6,454 results match your criteria patients pheochromocytoma


Tailored molecular imaging of pheochromocytoma and paraganglioma: which tracer and when.

Neuroendocrinology 2022 Jan 20. Epub 2022 Jan 20.

Pheochromocytoma and paraganglioma are rare neoplasms that fall within the category of neuroendocrine tumors. In the last decade, their diagnostic algorithm has been modified to include the evaluation of molecular pathways, genotype and biochemical phenotype, in order to correctly interpret anatomical and functional imaging results and tailor the best therapeutic choices to patients. More specifically, the identification of germline mutations has led to a three-way cluster classification: pseudo-hypoxic cluster, cluster of kinase receptor signaling and protein translation pathways, and cluster of Wnt-altered pathway. Read More

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January 2022

Simultaneous robotic resection of pheochromocytoma and gastrointestinal stromal tumour in an adult neurofibromatosis type 1 patient: A single-docking procedure using da Vinci XI platform.

J Minim Access Surg 2021 Oct 19. Epub 2021 Oct 19.

Department of Surgery, Faculty of Medicine, University of Ljubljana; Department for Urology, University Medical Centre Ljubljana, Slovenia.

The risk of tumours, including pheochromocytoma and gastrointestinal stromal tumour is higher in patients with neurofibromatosis Type 1 (NF1). The co-occurrence of these two tumours is rare, and most patients are symptomatic. In this case report we describe the case of an asymptomatic 42-year-old female patient with left adrenal mass and concurrent lesion in the jejunum, just distal to the ligament of Treitz. Read More

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October 2021

Surveillance improves outcomes for carriers of SDHB pathogenic variants: a multi-center study.

J Clin Endocrinol Metab 2022 Jan 17. Epub 2022 Jan 17.

Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, NSW, Australia.

Context: Carriers of succinate dehydrogenase type B (SDHB) pathogenic variants (PV) are at risk of pheochromocytoma and paraganglioma (PPGL) from a young age. It is widely recommended carriers enter a surveillance program to detect tumors but there are limited studies addressing outcomes of surveillance protocols for SDHB PV carriers.

Objective: The purpose of this study was to describe surveillance-detected (s-d) tumors in SDHB PV carriers enrolled in a surveillance program and to compare their outcomes to probands. Read More

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January 2022

Development of a radiomics model to diagnose pheochromocytoma preoperatively: a multicenter study with prospective validation.

J Transl Med 2022 Jan 15;20(1):31. Epub 2022 Jan 15.

Department of Urology, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, 107 Yan Jiang West Road, Guangzhou, 510120, Guangdong, People's Republic of China.

Background: Preoperative diagnosis of pheochromocytoma (PHEO) accurately impacts preoperative preparation and surgical outcome in PHEO patients. Highly reliable model to diagnose PHEO is lacking. We aimed to develop a magnetic resonance imaging (MRI)-based radiomic-clinical model to distinguish PHEO from adrenal lesions. Read More

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January 2022

Clinical significance of a 10-mm cutoff size for adrenal lesions: a retrospective study with 547 non-oncologic patients undergoing adrenal computed tomography.

Abdom Radiol (NY) 2022 Jan 13. Epub 2022 Jan 13.

Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, Republic of Korea.

Purpose: To investigate the proportion of clinically significant adrenal lesions in patients with a subcentimeter adrenal lesion, and the sensitivity of a cutoff size of 10 mm on computed tomography (CT).

Methods: This retrospective study included consecutive 547 non-oncologic patients who underwent adrenal CT. Clinically significant adrenal lesions were defined as those that were biochemically abnormal (n = 99) or surgically resected according to the clinician's decision (n = 23). Read More

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January 2022

Predicting surgical outcome in posterior retroperitoneoscopic adrenalectomy with the aid of a preoperative nomogram.

Surg Endosc 2022 Jan 13. Epub 2022 Jan 13.

Department of Urology, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.

Background: Posterior retroperitoneoscopic adrenalectomy (PRA) has several advantages over transperitoneal laparoscopic adrenalectomy (TLA) regarding operative time, blood loss, postoperative pain, and recovery. However, it can be a technically challenging procedure. To improve patient selection for PRA, we developed a preoperative nomogram to predict operative time. Read More

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January 2022

Phase 2 Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor.

Clin Cancer Res 2022 Jan 12. Epub 2022 Jan 12.

R&D, Oncoceutics Inc.

Purpose: Tumor dopamine-like DRD2 receptor expression is higher in pheochromocytoma-paraganglioma (PC-PG) compared to other cancers. ONC201 is a bitopic DRD2 antagonist with pre-clinical ONC201 activity in DSRCT.

Experimental Design: Patients (N=30) with neuroendocrine tumors were treated on this investigator-initiated trial (NCT03034200). Read More

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January 2022

[Bilateral adrenal incidentaloma in a patient with acute appendicitis].

Rev Med Inst Mex Seguro Soc 2021 Aug 2;59(4):352-357. Epub 2021 Aug 2.

Hospital Nacional Edgardo Rebagliati Martins, Servicio de Endocrinología. Lima, Perú.

Background: Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals . Read More

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Loss of SDHB Induces a Metabolic Switch in the hPheo1 Cell Line toward Enhanced OXPHOS.

Int J Mol Sci 2022 Jan 5;23(1). Epub 2022 Jan 5.

Department of Surgery and Department of Biomedical and Clinical Sciences (BKV), Linköping University, 581 83 Linköping, Sweden.

Background: Enzymes of tricarboxylic acid (TCA) have recently been recognized as tumor suppressors. Mutations in the SDHB subunit of succinate dehydrogenase (SDH) cause pheochromocytomas and paragangliomas (PCCs/PGLs) and predispose patients to malignant disease with poor prognosis.

Methods: Using the human pheochromocytoma cell line (hPheo1), we knocked down SDHB gene expression using CRISPR-cas9 technology. Read More

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January 2022

The association between the type of anesthesia and hemodynamic instability during pheochromocytoma surgery: a retrospective cohort study.

Surg Endosc 2022 Jan 10. Epub 2022 Jan 10.

Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Korea.

Background: Pheochromocytoma often carries a risk for perioperative hemodynamic instability (HDI). The aim of this study is to evaluate the risk factors of intraoperative HDI during minimally invasive posterior retroperitoneal adrenalectomy (PRA) for pheochromocytoma.

Materials And Methods: This retrospective study analyzed the prospectively collected data of 172 patients who underwent laparoscopic PRA or robotic PRA for pheochromocytoma between January 2014 and December 2020 at a single tertiary center. Read More

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January 2022

Multiple endocrine neoplasia 2A presenting in a family with a history of Hirschprung's disease.

Oxf Med Case Reports 2021 Nov-Dec;2021(11-12):omab122. Epub 2021 Dec 28.

Division of Endocrinology and Metabolism, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem 27157, USA.

Hirschprung's disease co-occurs with multiple endocrine neoplasia type 2A infrequently but at a higher rate with certain RET mutations. We present a case of a patient evaluated for an adrenal incidentaloma with a history of familial Hirschprung's. Our patient was found to have synchronous pheochromocytoma and medullary thyroid carcinoma illustrating the importance of genetic testing in these patients to determine appropriate screening for endocrine tumors. Read More

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December 2021

Bladder paraganglioma: CT and MR imaging characteristics in 16 patients.

Radiol Oncol 2021 Dec 30. Epub 2021 Dec 30.

Department of Radiology, First Medical Center, Chinese PLA General Hospital, Beijing, China.

Background: Bladder paraganglioma (BPG) is a rare extra-adrenal pheochromocytoma with variable symptoms and easy to be misdiagnosed and mishandled. The aim of the study was to document the imaging features of BPG using computed tomography (CT) and magnetic resonance imaging (MRI).

Patients And Methods: We retrospectively enrolled consecutive patients with pathology-proven BPG, who underwent CT or MRI examinations before surgery between October 2009 and October 2017. Read More

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December 2021

Von Hippel-Lindau Syndrome: Medical Syndrome or Surgical Syndrome? A Surgical Perspective.

J Kidney Cancer VHL 2022 5;9(1):27-32. Epub 2021 Dec 5.

Department of General Surgery, Carlo Urbani Hospital, Jesi, Italy.

Von Hippel-Lindau syndrome (VHL) is an autosomal dominant disease caused by a genetic aberration of the tumor suppressor gene VHL and characterized by multi-organ tumors. The most common neoplasm is retinal or cerebral hemangioblastoma, although spinal hemangioblastomas, Renal Clear Cell Carcinoma (RCCC), pheochromocytomas (Pheo), paragangliomas, Pancreatic Neuroendocrine Tumors (PNETs), cystadenomas of the epididymis, and tumors of the lymphatic sac can also be found. Neurological complications from retinal or CNS hemangioblastoma and metastases of RCCC are the most common causes of death. Read More

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December 2021

Title: Pheochromocytoma Crisis Rescued by Veno-Arterial Extracorporeal Membrane Oxygenation and Continuous Renal Replacement Therapy.

Am Surg 2021 Dec 28:31348211063573. Epub 2021 Dec 28.

Hepato-Biliary-Pancreatic Surgery Division, Department of Surgery, RinggoldID:13805National Center for Global Health and Medicine, Shinjuku-ku, Japan.

Pheochromocytoma is a rare catecholamine producing adrenal tumor. Pheochromocytoma crisis is a life-threatening condition inducing multiple organ failure and hemodynamic instability caused by too much catecholamines produced from pheochromocytoma. We report a 59-year-old woman with pheochromocytoma crisis rescued by veno-arterial extracorporeal membrane oxygenation (VA-ECMO), continuous renal replacement therapy (CRRT), and interval tumor resection. Read More

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December 2021

Catecholamine-induced cardiomyopathy: an endocrinologist's perspective.

Rev Cardiovasc Med 2021 Dec;22(4):1215-1228

Department of Endocrinology and Metabolism, Pilgrim Hospital, PE21 9QS Boston, UK.

Although many endocrine diseases can be associated with acquired cardiomyopathy and heart failure, conditions except hypothyroidism, hyperthyroidism, phaeochromocytoma-paraganglioma (PPGL), and primary hyperaldosteronism are rare. PPGL is a rare catecholamine-secreting neuroendocrine tumour arising from the adrenal gland in 80-85% or extra-adrenal chromaffin cells of the autonomic neural ganglia in the remainder. The annual incidence of PPGL is 3-8 cases per million per year in the general population. Read More

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December 2021

[13-YEAR-OLD GIRL WITH PHEOCHROMOCYTOMA PRESENTING WITH SECONDARY NOCTURNAL ENURESIS].

Harefuah 2021 Dec;160(12):818-821

Department of Pediatrics, Shamir (Assaf Harofeh) Medical Center, affiliated with Sackler Faculty of Medicine, Tel- Aviv University.

Introduction: Pheochromocytoma is a rare, catecholamine secreting tumor arising from chromaffin cells of the adrenal medulla and it is responsible for 0.5-2% of pediatric hypertension cases. We hereby present a case of a 13-year-old girl with obesity, excessive hypertension, prediabetes and secondary nocturnal enuresis, who was eventually diagnosed with pheochromocytoma. Read More

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December 2021

Dutch Founder SDHB Exon 3 Deletion in Patients with Pheochromocytoma-Paraganglioma in South Africa.

Endocr Connect 2021 Dec 1. Epub 2021 Dec 1.

A Daly, Service d'Endocrinologie, CHU de Liège, Liège, 4000, Belgium.

Objective: Screening studies have established genetic risk profiles for diseases such as multiple endocrine neoplasia type 1 (MEN1) and pheochromocytoma-paraganglioma (PPGL). Founder effects play an important role in regional/national epidemiology of endocrine cancers, particularly PPGL. Founder effects in the Netherlands have been described for various diseases, some of which established themselves in South Africa due to Dutch emigration. Read More

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December 2021

Assessment of genomic alterations in non-syndromic von Hippel-Lindau: Insight from integrating somatic and germline next generation sequencing genomic data.

Data Brief 2021 Dec 1;39:107653. Epub 2021 Dec 1.

Department of Pathology, Brigham and Women's Hospital, Boston, MA, United States.

Von Hippel-Lindau (VHL) syndrome is a hereditary cancer genetic condition associated with inactivating pathogenic alterations in the tumor suppressor gene located at 3p (short arm of chromosome 3). Classic features of VHL include clear cell renal cell carcinoma, hemangioblastomas of the brain, spinal cord, and retina, pheochromocytoma, pancreatic cysts, and neuroendocrine tumors. Two sets of genomic information may be available from patients with VHL: the germline data showing the constitutional genetic profile and somatic profile obtained from patient tumor(s). Read More

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December 2021

Could we assess the functional status, of hormone secreting, or non-secreting of the adrenal masses regarding their Magnetic Resonance Imaging (MRI) characteristics?

Endocr Metab Immune Disord Drug Targets 2021 Dec 19. Epub 2021 Dec 19.

Cukurova University, Faculty of Medicine, Division of Endocrinology, Adana, Turkey.

Background: Diagnostic imaging techniques including magnetic resonance imaging (MRI) should also perform on all patients with incidentalomas. However, there is a limited study whether the quantitative measurements (signal intensity index, adrenal to spleen ratio) in MRI could predict the functional status of adrenal adenomas.

Material-method: Between 2015-2020; 404 patients (265 females, 139 males) with adrenal mass who were referred to the university hospital for further investigation were included. Read More

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December 2021

Germline Leu56Met Variant Is Likely Not Causative of Multiple Endocrine Neoplasia Type 2.

Front Endocrinol (Lausanne) 2021 1;12:764512. Epub 2021 Dec 1.

Center for Genomic Medicine, Copenhagen University Hospital, Copenhagen, Denmark.

Activating variants in the receptor tyrosine kinase arranged during ransfection (RET) cause multiple endocrine neoplasia type 2 (MEN 2), an autosomal dominantly inherited cancer-susceptibility syndrome. The variant c.166C>A, p. Read More

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December 2021

Clinical features and signaling effects of RET D631Y variant multiple endocrine neoplasia type 2 (MEN2).

Korean J Intern Med 2021 Dec 15. Epub 2021 Dec 15.

Department of Biomedicine and Health Science, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Background/aims: Germline mutations of the rearranged during transfection (RET) gene cause multiple endocrine neoplasia type 2 (MEN2). About 85% of RET mutations in MEN2 occur in codon Cys634. The RET D631Y mutation has recently been discovered, and we have studied its molecular expression and clinical consequences. Read More

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December 2021

Local-Regional Recurrence of Pheochromocytoma/Paraganglioma: Characteristics, Risk Factors and Outcomes.

Front Endocrinol (Lausanne) 2021 19;12:762548. Epub 2021 Oct 19.

Key Laboratory of Endocrinology, Department of Endocrinology, National Health Commission of the People's Republic of China, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.

Objective: To study the characteristics, risk factors, and outcomes of local-regional recurrence of pheochromocytoma and paraganglioma (PPGL).

Methods: Clinical data of 96 PPGL patients with local-regional recurrence and 112 patients without recurrence were retrospectively analyzed.

Results: Recurrent patients exhibited a median recurrence time of 6. Read More

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October 2021

rs779805 Von Hippel-Lindau Gene Polymorphism Induced/Related Polycythemia Entity, Clinical Features, Cancer Association, and Familiar Characteristics.

Pathol Oncol Res 2021 26;27:1609987. Epub 2021 Nov 26.

Division of Hematology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.

Increased red blood cell count may result from primary erythrocytosis (polycythemia vera), but it is often due to secondary causes with increased erythropoietin levels. Secondary erythrocytosis may also be congenital due to different gene mutations of hemoglobin, hemoglobin stabilization proteins, EPO receptors, or oxygen sensing pathways. Von Hippel- Lindau gene mutation causes altered tissue oxygen sensation in VHL disease, usually with normal hemoglobin. Read More

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November 2021

Imaging pheochromocytoma in small animals: preclinical models to improve diagnosis and treatment.

EJNMMI Res 2021 Dec 11;11(1):121. Epub 2021 Dec 11.

Institute for Diabetes and Cancer, Helmholtz Zentrum München, Ingolstaedter Landstrasse 1, 85764, Neuherberg, Germany.

Pheochromocytomas (PCCs) and paragangliomas (PGLs), together referred to as PPGLs, are rare chromaffin cell-derived tumors. They require timely diagnosis as this is the only way to achieve a cure through surgery and because of the potentially serious cardiovascular complications and sometimes life-threatening comorbidities that can occur if left untreated. The biochemical diagnosis of PPGLs has improved over the last decades, and the knowledge of the underlying genetics has dramatically increased. Read More

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December 2021

Chlorpromazine Efficiently Treats the Crisis of Pheochromocytoma: Four Case Reports and Literature Review.

Front Cardiovasc Med 2021 22;8:762371. Epub 2021 Nov 22.

Division of Cardiology, Department of Internal Medicine, Tongji Medical College, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, China.

Pheochromocytoma multisystem crisis (PMC) is a potentially lethal emergency due to catecholamine secretion. The condition manifests as severe hypertension to intractable cardiogenic shock and has a high mortality rate. This study explored the efficacy and safety of applying chlorpromazine on PMC patients. Read More

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November 2021

Cross-Talk Between Nitrosative Stress, Inflammation and Hypoxia-Inducible Factor in Patients with Adrenal Masses.

J Inflamm Res 2021 30;14:6317-6330. Epub 2021 Nov 30.

Department of Hygiene, Epidemiology and Ergonomics, Medical University of Bialystok, Bialystok, Poland.

Background: Adrenal masses are the most common of all human tumors. The role of nitrosative stress and inflammation in cancer development has already been demonstrated. However, it is not known whether they are involved in the pathogenesis of adrenal tumors. Read More

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November 2021

An open-label, single-arm, multi-center, phase II clinical trial of single-dose [I]meta-iodobenzylguanidine therapy for patients with refractory pheochromocytoma and paraganglioma.

Ann Nucl Med 2021 Dec 6. Epub 2021 Dec 6.

Department of Nuclear Medicine, Kanazawa University Hospital, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8641, Japan.

Objective: In this phase II study, we aimed to investigate the efficacy and safety of single-dose [I]meta-iodobenzylguanidine (I-mIBG) therapy in patients with refractory pheochromocytoma and paraganglioma (PPGL).

Patients And Methods: This study was designed as an open-label, single-arm, multi-center, phase II clinical trial. The enrolled patients were administered 7. Read More

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December 2021