23,152 results match your criteria Paraganglioma of the Bladder
Minerva Pediatr 2018 Dec 3. Epub 2018 Dec 3.
Department of Laboratory Medicine and Pathology, Azienda USL of Modena, Modena, Italy.
Background: Sparse metanephrines reference intervals in pediatric populations are available and different study designs and technologies/ assays used in these studies lead to hardly transferable data from a laboratory to another. The objective of the study was to update pediatric reference intervals of total fractionated metanephrines in spot urine samples, using a commercial extraction kit run on a specific high pressure liquid chromatograph coupled with an electrochemical detector.
Methods: 452 spot pediatric urinary samples previously submitted to urinalysis were consecutively included in the study with the exclusion of children's samples with diagnosis or clinical suspicion of paraganglioma/pheochromocytoma, kidney diseases and arterial hypertension. Read More
Laryngoscope 2018 Nov 28. Epub 2018 Nov 28.
Department of Otolaryngology, Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, People's Republic of China.
Objectives: The goal of this study was to detect and explore the mechanisms of the succinate dehydrogenase (SDH) complex subunit-related gene mutations in cases of multiple paraganglioma (PGL) in the head and neck.
Methods: In Beijing Tongren Hospital (Capital Medical University, Beijing, People's Republic of China) between January 2013 and February 2017, 23 cases of head and neck multiple PGL were evaluated by genetic sequencing. From these cases, four hereditary families and 10 cases with sporadic occurrences were found. Read More
World Neurosurg 2018 Nov 20. Epub 2018 Nov 20.
Department of Neurosciences, Operative Unit of Otorhinolaryngology, University of Padova, Via Giustiniani, 2, 35128, Padova, Italy.
Objective: Large intradural growth of tympano-jugular paragangliomas (TJ-PGs) into the cerebellopontine angle is an infrequent condition that can determine an extensive involvement of vessels and brainstem, representing a surgical challenge. The current classifications lack in accuracy for defining large intradural TJ-PGs and assessing their operability. This study aims at retrospectively reappraising the management of our large intradural TJ-PGs surgical series, discussing operability criteria, treatment strategies and resection techniques. Read More
Neuroimaging Clin N Am 2019 Feb;29(1):145-172
Department of Radiology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles Street, Boston, MA 02114, USA.
In their variety, temporal bone tumors mirror the complexity of the structure from which they arise. They include more familiar lesions, such as vestibular schwannomas and paragangliomas, and also rarer neoplasms, such as nonvestibular schwannomas, sarcomas, giant cell tumors, Schneiderian papillomas, and endolymphatic sac tumors. Diagnostic imaging is invaluable in evaluating such lesions because they are typically challenging to access surgically and monitor clinically. Read More
Medicine (Baltimore) 2018 Nov;97(47):e13268
Pathology Department, Central South University Xiangya School of Medicine Affiliated Haikou Hospital, Haikou, Hainan, PR China.
Rationale: Paraganglioma is a catecholamine-producing neuroendocrine tumor. Management of paraganglioma including its diagnosis is difficult, because it has no characteristic symptoms and many diseases can manifest as headache and high blood pressure. Herein, we report a rare case of paraganglioma of the abdomen with headache and initial normal blood pressure. Read More
Eur Spine J 2018 Nov 20. Epub 2018 Nov 20.
Division of Spine Surgery, Department of Orthopaedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, 576-104, India.
Purpose: The conventional posterior approach is mostly advocated for excision of sacral tumours below S2. We describe an operative technique of single-stage en bloc resection of sacral tumours, extending up to S1, through an extended posterior approach.
Method: Nine patients, who had undergone resection of sacral tumours, by the described technique formed the basis of this study. Read More
Am J Surg Pathol 2018 Nov 16. Epub 2018 Nov 16.
Department of Oncology-Pathology, Karolinska Institutet, Karolinska University Hospital, CCK.
Pheochromocytomas (PCCs) and abdominal paragangliomas (PGLs), collectively abbreviated PPGL, are believed to exhibit malignant potential-but only subsets of cases will display full-blown malignant properties. The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) algorithm is a proposed histologic system to detect potential for aggressive behavior, but little is known regarding the coupling to underlying molecular genetics. In this study, a total of 92 PPGLs, previously characterized for susceptibility gene status and mRNA expressional profiles, were histologically assessed using the PASS criteria. Read More
Br J Neurosurg 2018 Nov 19:1-4. Epub 2018 Nov 19.
b Department of Neuroradiology , San Bortolo Hospital , Vicenza , Italy.
Objective: Intra-arterial embolization of jugular paragangliomas is an established endovascular technique. Intratumoral embolization by direct puncture has been proposed, prior to surgical treatment or radiosurgery to reduce the risk of cranial nerve deficits.
Methods: We examined the technical aspects of two patients with jugular paragangliomas embolized with liquid embolic agent by direct puncture of the lesion, as sole treatment. Read More
Radiol Med 2018 Nov 14. Epub 2018 Nov 14.
Department of Radiology, IEO, European Institute of Oncology IRCCS, Milan, Italy.
The past decade has witnessed a growing role and increasing use of whole-body magnetic resonance imaging (WB-MRI). Driving these successes are developments in both hardware and software that have reduced overall examination times and significantly improved MR imaging quality. In addition, radiologists and clinicians have continued to find promising new applications of this innovative imaging technique that brings together morphologic and functional characterization of tissues. Read More
World J Clin Cases 2018 Nov;6(13):683-687
Department of Urology, The First Affiliated Hospital of Anhui Medical University, Hefei 230022, Anhui Province, China.
We present a case of adrenal CD in a 26-year-old female. The patient was referred to our hospital because of left flank pain for 1 wk. A computed tomography scan revealed a 4 cm × 3 cm well-defined mass, considered as a paraganglioma. Read More
Endocr Pathol 2018 Nov 12. Epub 2018 Nov 12.
Department of Pathology, University Medical Center/Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Succinate dehydrogenase (SDH) is an enzyme complex, composed of four protein subunits, that plays a role in both the citric acid cycle and the electron transport chain. The genes for SDHA, SDHB, SDHC, and SDHD are located in the nuclear DNA, and mutations in these genes have initially been described in paragangliomas (PGL) and pheochromocytomas (PCC), which are relatively rare tumors derived from the autonomic nervous system and the adrenal medulla, respectively. Patients with SDH mutations, that are almost exclusively in the germline, are frequently affected by multiple PGL and/or PCC. Read More
J Thorac Dis 2018 Sep;10(9):5318-5327
Division of Pathology, Department of Emergency and Organ Transplantation, University of Bari "Aldo Moro", Bari, Italy.
Background: Pulmonary and mediastinal paragangliomas are rare tumors that may have neuroendocrine activity or be non-functional, incidental, in asymptomatic patients, or causing mass effect symptoms. Although being low-grade tumors, they can display an aggressive behaviour, developing local infiltration and distant metastases. We report our experience with three endothoracic paragangliomas and a Literature review, to point out diagnostic difficulties and problems related to surgical treatment. Read More
J Nucl Med 2018 Nov 9. Epub 2018 Nov 9.
Radboud University Medical Center, Netherlands.
Static single timeframe F-FDG PET/CT is useful for the localization and functional characterization of pheochromocytoma and paraganglioma (PPGL). F-FDG-uptake varies between PPGLs with different genotypes and highest standardized uptake values (SUVs) are observed in case of succinate dehydrogenase (SDH) mutations, possibly related to enhanced aerobic glycolysis in tumor cells. The exact determinants of F-FDG accumulation in PPGL are unknown. Read More
Surgery 2018 Nov 5. Epub 2018 Nov 5.
Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Orthop Surg 2018 Nov 8;10(4):343-349. Epub 2018 Nov 8.
Department of Neurosurgery, Tianjin Medical University General Hospital, Tianjin, China.
This study investigated the surgical results of a single-stage posterolateral approach with arc incision, unilateral laminectomy, and costotransversectomy for the management of dumbbell tumors and paraspinal tumors of the thoracic spine. From January 2010 to March 2017, 14 patients with dumbbell tumors or paraspinal tumors of the thoracic spine who underwent resection with single-stage posterolateral approach were followed up and analyzed retrospectively. The operations were performed using a single-stage posterolateral approach with arc incision, unilateral laminectomy, and costotransversectomy without any instrumentation. Read More
Case Rep Endocrinol 2018 14;2018:6358485. Epub 2018 Oct 14.
Einstein Medical Center, 5501 Old York Rd., Philadelphia, PA 19141, USA.
Background: Pheochromocytoma/paraganglioma is a rare tumor from neuroendocrine cells. 1/3 of cases have germline mutations. Papillary thyroid carcinoma (PTC) is a common neoplasm from follicular cells of the thyroid. Read More
J Thorac Cardiovasc Surg 2018 Sep 29. Epub 2018 Sep 29.
Department of Cardiac Surgery, Peking Union Medical College Hospital, Beijing, China.
Objective: Cardiac paragangliomas are rare neuroendocrine tumors. Early surgical treatment improves clinical symptoms and prolongs survival. We review our experience in 17 patients who underwent surgical resection for functional cardiac paraganglioma. Read More
Transplant Proc 2018 Nov 20;50(9):2630-2635. Epub 2018 Mar 20.
Division of Transplant Surgery, Medical College of Wisconsin and Children's Hospital of Wisconsin, Milwaukee, Wisconsin. Electronic address:
Primary hepatic functional paraganglioma is a rare form of extra-adrenal catecholamine-secreting tumor. Definitive treatment of functioning paraganglioma is challenging because of the critical location of the tumor frequently in close proximity to vital structures and risk of excessive catecholamine release during operative manipulation. We report the multidisciplinary management approach for a case of unresectable primary hepatic functional paraganglioma with invasion into the hepatic veins and suprahepatic vena cava. Read More
J Thorac Cardiovasc Surg 2018 Oct 4. Epub 2018 Oct 4.
Department of Cardiothoracic Surgery, Houston Methodist Hospital, Houston, Tex. Electronic address:
Radiologia 2018 Nov 1. Epub 2018 Nov 1.
Servicio de Radiodiagnóstico, Hospital Clínico Universitario, Valencia, España.
Tumors of the carotid body are uncommon neoplasms that originate in the neuroectoderm. These tumors are slow growing but well vascularized and very destructive. Between 5% and 13% of carotid body tumors are malignant, behaving aggressively at the local level, invading the adjacent lymph nodes, and resulting in remote metastases. Read More
Endocrinol Diabetes Nutr 2018 Nov 1. Epub 2018 Nov 1.
Laboratorio de Medicina, Servicio de Bioquímica Clínica, Hospital Universitario Central de Asturias, Oviedo, España; Departamento de Bioquímica y Biología Molecular, Universidad de Oviedo, Oviedo, España.
Introduction: Pheochromocytoma and paraganglioma are uncommon tumors whose best known symptoms include high blood pressure, palpitations, headache, and sweating. Clinical identification is not easy, however, and requires biochemical tests that allow for early diagnosis, including measurement of metanephrines levels. The aim of this study was to assess the diagnostic performance of plasma free metanephrines (PMETs) and to verify the transferability of the reference values used. Read More
Int J Surg Case Rep 2018 Oct 25;53:99-101. Epub 2018 Oct 25.
Department of Otorhinolaryngology Head and Neck Surgery,National Academy of Medical Sciences, Bir Hospital, Kathmandu, Nepal. Electronic address:
Introduction: Carotid body tumors also known as parganglioma or chemodactomas are one of the rare tumors of head and neck which present as slow growing masses in the neck region. We present a case of 40 years female with painless slow growing mass over left side of her neck for 6 months. Diagnosis was made on basis of clinical history, examination and radiological findings. Read More
Indian J Nucl Med 2018 Oct-Dec;33(4):348-350
Department of Nuclear Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
Laryngeal paraganglioma is a rare neuroendocrine tumor arising from neural crest cells of larynx, contributing to 0.6% of the laryngeal tumors. Patients usually present with compressive symptoms such as hoarseness of voice. Read More
HNO 2018 Oct 30. Epub 2018 Oct 30.
Fachbereich Gefäßchirurgie, Klinik für Allgemein‑, Viszeral‑, Gefäß- und Transplantationschirurgie, Universitätsklinikum Magdeburg A.ö.R., Leipziger Str. 44, 39120, Magdeburg, Deutschland.
Background: Neck tumors are challenging regarding the diagnostic and therapeutic management particularly in cases of malignant growth near relevant vessels to achieve R0 resection status and the best prognosis.
Aim: The aim of this case report on a patient with a rare malignant tumor of the glomus caroticum (paraganglioma) is to present the successful outcome of a demanding interdisciplinary surgical approach (otorhinolaryngology and vascular surgery). Surgical re-intervention was necessary due to malignant tumor growth (detected in the histopathological investigation of the first specimen) including vascular resection using a hybrid graft for vascular reconstruction of the internal carotid artery near the skull base. Read More
Int J Neurosci 2018 Oct 30:1-6. Epub 2018 Oct 30.
a Department of Molecular Bases of Human Genetics , Institute of Molecular Genetics, Russian Academy of Sciences , Moscow , Russia.
Aim Of The Study: It was found that the mutations in the SDHD gene, encoding one of subunits of the succinate dehydrogenase complex, lead to the development of head and neck paraganglioma (HNPGL). We analyzed this gene in 91 patients with HNPGL from Russia.
Materials And Methods: DNA was isolated from the whole blood. Read More
Ann Clin Biochem 2018 Nov 14:4563218811865. Epub 2018 Nov 14.
Department of Diabetes and Endocrinology, Royal Hobart Hospital, School of Medicine, University of Tasmania, Tasmania, Australia.
Background: Patients with mutations of succinate dehydrogenase B (SDHB) and succinate dehydrogenase D (SDHD) are at high risk of paraganglioma necessitating surveillance. Chromogranin A has been proposed as a biochemical marker of paraganglioma. We sought to determine the diagnostic utility of chromogranin A in a population-based SDHx sample. Read More
Ann R Coll Surg Engl 2018 Oct 29:e1-e4. Epub 2018 Oct 29.
Post Graduate Institute of Medical Education and Research, Advanced Urology Centre Nehru Hospital, Pgimer , Chandigarh , India.
Retroperitoneal paragangliomas are rare tumours arising from neural crest cells located near or in the autonomic nervous system; right from the skull bass to the pelvic floor. Often called the great masqueraders, their diagnosis and treatment is often difficult and fraught with danger, considering the close proximity of these tumours to important retroperitoneal organs. A 29-year-old woman presented to the outpatient department with complaints of paroxysms of headache, sweating and palpitations for one year. Read More
Int J Surg Case Rep 2018 Oct 19;53:5-8. Epub 2018 Oct 19.
Department of Otorhinolaryngology-Head & Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Health Campus, Kubang Kerian, Kelantan, 16150, Malaysia. Electronic address:
Background: Paraganglioma of head and neck is a rare tumor and vagal schwannoma is even rarer. The majority of patients with vagal schwannoma presents with a lateral neck mass. Its management is delicate as the need to confirm the diagnosis by histopathology via a biopsy is contraindicated. Read More
J Clin Endocrinol Metab 2018 Oct 24. Epub 2018 Oct 24.
Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, University of Toronto.
Appl Immunohistochem Mol Morphol 2018 Oct 24. Epub 2018 Oct 24.
Institute of Pathology, University Hospital Heidelberg.
Objective: Recognition of neuroendocrine differentiation is important for tumor classification and treatment stratification. To detect and confirm neuroendocrine differentiation, a combination of morphology and immunohistochemistry is often required. In this regard, synaptophysin, chromogranin A, and CD56 are established immunohistochemical markers. Read More
Case Rep Surg 2018 26;2018:6292789. Epub 2018 Sep 26.
Division of Hepato-Pancreato-Biliary Surgery, Virginia Surgery Associates, Fairfax, VA, USA.
Introduction: Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. Read More
Case Rep Endocrinol 2018 25;2018:2091257. Epub 2018 Sep 25.
Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy.
Studies on genome-wide transcription patterns have shown that many genetic alterations implicated in pheochromocytoma-paraganglioma (P-PGL) syndromes cluster in a common cellular pathway leading to aberrant activation of molecular response to hypoxia in normoxic conditions (the pseudohypoxia hypothesis). Several cases of P-PGL have been reported in patients with cyanotic congenital heart disease (CCHD). Patients affected with CCHD have an increased likelihood of P-PGL compared to those affected with noncyanotic congenital heart disease. Read More
Endocr Connect 2018 Sep 1. Epub 2018 Sep 1.
F Beuschlein, LMU München, Medizinische Klinik IV, Munich, Germany.
Context: Pheochromocytomas and paragangliomas (PPGLs) are rare but potentially harmful tumors that can vary in their clinical presentation. Tumors may be found due to signs and symptoms, as part of a hereditary syndrome or following an imaging procedure.
Objective: To investigate potential differences in clinical presentation between PPGLs discovered by imaging (iPPGLs), symptomatic cases (sPPGLs) and those diagnosed during follow-up because of earlier disease/known hereditary mutations (fPPGL). Read More
Endocr Connect 2018 Dec;7(12):1217-1225
Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People's Republic of China.
Pheochromocytoma and paragangliomas (PCC/PGL) are neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and sympathetic/parasympathetic ganglia, respectively. Of clinical relevance regarding diagnosis is the highly variable presentation of symptoms in PCC/PGL patients. To date, the clear-cut correlations between the genotypes and phenotypes of PCC/PGL have not been entirely established. Read More
Cancer Manag Res 2018 11;10:4479-4489. Epub 2018 Oct 11.
Department of Urology, Subei People's Hospital of Jiangsu Province (Clinical Medical College, Yangzhou University), Yangzhou, Jiangsu, People's Republic of China,
Background: Bladder neuroendocrine carcinomas (BNECs) are relatively a rare type of tumor. The aim of this study was to examine the clinicopathological characteristics and predictors of survival outcomes of patients with BNECs based on the analysis of the national Surveillance, Epidemiology, and End Results (SEER) database.
Materials And Methods: Kaplan-Meier analysis with log-rank test was used for survival comparisons. Read More
F1000Res 2018 20;7. Epub 2018 Sep 20.
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare chromaffin cell tumors (PPGLs) that at times raise significant challenges in clinical recognition, diagnosis, and therapy and when undiagnosed could associate with severe morbidity. Recent discoveries in PPGL genetics propelled our understanding in the pathophysiology of tumorigenesis and allowed the application of functional classification of pathogenetically distinct groups of PPGLs. This also resulted in a qualitative change in our approach to clinical assessment, diagnosis, and therapy of different subgroups of PPGLs. Read More
Surgery 2018 Oct 16. Epub 2018 Oct 16.
University of Tennessee Health Science Center, Department of Surgery, Division of Surgical Oncology, Memphis TN. Electronic address:
Background: Multifocal pheochromocytoma/paraganglioma presenting at an early age is commonly associated with a hereditary syndrome.
Case Report: A 29-year-old woman was referred for evaluation of multifocal pheochromocytoma/paraganglioma. Interestingly, her family history did not include pheochromocytoma/paraganglioma, and comprehensive genetic testing for the well-documented pheochromocytoma/paraganglioma susceptibility genes was negative. Read More
Eur J Cardiothorac Surg 2018 Oct 18. Epub 2018 Oct 18.
Department of Cardiovascular and Thoracic Surgery, Northwell Health, Lenox Hill Hospital, New York, NY, USA.
Aortopulmonary paragangliomas are rare, neural crest-derived tumours that arise in the aortopulmonary window and present a significant surgical challenge due to their proximity to major vessels. In the relatively few cases reported in the study, thoracotomy and video-assisted thorascopic surgery (VATS) approaches have been utilized for the treatment of such masses. In this report, we describe the application of a totally endoscopic robotic-assisted VATS approach for the excision of an aortopulmonary paraganglioma. Read More
J Ultrason 2018 ;18(73):96-102
Department of Interventional Radiology and Neuroradiology, Medical University of Lublin, Lublin, Poland.
Aim: The purpose of this study was the evaluation of the sonographic appearance of neck tumors and determining the features useful in differential diagnosis.
Material And Method: The studied group consisted of 57 patients: 16 patients with carotid body tumors, 9 patients with neurogenic tumors, 8 patients with venous anomalies, 12 patients with neck cysts, 6 patients with lipomas, 5 patients with extracranial carotid artery aneurysms and 1 with a laryngocele.
Results: All carotid paragangliomas were located within the carotid bifurcation and demonstrated rich low-resistance vascular flow, with higher maximum velocity and lower flow resistance parameters registered in the ipsilateral external carotid artery. Read More
Clin Nucl Med 2018 Oct 15. Epub 2018 Oct 15.
Division of Diagnostic Imaging, Department of Nuclear Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX.
A 65-year-old man with a history of hypertension and nephrolithiasis presented with flank pain. Urographic CT demonstrated a 1.7-cm nodule arising from the bladder wall. Read More
BMJ Case Rep 2018 Oct 14;2018. Epub 2018 Oct 14.
Neurological Imaging and Interventional Sevice of Western Australia, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.
Curr Probl Diagn Radiol 2018 Sep 11. Epub 2018 Sep 11.
Department of Radiology, Allegheny Health Network, Pittsburgh, PA.
Paragangliomas are extra-adrenal tumors that are derived from neuroendocrine chromaffin cells. The rare disease has a variable presentation depending upon its anatomic location and functionality. We describe the case of a 56-year-old female patient who had an incidental mass found on imaging. Read More
Medicine (Baltimore) 2018 Oct;97(41):e12586
Department of Pathology, China-Japan Union Hospital of Jilin University.
Introduction: This study aims to investigate the pathological features of a patient with paraganglioma in the lumbar spinal canal.
Case Presentation: The patient was 36 years old male with electrical pain in the waist and buttock which occurred intermittently, and was not relieved under oral use of analgesics. Immunohistochemical staining was performed to diagnose the disease. Read More
J Am Vet Med Assoc 2018 Nov;253(9):1169-1176
CASE DESCRIPTION A 7-year-old female domestic shorthair cat was referred for evaluation of azotemia and unilateral hydronephrosis. CLINICAL FINDINGS Abdominal ultrasonography revealed right-sided hydronephrosis and dilation of the proximal aspect of the ureter; the left kidney was small with irregular margins. A highly vascular, irregular retroperitoneal mass, not clearly associated with the ureteral obstruction, was also visualized. Read More
Chirurg 2018 Oct 10. Epub 2018 Oct 10.
Medizinische Universitätsklinik, Universitätsklinikum Freiburg, Freiburg, Deutschland.
Chromaffin tumors, e.g. pheochromocytomas and paragangliomas are caused by germline mutations of several susceptibility genes in 30-40% of the patients. Read More
Clin Endocrinol (Oxf) 2018 Oct 10. Epub 2018 Oct 10.
Department of Endocrinology, St Bartholomew's Hospital, Barts Health NHS Trust, London, UK.
Objective: With the discovery that familial phaeochromocytoma and paraganglioma syndrome can be caused by mutations in each subunit of the succinate dehydrogenase enzyme (SDH), has come the recognition that mutations in the individual subunits have their own distinct natural histories. Increased genetic screening is leading to the identification of increasing numbers of, mostly asymptomatic, gene mutation carriers and the implementation of screening strategies for these individuals. Yet there is, to date, no international consensus regarding screening strategies for asymptomatic carriers. Read More
Indian J Pathol Microbiol 2018 Oct-Dec;61(4):580-582
Institute of Liver Gastroenterology and Pancreatico-Biliary Sciences, Sir Ganga Ram Hospital, Rajender Nagar, New Delhi, India.
Duodenal gangliocytic paragangliomas are rare neoplasms often arising in the duodenum in close proximity to the ampulla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastases even more so. Although a standardized treatment has not been determined, a margin-free tumor resection seems to be the best treatment modality. Read More
Clin Cancer Res 2018 Oct 9. Epub 2018 Oct 9.
INSERM, UMR970, Paris-Cardiovascular Research Center, Equipe Labellisée par la Ligue contre le Cancer, Paris, France.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors. Whereas most PPGLs are benign, up to 20% may become metastatic with - and -mutated tumors showing the higher risk. We aimed at determining the contribution of immortalization mechanisms to metastatic progression. Read More
Pediatr Dev Pathol 2018 Oct 9:1093526618805354. Epub 2018 Oct 9.
2 Department of Pathology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.
Gastrointestinal stromal tumors (GISTs) are rare in children. Succinate dehydrogenase (SDH)-deficient GISTs are wild type and lack KIT proto-oncogene receptor tyrosine kinase and platelet-derived growth factor receptor A ( KIT or PDGFRA) mutations. These tumors result from germline SDH mutations, somatic SDH mutations, or SDH epimutants. Read More