can be varied even among members of the same family with identical mutations. One of the possible clinical manifestations of this pathology is pheochromocytoma, the development of which is associated with a high risk of developing life-threatening conditions. Timely diagnosis of the disease, the choice of treatment tactics for the patient, genetic testing of blood relatives can significantly improve the survival rate and prognosis of the disease. In this article, on the presented clinical examples of patients with a typical and atypical course of type 1 neurofibromatosis in combination with pheochromocytoma, the issues of managing patients with this pathology are outlined.

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Pheochromocytoma

Cited by 8 publications

References 13 publications

Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

Metastatic risk factors in pheochromocytoma/paraganglioma

Atypical and typical course of neurofibromatosis type 1 in combination with pheochromocytoma

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