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Clinical Radiology Dec 2022Paragangliomas are neuroendocrine tumours of the sympathetic and parasympathetic nervous systems originating from neural crest cells. Less than 1% of paragangliomas in... (Review)
Review
Paragangliomas are neuroendocrine tumours of the sympathetic and parasympathetic nervous systems originating from neural crest cells. Less than 1% of paragangliomas in the heart originate from intrinsic cardiac ganglia cells in the posterior wall of the atria, atrioventricular groove, and along the root of the great vessels. We describe the tumour characteristics, patient demographics, presentation, means of diagnosis, pathology correlation, management, and outcome in 11 patients with intrapericardial paragangliomas. To the authors' knowledge, this is the largest case series reported, with emphasis on multimodality imaging findings.
Topics: Humans; Paraganglioma; Heart Atria; Neuroendocrine Tumors
PubMed: 36175258
DOI: 10.1016/j.crad.2022.08.131 -
Otolaryngology--head and Neck Surgery :... Apr 2016Head and neck paragangliomas are a group of slow-growing hypervascular tumors associated with the paraganglion system. The approach to evaluate and treat these lesions... (Review)
Review
OBJECTIVE
Head and neck paragangliomas are a group of slow-growing hypervascular tumors associated with the paraganglion system. The approach to evaluate and treat these lesions has evolved over the last 2 decades. While radical surgery had been the traditional approach, improvements in diagnostic imaging as well as radiation therapy techniques have led to an emphasis on observation and nonsurgical therapy in many patients. This article reviews the contemporary approach to the workup and management of head and neck paragangliomas.
DATA SOURCE
Articles were identified from PubMed.
REVIEW METHODS
PubMed searches with the following keywords were performed: carotid body paraganglioma management, vagal paraganglioma management, jugulotympanic paraganglioma management, imaging of head and neck paragangliomas, head and neck paraganglioma embolization, paraganglioma radiation, head and neck paraganglioma management, observation of head and neck paragangliomas, bilateral carotid body paragangliomas, and genetics of paragangliomas. Review and original research articles available in the English language and published during or after 2009 were selected on the basis of their clinical relevance and scientific strength. Certain articles published prior to 2009 were also included if they provided background information that was relevant.
CONCLUSIONS/IMPLICATIONS FOR PRACTICE
Workup and treatment of head and neck paragangliomas are changing. With more now known regarding the longitudinal behavior of these tumors, observation and nonsurgical therapy are indicated in many instances. For patients where surgery is the most appropriate option, improved diagnostic and perioperative techniques are allowing patients to tolerate resection, often with reduced morbidity.
Topics: Diagnostic Imaging; Disease Management; Head and Neck Neoplasms; Humans; Paraganglioma
PubMed: 26861230
DOI: 10.1177/0194599815627667 -
Cell and Tissue Research May 2018Pheochromocytomas and their extra-adrenal counterpart paragangliomas (PGLs; together called PPGLs), belong to the family of neural crest-derived tumors. Given the... (Review)
Review
Pheochromocytomas and their extra-adrenal counterpart paragangliomas (PGLs; together called PPGLs), belong to the family of neural crest-derived tumors. Given the overexpression of a wide variety of specific targets in PPGLs, it seems that these tumors are optimally suited to be imaged by specific radiopharmaceuticals. Thus, theranostics approaches with somatostatin agonists and antagonists are rapidly evolving in the setting of these tumors and may be considered as the next step in the therapeutic arsenal of metastatic PPGLs.
Topics: Adrenal Gland Neoplasms; Humans; Molecular Imaging; Paraganglioma; Pheochromocytoma; Radiopharmaceuticals; Theranostic Nanomedicine
PubMed: 29450723
DOI: 10.1007/s00441-018-2791-4 -
Seminars in Nuclear Medicine May 2016Pheochromocytomas and paragangliomas are rare tumors arising from chromaffin cells. Available therapeutic modalities consist of chemotherapy, tyrosine kinase inhibitors,... (Review)
Review
Pheochromocytomas and paragangliomas are rare tumors arising from chromaffin cells. Available therapeutic modalities consist of chemotherapy, tyrosine kinase inhibitors, and I-131 metaiodobenzylguanidine (MIBG). I-131 MIBG is taken up via specific receptors and localizes into many but not all pheochromocytomas and paragangliomas. Because these tumors are rare, most therapy studies are retrospective presentations of clinical experience. Numerous retrospective studies and a few prospective studies have shown favorable responses in this disease, including symptomatic, biochemical, and objective responses. In this report, we review the experience of using I-131 MIBG therapy for targeting pheochromocytoma and paragangliomas.
Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Humans; Paraganglioma; Pheochromocytoma
PubMed: 27067501
DOI: 10.1053/j.semnuclmed.2016.01.011 -
Clinical Nuclear Medicine Sep 2019Paratesticular paragangliomas are a rare occurrence. We present the case of a 43-year-old man who presented with paresthesia and paraparesis and was found to have...
Paratesticular paragangliomas are a rare occurrence. We present the case of a 43-year-old man who presented with paresthesia and paraparesis and was found to have pathologic fracture involving D1 vertebra as a manifestation of metastasis from a nonsecretory right paratesticular paraganglioma.
Topics: Adult; Humans; Magnetic Resonance Imaging; Male; Neoplasm Metastasis; Paraganglioma; Testicular Neoplasms
PubMed: 31348093
DOI: 10.1097/RLU.0000000000002696 -
Endocrine Practice : Official Journal... Jan 2018Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors derived from adrenal or extra-adrenal locations, respectively. Upon suspicion of PPGL, specific... (Review)
Review
OBJECTIVE
Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors derived from adrenal or extra-adrenal locations, respectively. Upon suspicion of PPGL, specific metabolomic, molecular, biochemical, imaging, and histopathologic studies are performed to prove, localize, treat, and monitor disease progression. Improved diagnostic tools allow physicians to accurately diagnose PPGL, even in patients presenting with small (<1 cm) or biochemically silent tumors, which previously delayed proper detection and treatment.
METHODS
This review outlines the most updated approach to PPGL patients and presents a new diagnostic protocol for physicians to increase earlier tumor identification and accurately assess metastatic behavior.
CONCLUSION
We present the most recent advances in genetics, epigenetics, metabolomics, biochemical, and imaging diagnoses of this rare tumor to properly assess disease, decide treatment options, and manage follow-up. We also elaborate on new therapeutic perspectives in these very rare neoplastic entities.
ABBREVIATIONS
ATRX = ATRX chromatin remodeler; ccRCC = clear cell renal cell carcinoma; c-MYC = MYC proto oncognene; CT = computed tomography; DOTATATE = DOTA-octreotate; EGLN1/2 = egl-9 family hypoxia inducible factor 1/2; EGLN2/PHD1 = egl-9 family hypoxia inducible factor 2; EPAS1/HIF2A = endothelial PAS domain protein 2/hypoxia-inducible factor 2α; ERK = extracellular signal-regulated kinase; HIFs = hypoxia-inducible factors; HIF-α = hypoxia-inducible factor alpha; HNPGLs = head and neck paragangliomas; Lu-DOTATATE = lutetium octreotate; MAX = myc-associated factor X; MDH2 = malate dehydrogenase; MIBG = metaiodobenzylguanidine; MN = metanephrine; MRI = magnetic resonance imaging; mTOR = mammalian target of rapamycin; NETs = neuroendocrine tumors; NF1 = neurofibromin 1; NMN = normetanephrine; PHD = prolyl hydroxylase domain protein; PI3K = phosphoinositide 3-kinase; PPGLs = pheochromocytoma and paragangliomas; PRRT = peptide receptor radionuclide therapy; Pvhl = von Hippel-Lindau protein; RAS = rat sarcoma oncogene; RET = rearranged during transfection proto-oncogene; SDH = succinate dehydrogenase; SDHA, -B, -C, -D = succinate dehydrogenase subunits A, B, C, D; SDHAF2 = succinate dehydrogenase complex assembly factor 2; SDHB, C, D = succinate dehydrogenase subunits B, C, D; SDHx = succinate dehydrogenase subunits; SSTRs = somatostatin receptors; VHL = von Hippel-Lindau.
Topics: Adrenal Gland Neoplasms; Humans; Metabolomics; Paraganglioma, Extra-Adrenal; Pheochromocytoma; Proto-Oncogene Mas
PubMed: 29144820
DOI: 10.4158/EP-2017-0057 -
The Journal of Clinical Endocrinology... May 2018Pheochromocytomas (PCCs) are tumors that are derived from the chromaffin cells of the adrenal medulla. Extra-adrenal PCCs called paragangliomas (PGLs) are derived from... (Review)
Review
CONTEXT
Pheochromocytomas (PCCs) are tumors that are derived from the chromaffin cells of the adrenal medulla. Extra-adrenal PCCs called paragangliomas (PGLs) are derived from the sympathetic and parasympathetic chain ganglia. PCCs secrete catecholamines, which cause hypertension and have adverse cardiovascular consequences as a result of catecholamine excess. PGLs may or may not produce catecholamines depending on their genetic type and anatomical location. The most worrisome aspect of these tumors is their ability to become aggressive and metastasize; there are no known cures for metastasized PGLs.
METHODS
Original articles and reviews indexed in PubMed were identified by querying with specific PCC/PGL- and Krebs cycle pathway-related terms. Additional references were selected through the in-depth analysis of the relevant publications.
RESULTS
We primarily discuss Krebs cycle mutations that can be instrumental in helping investigators identify key biological pathways and molecules that may serve as biomarkers of or treatment targets for PCC/PGL.
CONCLUSION
The mainstay of treatment of patients with PCC/PGLs is surgical. However, the tide may be turning with the discovery of new genes associated with PCC/PGLs that may shed light on oncometabolites used by these tumors.
Topics: Adrenal Gland Neoplasms; Cell Transformation, Neoplastic; Energy Metabolism; Humans; Paraganglioma; Pheochromocytoma
PubMed: 29409060
DOI: 10.1210/jc.2017-01991 -
Cancer Control : Journal of the Moffitt... Jul 2016Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of... (Review)
Review
BACKGROUND
Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of the neural crest. Frequently, these tumors are associated with the vagus or tympanic plexus nerve or the carotid artery, or jugular bulb. Their clinical presentation can vary across a wide spectrum of signs and symptoms.
METHODS
We reviewed and compared standard treatment approaches for paragangliomas of the head and neck.
RESULTS
In general, surgery is the first-line choice of therapy for carotid body tumors, whereas radiotherapy is the first-line option for jugular and vagal paragangliomas.
CONCLUSIONS
Because of the complexity of clinical scenarios and treatment options for paragangliomas, a multidisciplinary algorithmic approach should be used for treating paragangliomas. The approach should emphasize single-modality treatment that yields excellent rates of tumor control, low rates of severe, iatrogenic morbidity, and the preservation of long-term function in this patient population.
Topics: Female; Head and Neck Neoplasms; Humans; Male; Paraganglioma
PubMed: 27556663
DOI: 10.1177/107327481602300306 -
Hypertension in Pheochromocytoma and Paraganglioma: Evaluation and Management in Pediatric Patients.Current Hypertension Reports May 2021The rare catecholamine-secreting tumors, pheochromocytomas and paragangliomas (PPGL), account for a minority of cases of secondary hypertension in pediatrics. As such,... (Review)
Review
PURPOSE OF REVIEW
The rare catecholamine-secreting tumors, pheochromocytomas and paragangliomas (PPGL), account for a minority of cases of secondary hypertension in pediatrics. As such, perioperative blood pressure (BP) management in pediatric patients presents a distinct challenge. This review will expand the practitioner's knowledge of antihypertensive treatment options for the pediatric patient with PPGL with a focus on literature in the past several years.
RECENT FINDINGS
There continue to be only small case series and single-center experiences to provide guidelines regarding BP management. While phenoxybenzamine has been more routinely used, selective α-blockers, such as doxazosin, as well as calcium channel blockers, have also been utilized with success in pediatric patients. While the concept of obligatory α-adrenergic blockade for adult patients has been recently challenged, international guidelines and current practice patterns among pediatric clinicians continue to support preoperative α-adrenergic blockade to ensure the best possible patient outcomes. Selective α-blockers and calcium channel blockers are becoming more commonly used given the high cost, limited availability, and undesirable side effect profile of phenoxybenzamine.
Topics: Adrenal Gland Neoplasms; Adrenergic alpha-Antagonists; Adult; Child; Humans; Hypertension; Paraganglioma; Pediatrics; Pheochromocytoma
PubMed: 34041599
DOI: 10.1007/s11906-021-01150-9 -
Endocrine Regulations Jan 2018Pheochromocytomas and paragangliomas (PPGLs) are tumors arising from the adrenal medulla and sympathetic/parasympathetic paraganglia, respectively. According to Th e... (Review)
Review
Pheochromocytomas and paragangliomas (PPGLs) are tumors arising from the adrenal medulla and sympathetic/parasympathetic paraganglia, respectively. According to Th e Cancer Genome Atlas (TCGA), approximately 40% of PPGLs are due to germ line mutations in one of 16 susceptibility genes, and a further 30% are due to somatic alterations in at least seven main genes (VHL, EPAS1, CSDE1, MAX, HRAS, NF1, RET, and possibly KIF1B). Th e diagnosis of malignant PPGL was straight forward in most cases as it was defined as presence of PPGL in non-chromaffin tissues. Accordingly, there is an extreme need for new diagnostic marker(s) to identify tumors with malignant prospective. Th e aim of this study was to review all suggested genetic and epigenetic alterations that are remarkably different between benign and malignant PPGLs. It seems that more than two genetic mutation clusters in PPGLs and other genetic and methylation biomarkers could be targeted for malignancy discrimination in different studies.
Topics: Adrenal Gland Neoplasms; Biomarkers, Tumor; Epigenesis, Genetic; Humans; Paraganglioma; Pheochromocytoma
PubMed: 29453919
DOI: 10.2478/enr-2018-0006