pheochromocytoma treatment

Gastrointestinal stromal tumors (GISTs) represent a rare form of gastrointestinal neoplasm. This report details a medical case involving a 44-year-old woman who underwent bilateral pheochromocytoma resection, GIST gastrectomy, and laparoscopic adrenalectomy with intestinal resection. Despite an initially positive response to oral imatinib, treatment was delayed due to economic constraints. This delay resulted in a critical event marked by abdominal GIST metastasis to the abdominal wall, subsequent rupture leading to hemoperitoneum, and emergency surgery...

INTRODUCTION: Pheochromocytomas are rare catecholamine-secreting tumors with a high potential for recurrence post-surgery, necessitating prolonged follow-up. This case highlights the diagnostic and therapeutic challenges in managing recurrent pheochromocytoma. CASE PRESENTATION: A 25-year-old female, with a history of left pheochromocytoma treated with adrenalectomy a decade earlier, presented with a right adrenal mass. Despite controlled hypertension, elevated urine metanephrines suggested recurrence...

BACKGROUND: Tumour-producing catecholamines arise in the adrenal medulla (pheochromocytomas), as well as in extra-adrenal chromaffin cells (paragangliomas). The origin can be from any location; however, it is very rare in the heart. CASE SUMMARY: A 43-year-old woman with a history of arterial hypertension presented with dyspnoea on moderate exertion, New York Class Association (NYHA) functional classes III and IV, and oedema in the lower extremities. Medical and laboratory evaluation revealed an NT-proBNP of 6046 pg/mL, a left ventricular ejection fraction (LVEF) of 15%, longitudinal strain of -7%, and a mass located on the inner surface of the left atrioventricular groove...

Pheochromocytomas are rare catecholamine-secreting tumors that occur in 0.002% of pregnancies. These tumors result in high maternal and fetal morbidity and mortality unless diagnosed in early stages of development, because excess levels of catecholamines cause vasoconstriction of both maternal and uteroplacental vasculature. Paroxysmal hypertension is the most common manifestation, but its variability in presentation and similarity to other pregnancy-related conditions often make diagnosis of pheochromocytoma difficult...

BACKGROUND: The aim of this study was to investigate the clinical features and treatment options for pediatric adrenal incidentalomas(AIs) to guide the diagnosis and treatment of these tumors. METHODS: The clinical data of AI patients admitted to our hospital between December 2016 and December 2022 were collected and retrospectively analyzed. All patients were divided into neonatal and nonneonatal groups according to their age at the time of the initial consultation...

BACKGROUND: Secondary hypertension (SH) is a form of high blood pressure caused by an identifiable underlying condition. Although, it accounts for a small fraction of the overall hypertensive population, detection and management of SH is of utmost importance, because SH phenotypes carry a high cardiovascular risk and can possibly be cured by timely treatment. CONTENT: This review focuses on the endocrine causes of SH, such as primary aldosteronism, Cushing syndrome, thyroid disease, pheochromocytoma and paraganglioma, acromegaly, and rare monogenic forms...

To compare the clinical efficacy and safety of robot-assisted adrenalectomy (RA) and laparoscopic adrenalectomy (LA) for pheochromocytoma (PHEO). We conducted a comprehensive search of PubMed, the Cochrane Library, and Embase databases for studies comparing RA and LA treatment for PHEO, covering the period from database inception to January 1, 2024. Two researchers will independently screen literature and extract data, followed by meta-analysis using Review Manager 5.3 software. Six studies with 658 patients were included in the analysis...

The latest trend shows a strong demand for minimally invasive surgery. The popularity of robot-assisted surgeries has increased because they eliminate many of the disadvantages of conventional laparoscopic methods.  However, compared to the conventional method of anesthesia treatment, robotic surgery may require adjustments in the way patients are positioned and the general arrangement of personnel and equipment. Pheochromocytomas (PHEOs) are tumours of neural crest cells that make catecholamines. Early detection and appropriate treatment are required for PHEO...

PURPOSE: Data guiding management of pheochromocytoma and paraganglioma (PPGL) in pregnant women is limited, and long-term effects on the child are unknown. The aim of this retrospective registry-based case-cohort study was to assess how maternal PPGL and treatment impacts maternal and fetal outcome, including long-term outcome for the child. The main outcomes were maternal and fetal mortality and morbidity at delivery and relative healthcare consumption in children born by mothers with PPGL during pregnancy...

Despite all recent progresses in nerve tissue engineering, critical-sized nerve defects are still extremely challenging to repair. Therefore, this study targets the bridging of critical nerve defects and promoting an oriented neuronal outgrowth by engineering innovative nerve guidance conduits (NGCs) synergistically possessing exclusive topographical, chemical, and mechanical cues. To do so, a mechanically adequate mixture of polycaprolactone (PCL) and polylactic- co -glycolic acid (PLGA) was first carefully selected as base material to electrospin nanofibrous NGCs simulating the extracellular matrix...

Von Hippiel-Lindau (VHL) disease is a rare genetic disorder characterized by a variety of benign and malignant neoplastic growths arising in multiple different organ systems. About 60%-84% of patients develop hemangioblastomas, benign tumors comprised of newly formed blood vessels that often occur in the central nervous system (CNS) and retinas. Treatment options for this disease were limited before the Food and Drug Administration (FDA) approval of belzutifan, a HIF2α inhibitor. We present a case of a 25-year-old woman with VHL who underwent treatment with belzutifan over 18 months...

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Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from neural crest cells from adrenal medullary chromaffin tissues or extra-adrenal paraganglia, respectively. Although the current treatment for PPGLs is surgery, optimal treatment options for advanced and metastatic cases have been limited. Hence, understanding the role of the immune system in PPGL tumorigenesis can provide essential knowledge for the development of better therapeutic and tumor management strategies, especially for those with advanced and metastatic PPGLs...

Background: Robotic adrenalectomy has become a surgical treatment option for benign and selected malignant adrenal diseases. We aimed to evaluate the eligibility of two-port robotic posterior retroperitoneoscopic adrenalectomy (PRA) as an alternative to the conventional three-port technique by comparing their surgical outcomes. Materials and Methods: This retrospective cohort study compared the clinicopathological factors and surgical outcomes among 197 patients who underwent two-port or three-port robotic adrenalectomy between 2016 and 2020 in a single tertiary center...

Metastatic pheochromocytoma/paraganglioma (MPP) is a rare endocrine tumor that originates from extra-adrenal chromaffin cells such as the paraganglia cells of sympathetic and parasympathetic nerves. It usually causes multiple solid tumors and exhibits strong aggressiveness with poor prognosis, with a reported 5-year survival rate of less than 50%. Cases of brain and retroperitoneal metastases at the initial diagnosis have not yet been reported. We report a 41-year-old male patient initially diagnosed with MPP in the brain and retroperitoneum who underwent multi-disciplinary collaborative surgery and simultaneous removal of two tumors at our center...

BACKGROUND: Malignant pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumors and available systemic therapies are limited. AIM: To explore the role of peptide receptor radionuclide therapy (PRRT) with Yttrium-90 (90 Y) and Lutetium-177 (177 Lu) peptides in pheochromocytomas (PCCs) and paragangliomas (PGLs). METHODS: We retrospectively analyzed more than 1500 patients with histologically proven neuroendocrine tumors treated with 177 Lu- or 90 Y-DOTA-TATE or -TOC between 1999 to 2017 at our Institute...

INTRODUCTION AND IMPORTANCE: Phaeochromocytomas are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and are known as intra-adrenal paragangliomas. On the contrary, paragangliomas are non-epithelial neuroendocrine neoplasms that produce other peptide hormones such as norepinephrine, epinephrine, and dopamine. In a setting with limited resources, such a condition may result in inaccurate diagnosis and treatment, which may lose a patient life if left untreated...

INTRODUCTION: To investigate the clinical features, pregnancy care, timing, and approaches of pregnancy termination as well as the perinatal management of pregnant women with ectopic pheochromocytomas (EPCC) (paragangliomas, PGL). METHODS: We report the diagnosis and treatment of a pregnant women with EPCC which was confirmed in the third trimester in our hospital. Literature in relation to EPCC during pregnancy both in and outside China was searched for data analysis such as maternal clinical features and maternal and fetal prognosis...

BACKGROUND: Neurotrophic activity constitutes a crucial factor in the recovery from neurological injuries and is impaired in neurodegenerative disorders. Preclinical studies of neurotrophic factors to improve outcome of neurodegenerative diseases have yielded promising results. However, due to the complexity of these therapies, the clinical translation of this approach was so far not successful and more feasible treatments with neurotrophic activity may be promising alternatives. Therefore, highly sensitive and robust assays for compound screening are required...

PURPOSE: Data regarding treatment options and their efficacy for metastatic paragangliomas (mPPGL) is limited. This study aims to report a single center experience in treating mPPGL, comparing the efficacy and safety of various treatment approaches. METHODS: Retrospective analysis of patients with mPPGL treated at an Endocrinology Department of a cancer institute between January 2000 and October 2022. RESULTS: We analyzed 25 patients with mPPGL, 8 pheochromocytomas and 20 paragangliomas (12% multifocal), followed for a median of 9 [4; 14] years...