pheochromocytoma

Summary

Summary: A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298)

Top Publications

  1. ncbi Pheochromocytoma as an endocrine emergency
    Frederieke M Brouwers
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, University Medical Center, St Radboud Nijmegen, Nijmegen, The Netherlands
    Rev Endocr Metab Disord 4:121-8. 2003
  2. ncbi Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma
    Iñaki Comino-Méndez
    Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre CNIO, Madrid, Spain
    Nat Genet 43:663-7. 2011
  3. ncbi Adrenomedullin: a novel hypotensive peptide isolated from human pheochromocytoma
    K Kitamura
    Department of First Internal Medicine, Miyazaki Medical College, Japan
    Biochem Biophys Res Commun 192:553-60. 1993
  4. ncbi Germ-line mutations in nonsyndromic pheochromocytoma
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    N Engl J Med 346:1459-66. 2002
  5. ncbi Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A
    L M Mulligan
    Department of Pathology, University of Cambridge, UK
    Nature 363:458-60. 1993
  6. ncbi An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis
    Francien H van Nederveen
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC, University Medical Center, Rotterdam, Netherlands
    Lancet Oncol 10:764-71. 2009
  7. ncbi Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    JAMA 292:943-51. 2004
  8. ncbi Research resource: Transcriptional profiling reveals different pseudohypoxic signatures in SDHB and VHL-related pheochromocytomas
    Elena López-Jiménez
    Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre, Madrid, Spain
    Mol Endocrinol 24:2382-91. 2010
  9. ncbi An update on the genetics of paraganglioma, pheochromocytoma, and associated hereditary syndromes
    A P Gimenez-Roqueplo
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 rue Leblanc, Paris, France
    Horm Metab Res 44:328-33. 2012
  10. ncbi Somatic NF1 inactivation is a frequent event in sporadic pheochromocytoma
    Nelly Burnichon
    INSERM, UMR970, Paris Cardiovascular Research Center, Paris, France
    Hum Mol Genet 21:5397-405. 2012

Detail Information

Publications356 found, 100 shown here

  1. ncbi Pheochromocytoma as an endocrine emergency
    Frederieke M Brouwers
    Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, University Medical Center, St Radboud Nijmegen, Nijmegen, The Netherlands
    Rev Endocr Metab Disord 4:121-8. 2003
  2. ncbi Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma
    Iñaki Comino-Méndez
    Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre CNIO, Madrid, Spain
    Nat Genet 43:663-7. 2011
    Hereditary pheochromocytoma (PCC) is often caused by germline mutations in one of nine susceptibility genes described to date, but there are familial cases without mutations in these known genes...
  3. ncbi Adrenomedullin: a novel hypotensive peptide isolated from human pheochromocytoma
    K Kitamura
    Department of First Internal Medicine, Miyazaki Medical College, Japan
    Biochem Biophys Res Commun 192:553-60. 1993
    A novel hypotensive peptide was discovered in human pheochromocytoma by monitoring the elevating activity of platelet cAMP...
  4. ncbi Germ-line mutations in nonsyndromic pheochromocytoma
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    N Engl J Med 346:1459-66. 2002
    The group of susceptibility genes for pheochromocytoma that included the proto-oncogene RET (associated with multiple endocrine neoplasia type 2 [MEN-2]) and the tumor-suppressor gene VHL (associated with von Hippel-Lindau disease) now ..
  5. ncbi Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A
    L M Mulligan
    Department of Pathology, University of Cambridge, UK
    Nature 363:458-60. 1993
    ..Further, 19 of these 20 mutations affect the same conserved cysteine residue at the boundary of the RET extracellular and transmembrane domains...
  6. ncbi An immunohistochemical procedure to detect patients with paraganglioma and phaeochromocytoma with germline SDHB, SDHC, or SDHD gene mutations: a retrospective and prospective analysis
    Francien H van Nederveen
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC, University Medical Center, Rotterdam, Netherlands
    Lancet Oncol 10:764-71. 2009
    ..We investigated whether SDHB immunohistochemistry could effectively discriminate between SDH-related and non-SDH-related phaeochromocytomas and paragangliomas in large retrospective and prospective tumour series...
  7. ncbi Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations
    Hartmut P H Neumann
    Department of Nephrology and Hypertension, Albert Ludwigs University, Freiburg, Germany
    JAMA 292:943-51. 2004
    ..In both syndromes, pheochromocytomas as well as head and neck paragangliomas occur; however, details for individual risks and other clinical characteristics are unknown...
  8. ncbi Research resource: Transcriptional profiling reveals different pseudohypoxic signatures in SDHB and VHL-related pheochromocytomas
    Elena López-Jiménez
    Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre, Madrid, Spain
    Mol Endocrinol 24:2382-91. 2010
    The six major genes involved in hereditary susceptibility for pheochromocytoma (PCC)/paraganglioma (PGL) (RET, VHL, NF1, SDHB, SDHC, and SDHD) have been recently integrated into the same neuronal apoptotic pathway where mutations in any ..
  9. ncbi An update on the genetics of paraganglioma, pheochromocytoma, and associated hereditary syndromes
    A P Gimenez-Roqueplo
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 rue Leblanc, Paris, France
    Horm Metab Res 44:328-33. 2012
    ..Most familial cases of pheochromocytoma and/or paraganglioma and 10-20% sporadic cases carry germline mutations in VHL, RET, NF1, SDHA, SDHB, SDHC, ..
  10. ncbi Somatic NF1 inactivation is a frequent event in sporadic pheochromocytoma
    Nelly Burnichon
    INSERM, UMR970, Paris Cardiovascular Research Center, Paris, France
    Hum Mol Genet 21:5397-405. 2012
    ..SDHA, SDHAF2, SDHB, SDHC, SDHD, MAX, TMEM127, NF1 or VHL genes are identified in about 30% of patients with pheochromocytoma or paraganglioma and somatic mutations in RET, VHL or MAX genes are reported in 17% of sporadic tumors...
  11. ncbi Rationale for anti-angiogenic therapy in pheochromocytoma and paraganglioma
    Judith Favier
    INSERM, UMR970, Paris Cardiovascular Research Center, Paris, France
    Endocr Pathol 23:34-42. 2012
    ..Our results suggest that there is a strong rationale for anti-VEGF-based therapeutic strategies in malignant pheochromocytomas and paragangliomas, in particular in those associated with mutations in the SDHB gene...
  12. ncbi Integrative genomic analysis reveals somatic mutations in pheochromocytoma and paraganglioma
    Nelly Burnichon
    INSERM, UMR970, Paris Cardiovascular Research Center, F 75015 Paris, France
    Hum Mol Genet 20:3974-85. 2011
    ..Although genome-wide expression studies have revealed some of the mechanisms likely to be involved in pheochromocytoma/paraganglioma tumorigenesis, the complete molecular distinction of all subtypes of hereditary tumors has not ..
  13. ncbi Biochemical diagnosis of pheochromocytoma: which test is best?
    Jacques W M Lenders
    Department of Internal Medicine, St Radboud University Medical Center, Geert Grooteplein Zuid 8, PO Box 9101, 6500 HB, Nijmegen, The Netherlands
    JAMA 287:1427-34. 2002
    Diagnosis of pheochromocytoma depends on biochemical evidence of catecholamine production by the tumor. However, the best test to establish the diagnosis has not been determined.
  14. pmc Characterization of an animal model of aggressive metastatic pheochromocytoma linked to a specific gene signature
    Lucia Martiniova
    Section on Medical Neuroendocrinology, Reproductive and Adult Endocrinology Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development NIH, 10 Center Drive MSC 1109, Bethesda, MD 20892 1109, USA
    Clin Exp Metastasis 26:239-50. 2009
    Pheochromocytomas are chromaffin cell-derived neuroendocrine tumors. There is presently no cure for metastatic pheochromocytoma and no reliable way to distinguish malignant from benign tumors before the development of metastases...
  15. ncbi High penetrance of pheochromocytoma associated with the novel C634Y/Y791F double germline mutation in the RET protooncogene
    Rodrigo A Toledo
    Faculdade de Medicina da Universidade de Sao Paulo, Avenida Dr Arnaldo, 455, 5 degrees andar, Cerqueira Cesar, 012406 903, Sao Paulo, Brazil
    J Clin Endocrinol Metab 95:1318-27. 2010
    ..Previous studies have shown that double RET mutations may be associated with unusual multiple endocrine neoplasia type 2 (MEN 2) phenotypes...
  16. ncbi Clinical predictors and algorithm for the genetic diagnosis of pheochromocytoma patients
    Zoran Erlic
    Department of Nephrology, Section of Preventive Medicine, Albert Ludwigs University, Hugstetter Strasse 55, Freiburg, Germany
    Clin Cancer Res 15:6378-85. 2009
    Six pheochromocytoma susceptibility genes causing distinct syndromes have been identified; approximately one of three of all pheochromocytoma patients carry a predisposing germline mutation...
  17. ncbi The diagnosis and management of malignant phaeochromocytoma and paraganglioma
    Alexandra Chrisoulidou
    Department of Endocrinology and Endocrine Oncology, Theagenion Hospital, Thessaloniki, Greece
    Endocr Relat Cancer 14:569-85. 2007
    ..Ongoing microarray studies may provide novel intracellular pathways of importance for proliferation/cell cycle control, and lead to the development of novel pharmacological agents...
  18. ncbi Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators
    Montserrat Ayala-Ramirez
    Department of Endocrine Neoplasia and Hormonal Disorders, Unit 1461, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, Houston, Texas 77030, USA
    J Clin Endocrinol Metab 96:717-25. 2011
    ..Pheochromocytomas and sympathetic paragangliomas are rare neuroendocrine tumors for which no precise histological or molecular markers have been identified to differentiate benign from malignant tumors...
  19. ncbi Animal models of pheochromocytoma
    A S Tischler
    Department of Pathology, Tufts University School of Medicine, Tufts New England Medical Center, Boston, MA 02111, USA
    Histol Histopathol 19:883-95. 2004
    ..Concurrence of pheochromocytoma with other tumors sometimes parallels these human syndromes in rats, bovines, horses and dogs but a shared ..
  20. ncbi Pheochromocytoma cell lines from heterozygous neurofibromatosis knockout mice
    J F Powers
    Department of Pathology, Box 802, New England Medical Center and Tufts University School of Medicine, 750 Washington Street, Boston, MA 02111, USA
    Cell Tissue Res 302:309-20. 2000
    ..Nf1 encodes a ras-GTPase-activating protein, neurofibromin, and mouse pheochromocytoma (MPC) cells in primary cultures typically show extensive spontaneous neuronal differentiation that may result ..
  21. ncbi Management of the clinically inapparent adrenal mass ("incidentaloma")
    Melvin M Grumbach
    University of California, San Francisco, San Francisco, California, USA
    Ann Intern Med 138:424-9. 2003
    ..renin activity ratio for patients with hypertension; and surgery for patients with biochemical evidence of pheochromocytoma, patients with tumors greater than 6 cm, and patients with tumors greater than 4 cm who also meet other ..
  22. ncbi Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes
    Anthony J Gill
    Department of Anatomical Pathology, Royal North Shore Hospital, Sydney 2065, Australia
    Hum Pathol 41:805-14. 2010
    ..Completely absent staining is more commonly found with SDHB mutation, whereas weak diffuse staining often occurs with SDHD mutation...
  23. ncbi Recommended evaluation of adrenal incidentalomas is costly, has high false-positive rates and confers a risk of fatal cancer that is similar to the risk of the adrenal lesion becoming malignant; time for a rethink?
    T J Cawood
    Department of Endocrinology, Christchurch Hospital, Private Bag 4710, Christchurch, New Zealand
    Eur J Endocrinol 161:513-27. 2009
    ..Surgical series, histopathological series and oncological series were reviewed and most were excluded...
  24. ncbi SDH mutations establish a hypermethylator phenotype in paraganglioma
    Eric Letouze
    Programme Cartes d Identité des Tumeurs, Ligue Nationale Contre le Cancer, 75013 Paris, France
    Cancer Cell 23:739-52. 2013
    ..Finally, inactivating FH mutations were identified in the only hypermethylated tumor without SDHx mutations. These findings emphasize the interplay between the Krebs cycle, epigenomic changes, and cancer...
  25. ncbi Genetics of pheochromocytoma and paraganglioma in Spanish patients
    Alberto Cascon
    Hereditary Endocrine Cancer Group, Institute de Salud Carlos III Center for Biomedical Research on Rare Diseases, Madrid, Spain
    J Clin Endocrinol Metab 94:1701-5. 2009
    The presence of familial history in pheochromocytoma/paraganglioma patients, including syndromic antecedents, leads in the majority of cases to a positive genetic testing for mutations in one of the major susceptibility genes described so ..
  26. ncbi Inhibition of autophagy enhances sunitinib-induced cytotoxicity in rat pheochromocytoma PC12 cells
    Tatsuhiko Ikeda
    Department of Endocrine Surgery, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Japan
    J Pharmacol Sci 121:67-73. 2013
    ..Previously, we demonstrated that sunitinib directly inhibited mTORC1 signaling in rat pheochromocytoma PC12 cells...
  27. pmc Acquired hypermethylation of the P16INK4A promoter in abdominal paraganglioma: relation to adverse tumor phenotype and predisposing mutation
    Nimrod B Kiss
    Department of Molecular Medicine and Surgery, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden
    Endocr Relat Cancer 20:65-78. 2013
    ..methylation of tumor suppressor genes (TSGs) and LINE1 (L1RE1) repeat elements were previously reported in pheochromocytoma and abdominal paraganglioma...
  28. ncbi Integrative genomics reveals frequent somatic NF1 mutations in sporadic pheochromocytomas
    Jenny Welander
    Department of Clinical and Experimental Medicine, Linkoping University, Linkoping, Sweden
    Hum Mol Genet 21:5406-16. 2012
    ..In this study, we elucidate some of the molecular mechanisms behind sporadic pheochromocytoma by performing a comprehensive analysis of copy number alterations, gene expression, promoter methylation and ..
  29. ncbi MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma
    Nelly Burnichon
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Paris, France
    Clin Cancer Res 18:2828-37. 2012
    ..How MAX mutations contribute to PCC/PGL and associated phenotypes remain unclear. This study aimed to examine the prevalence and associated phenotypic features of germline and somatic MAX mutations in PCC/PGL...
  30. pmc Pheochromocytoma and paraganglioma: understanding the complexities of the genetic background
    Lauren Fishbein
    Division of Endocrinology, Department of Medicine, Diabetes and Metabolism, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
    Cancer Genet 205:1-11. 2012
    ..This review summarizes the known germline and somatic mutations leading to the development of PCC and PGL, as well as biochemical profiling for PCCs/PGLs and screening of mutation carriers...
  31. ncbi Signaling pathways in pheochromocytomas and paragangliomas: prospects for future therapies
    Svenja Nölting
    Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, UK
    Endocr Pathol 23:21-33. 2012
    ..Increasing understanding of the germline and somatic mutations leading to pheochromocytoma and paraganglioma development has revealed crucial insights into the molecular pathology of these tumors...
  32. ncbi Overexpression of the natural antisense hypoxia-inducible factor-1alpha transcript is associated with malignant pheochromocytoma/paraganglioma
    P N Span
    Department of Laboratory Medicine, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The
    Endocr Relat Cancer 18:323-31. 2011
    ..Moreover, overexpression of aHIF and VEGF marks a higher metastatic potential in PGL...
  33. ncbi Pheochromocytoma penetrance varies by RET mutation in MEN 2A
    Frank J Quayle
    Washington University School of Medicine, St Louis, MO 63110, USA
    Surgery 142:800-5; discussion 805.e1. 2007
    The occurrence of pheochromocytoma in multiple endocrine neoplasia type 2A (MEN 2A) carriers varies greatly. This study aims to determine PC expression for specific MEN 2A RET mutations.
  34. ncbi Array-comparative genomic hybridization in sporadic benign pheochromocytomas
    Francien H van Nederveen
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC University Medical Center Rotterdam, Rotterdam, The Netherlands
    Endocr Relat Cancer 16:505-13. 2009
    ..In addition, genes on 21q and 22q might play a more important role in PCC pathogenesis than had been assumed thus far...
  35. ncbi Codon-specific development of pheochromocytoma in multiple endocrine neoplasia type 2
    Andreas Machens
    Department of General, Visceral, and Vascular Surgery, Martin Luther University Halle Wittenberg, Ernst Grube Strasse 40, D 06097 Halle Saale, Germany
    J Clin Endocrinol Metab 90:3999-4003. 2005
    ..Recent data suggest a codon-specific, age-related development of multiple endocrine neoplasia type 2...
  36. pmc C-cell hyperplasia, pheochromocytoma and sympathoadrenal malformation in a mouse model of multiple endocrine neoplasia type 2B
    C L Smith-Hicks
    Department of Genetics and Development, College of Physicians and Surgeons, Columbia University, 701 W 168th Street, New York, NY 10032, USA
    EMBO J 19:612-22. 2000
    ..Mutant mice displayed C-cell hyperplasia and chromaffin cell hyperplasia progressing to pheochromocytoma. Homozygotes did not develop gastrointestinal ganglioneuromas, but displayed ganglioneuromas of the adrenal ..
  37. ncbi MicroRNA expression profiling in benign (sporadic and hereditary) and recurring adrenal pheochromocytomas
    Zsófia Tömböl
    Faculty of Medicine, 2nd Department of Medicine, Semmelweis University, Budapest, Hungary
    Mod Pathol 23:1583-95. 2010
    ..Furthermore, microRNA expression profiles of a malignant pheochromocytoma and a pair of primary and recurrent tumors were studied by TaqMan Human MicroRNA Cards...
  38. ncbi A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines
    Anna M Sawka
    Department of Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA
    J Clin Endocrinol Metab 88:553-8. 2003
    ..measurements to measurements of 24-h urinary total metanephrines and catecholamines in outpatients tested for pheochromocytoma at Mayo Clinic Rochester from January 1, 1999, until November 27, 2000...
  39. ncbi Pheochromocytoma: current approaches and future directions
    Joel T Adler
    Section of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, Wisconsin, USA
    Oncologist 13:779-93. 2008
    ..While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure...
  40. pmc Noninvasive monitoring of a murine model of metastatic pheochromocytoma: a comparison of contrast-enhanced microCT and nonenhanced MRI
    Lucia Martiniova
    Reproductive and Adult Endocrinology Program, National Institutes of Child Health and Human Development, Bethesda, Maryland 20892 1109, USA
    J Magn Reson Imaging 29:685-91. 2009
    ..and nonenhanced respiratory-triggered magnetic resonance imaging (MRI) in an animal model of metastatic pheochromocytoma. Animal models are becoming important in the study of cancer treatment and imaging is useful in minimizing ..
  41. ncbi Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005
    Karel Pacak
    National Institute of Child Health and Development, NIH, Bethesda, MD, USA
    Nat Clin Pract Endocrinol Metab 3:92-102. 2007
    The First International Symposium on Pheochromocytoma, held in October 2005, included discussions about developments concerning these rare catecholamine-producing tumors...
  42. pmc Germline mutations in TMEM127 confer susceptibility to pheochromocytoma
    Yuejuan Qin
    Departments of Medicine, San Antonio, Texas, USA
    Nat Genet 42:229-33. 2010
    ..We identified the transmembrane-encoding gene TMEM127 on chromosome 2q11 as a new pheochromocytoma susceptibility gene...
  43. ncbi The effects of carbidopa on uptake of 6-18F-Fluoro-L-DOPA in PET of pheochromocytoma and extraadrenal abdominal paraganglioma
    Henri J L M Timmers
    Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 1109, USA
    J Nucl Med 48:1599-606. 2007
    ..The aim of this study was to investigate the sensitivity of (18)F-DOPA PET in the detection of paraganglioma and its metastatic lesions and to evaluate whether tracer uptake by the tumors is enhanced by carbidopa...
  44. ncbi Expression of vascular endothelial growth factor (VEGF) and its cognate receptors in human pheochromocytomas
    Kazuhiro Takekoshi
    Department of Clinical Pathology, Institute of Clinical Medicine, University of Tsukuba, 1 1 1 Tennoudai, Tsukuba, 305 8575, Japan
    Life Sci 74:863-71. 2004
    ..Therefore, we speculate that upregulation of VEGF expression and its receptors might be important in the pathogenesis of pheochromocytomas...
  45. ncbi Pheochromocytoma and extra-adrenal paraganglioma: updates
    Arthur S Tischler
    Department of Pathology, Tufts New England Medical Center, Boston, MA 02111, USA
    Arch Pathol Lab Med 132:1272-84. 2008
    Advances in genetics and gene expression profiling have led to new ways of thinking about the pathobiology of pheochromocytoma and extra-adrenal paraganglioma...
  46. ncbi Point mutation within the tyrosine kinase domain of the RET proto-oncogene in multiple endocrine neoplasia type 2B and related sporadic tumours
    C Eng
    Department of Pathology, University of Cambridge, UK
    Hum Mol Genet 3:237-41. 1994
    ..DNA from five of 13 apparently sporadic MTC and one of 12 apparently sporadic phaeochromocytomas harboured a similar mutation, but the corresponding germline DNA was wildtype in each case...
  47. pmc Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma
    Henri J L M Timmers
    Reproductive and Adult Endocrinology Program, Eunice Kennedy Shriver National Institutes of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 94:4757-67. 2009
    ....
  48. ncbi Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas
    Anne Paule Gimenez-Roqueplo
    AP HP, Hopital Europeen Georges Pompidou, Département de Génétique Moléculaire, Paris
    Cancer Res 63:5615-21. 2003
    ..These data strongly suggest that SDHB gene is a tumor suppressor gene and that the identification of germ-line mutations in SDHB gene in patients with ASPs should be considered as a high-risk factor for malignancy or recurrence...
  49. ncbi Expression of full-length polyglutamine-expanded Huntingtin disrupts growth factor receptor signaling in rat pheochromocytoma (PC12) cells
    Cheng Song
    Department of Pharmacology, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    J Biol Chem 277:6703-7. 2002
    ..Thus, our results may reflect an important molecular mechanism for the pathogenesis of the disease...
  50. ncbi Warburg tumours and the mechanisms of mitochondrial tumour suppressor genes. Barking up the right tree?
    Jean Pierre Bayley
    Department of Human Genetics, Center for Human and Clinical Genetics, Leiden University Medical Center, Building 2, Leiden, The Netherlands
    Curr Opin Genet Dev 20:324-9. 2010
    ..Here we discuss these diverse hypotheses and highlight very recent findings on the possible effects of IDH gene mutations...
  51. pmc Angiogenesis and vascular architecture in pheochromocytomas: distinctive traits in malignant tumors
    Judith Favier
    Collège de France and Institut National de la Santé et de la Recherche Médicale U36, Paris, France
    Am J Pathol 161:1235-46. 2002
    ..Altogether, our observations show that analysis of angiogenesis provides promising new criteria for the diagnosis of malignant pheochromocytomas...
  52. pmc The kinesin KIF1Bbeta acts downstream from EglN3 to induce apoptosis and is a potential 1p36 tumor suppressor
    Susanne Schlisio
    Department of Medical Oncology, Dana Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts 02115, USA
    Genes Dev 22:884-93. 2008
    ..Germline mutations of these genes cause familial pheochromocytoma and other neural crest-derived tumors...
  53. ncbi Imaging of neuroendocrine tumors
    Vittoria Rufini
    Department of Nuclear Medicine, Universita Cattolica del Sacro Cuore, Rome, Italy
    Semin Nucl Med 36:228-47. 2006
    ..New somatostatin analogs with different receptor affinity as well as other peptides are currently under investigation and will further improve our diagnostic and therapeutic capabilities in the future...
  54. ncbi N-cadherin expression in adrenal tumors: upregulation in malignant pheochromocytoma and downregulation in adrenocortical carcinoma
    Amir Khorram-Manesh
    Lundberg Laboratory for Cancer Research, Department of Surgery, Sahlgrenska University Hospital, Goteborg, Sweden
    Endocr Pathol 13:99-110. 2002
    ..NCAD was upregulated in MPCs, but downregulated in adrenocortical carcinoma. Thus, NCAD appears to be involved in the development of both cortical and medullary adrenal tumors...
  55. ncbi Alpha 7 nicotinic acetylcholine receptor expression and activity during neuronal differentiation of PC12 pheochromocytoma cells
    Arthur A Nery
    Departamento de Bioquimica, Instituto de Quimica, Universidade de Sao Paulo, Sao Paulo, Brazil
    J Mol Neurosci 41:329-39. 2010
    ..We show that PC12 pheochromocytoma cells induced to differentiation into neurons are an adequate model for studying differential nAChR gene ..
  56. ncbi The significance of angiogenesis in malignant pheochromocytomas
    Patrick P G M Rooijens
    Department of Surgery, Medical Centre Rijnmond Zuid, Location Clara, Rotterdam, The Netherlands
    Endocr Pathol 15:39-45. 2004
    ..However, the clinical significance of this prognostic marker is rather weak, because only 4 of the 19 malignant pheochromocytomas had microvessel density higher than this threshold of 28.5%...
  57. pmc The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer
    Herbert Chen
    Department of Surgery, University of Wisconsin, Madison, WI 53792 7375, USA
    Pancreas 39:775-83. 2010
    ..Genetic testing for RET mutations has allowed identification of familial cases and prophylactic thyroidectomy for cure. The only curative treatment is complete surgical resection...
  58. ncbi SDHB loss predicts malignancy in pheochromocytomas/sympathethic paragangliomas, but not through hypoxia signalling
    Annika Blank
    Institute of Pathology, Klinikum rechts der Isar, Technische Universitat Munchen, Munich, Germany
    Endocr Relat Cancer 17:919-28. 2010
    ..The lack of correlation of SDHB loss with classic hypoxia signals argues against the current hypoxia hypothesis in malignant PCC/PGL. We suggest SDHB protein loss as a marker of adverse outcome both in sporadic and in familial PCC/PGL...
  59. ncbi VEGF in 105 pheochromocytomas: enhanced expression correlates with malignant outcome
    Kaisa Salmenkivi
    Department of Pathology, Haartman Institute, University of Helsinki, Helsinki University Central Hospital, Helsinki, Finland
    APMIS 111:458-64. 2003
    ..Normal medullary cells are immunohistochemically negative. Thus, low VEGF expression in pheochromocytomas favors a benign diagnosis...
  60. ncbi Histological grading of adrenal and extra-adrenal pheochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal pheochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumors
    Noriko Kimura
    Department of Pathology and Laboratory Medicine, Tohoku Rosai Hospital, Japan Labor Health and Welfare Organization, 21 3 4 Dainohara Aoba ku Sendai 981 8563, Japan
    Endocr Pathol 16:23-32. 2005
    ..Differences between groups were statistically significant. The data show that using this grading scoring system for sympathetic paragangliomas correlates with both metastatic potential and patient survival...
  61. ncbi Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases
    Lester D R Thompson
    Department of Endocrine and Otorhinolaryngic Head and Neck Pathology, Armed Forces Institute of Pathology, Washington, DC 20306 6000, USA
    Am J Surg Pathol 26:551-66. 2002
    No comprehensive series has evaluated the histologic features of pheochromocytoma to separate benign from malignant pheochromocytoma by histomorphologic parameters only...
  62. ncbi Extra-adrenal pheochromocytoma: diagnosis and management
    Grant I S Disick
    Department of Urology, The Mount Sinai Medical Center, One Gustave L Levy Place, Box 1272, New York, NY 10029, USA
    Curr Urol Rep 8:83-8. 2007
    ..Recent reports suggest that a laparoscopic approach, along with intraoperative ultrasound, can safely remove these tumors. EAPs recur and metastasize more often than their adrenal counterparts, making lifelong follow-up essential...
  63. pmc Prevalence of germline mutations in patients with pheochromocytoma or abdominal paraganglioma and sporadic presentation: a population-based study in Western Sweden
    Andreas Muth
    Department of Surgery, Sahlgrenska University Hospital, 413 45 Gothenburg, Sweden
    World J Surg 36:1389-94. 2012
    ..5-24% of patients with pheochromocytoma (Pheo) or paraganglioma (PGL) and sporadic presentation...
  64. ncbi Pheochromocytoma: evaluation, diagnosis, and treatment
    M M Walther
    Urologic Oncology Branch, DCS NCI NIH, Bethesda, MD 20892 1501, USA
    World J Urol 17:35-9. 1999
    b>Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system. Signs and symptoms are generally related to catecholamine excess; these include hypertension, sweating, palpitatione, headaches, and anxiety attacks...
  65. pmc Characterization of two mouse models of metastatic pheochromocytoma using bioluminescence imaging
    Alessio Giubellino
    Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, Bethesda, MD 20892 1109, USA
    Cancer Lett 316:46-52. 2012
    b>Pheochromocytoma is the most common tumor of the adrenal medulla in adults. The lack of sensitive animal models of pheochromocytoma has hindered the study of this tumor and in vivo evaluation of antitumor agents...
  66. ncbi Usefulness of [18F]-DA and [18F]-DOPA for PET imaging in a mouse model of pheochromocytoma
    Lucia Martiniova
    Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, Bethesda, MD 20892 1109, USA
    Nucl Med Biol 39:215-26. 2012
    ..c.) and metastatic pheochromocytoma in mice; to assess the expression of the norepinephrine transporter (NET) and vesicular monoamine ..
  67. ncbi The genetics of phaeochromocytoma: using clinical features to guide genetic testing
    Mariam Jafri
    Centre for Rare Diseases and Personalised Medicine, The Medical School, Institute of Biomedical Research West, University of Birmingham, Edgbaston, Birmingham B15 2TT, UK
    Eur J Endocrinol 166:151-8. 2012
    ..Mutation carriers should be offered specific surveillance programmes (according to the relevant gene). In this review, we will describe the genetics of aPCA/eFPGL and strategies for genetic testing...
  68. ncbi Malignant pheochromocytoma: a review
    Avital Harari
    Department of Surgery, Division of Endocrine Surgery of University of California, San Francisco, CA 94115, USA
    Am J Surg 201:700-8. 2011
    ..Traditionally, there has been no reliable method available to predict the malignant potential of pheochromocytoma. However, recent research has increased focus on differentiating at the time of surgery/diagnosis those ..
  69. ncbi Comparison of diagnostic accuracy of urinary free metanephrines, vanillyl mandelic Acid, and catecholamines and plasma catecholamines for diagnosis of pheochromocytoma
    James G Boyle
    Division of Cardiovascular and Medical Sciences, Western Infirmary, University of Glasgow, Glasgow G12 8TA, United Kingdom
    J Clin Endocrinol Metab 92:4602-8. 2007
    ..suggests that plasma-free metanephrines provide a highly sensitive test in patients requiring exclusion of pheochromocytoma. The diagnostic efficacy of urinary free metanephrines, however, has not been evaluated...
  70. ncbi Outcome in patients with adrenal incidentaloma selected for surgery: an analysis of 88 cases investigated in a single clinical center
    J P Luton
    Clinique des Maladies Endocriniennes et Métaboliques, Hopital Cochin, and Université René Descartes Paris V, Paris, France
    Eur J Endocrinol 143:111-7. 2000
    ..The study was designed to evaluate the clinical, endocrinological and radiological parameters used to investigate adrenal incidentalomas and select patients for surgery...
  71. ncbi Spectrum and prevalence of FP/TMEM127 gene mutations in pheochromocytomas and paragangliomas
    Li Yao
    Division of Hematology, Department of Medicine, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA
    JAMA 304:2611-9. 2010
    ..We recently identified germline mutations of the novel transmembrane-encoding gene FP/TMEM127 in familial and sporadic pheochromocytomas consistent with a tumor suppressor effect...
  72. pmc Increased uptake of [¹²³I]meta-iodobenzylguanidine, [¹⁸F]fluorodopamine, and [³H]norepinephrine in mouse pheochromocytoma cells and tumors after treatment with the histone deacetylase inhibitors
    Lucia Martiniova
    Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E 3140, 10 Center Drive MSC 1109, Bethesda, Maryland 20892 1109, USA
    Endocr Relat Cancer 18:143-57. 2011
    I]meta-iodobenzylguanidine ([¹³¹I]MIBG) is the most commonly used treatment for metastatic pheochromocytoma and paraganglioma...
  73. pmc Pheochromocytoma: implications in tumorigenesis and the actual management
    U Shah
    Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Minerva Endocrinol 37:141-56. 2012
    ..The study of families with genetically inherited mutations in pheochromocytoma susceptibility genes has greatly enhanced our understanding of the pathophysiology and mechanisms of ..
  74. ncbi A decade (2001-2010) of genetic testing for pheochromocytoma and paraganglioma
    A Buffet
    Assistance Publique Hopitaux de Paris, Hopital Europeen Georges Pompidou, Service de Genetique, 20 rue Leblanc, Paris, France
    Horm Metab Res 44:359-66. 2012
    The identification of 9 susceptibility genes for paraganglioma/pheochromocytoma between 2001 and 2010 has led to the development of routine genetic tests...
  75. pmc Combined inhibition of mTORC1 and mTORC2 signaling pathways is a promising therapeutic option in inhibiting pheochromocytoma tumor growth: in vitro and in vivo studies in female athymic nude mice
    Alessio Giubellino
    Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA
    Endocrinology 154:646-55. 2013
    ..out an important role for the mammalian target of rapamycin (mTOR) signaling pathway in the development of pheochromocytoma. Analyzing a set of pheochromocytomas from patients with different genetic backgrounds, we observed and ..
  76. pmc Murine models and cell lines for the investigation of pheochromocytoma: applications for future therapies?
    Esther Korpershoek
    Department of Pathology, Josephine Nefkens Institute, Erasmus MC University Medical Center Rotterdam, Room Ae304, P O Box 2040, 3000, CA, Rotterdam, The Netherlands
    Endocr Pathol 23:43-54. 2012
    ..which chemotherapeutic options could be useful for the treatment of metastatic PCC, cell lines such as mouse pheochromocytoma (MPC) and mouse tumor tissue (MTT) cells have been recently introduced and they both showed metastatic ..
  77. ncbi Genetic studies in a coexistence of acromegaly, pheochromocytoma, gastrointestinal stromal tumor (GIST) and thyroid follicular adenoma
    Cesar Luiz Boguszewski
    Endocrine Division, Department of Internal Medicine, University Hospital at Universidade Federal do Parana, Curitiba, Brazil
    Arq Bras Endocrinol Metabol 56:507-12. 2012
    We report on an adult woman with rare coexistence of acromegaly, pheochromocytoma (PHEO), gastrointestinal stromal tumor (GIST), intestinal polyposis, and thyroid follicular adenoma...
  78. ncbi Global and regional CpG methylation in pheochromocytomas and abdominal paragangliomas: association to malignant behavior
    Janos Geli
    Department of Molecular Medicine and Surgery, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden
    Clin Cancer Res 14:2551-9. 2008
    ..This study aims to quantitatively assess promoter and global methylation changes in pheochromocytomas and abdominal paragangliomas and its relation to tumor phenotypes...
  79. ncbi Pheochromocytoma and functional paraganglioma
    Heather Yeo
    Department of General Surgery, Yale University School of Medicine, New Haven, CT 06520, USA
    Curr Opin Oncol 17:13-8. 2005
    b>Pheochromocytoma is a rare, but clinically important tumor of chromaffin cells...
  80. ncbi Pheochromocytoma in Italy: a multicentric retrospective study
    M Mannelli
    Department of Clinical Physiopathology, Endocrine Unit, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy
    Eur J Endocrinol 141:619-24. 1999
    To conduct an epidemiological study on pheochromocytoma in Italy.
  81. pmc Deletion of the synaptic protein interaction site of the N-type (CaV2.2) calcium channel inhibits secretion in mouse pheochromocytoma cells
    Amy B Harkins
    Department of Pharmacological and Physiological Science, Saint Louis University School of Medicine, 1402 South Grand Boulevard, St Louis, MO 63104, USA
    Proc Natl Acad Sci U S A 101:15219-24. 2004
    ..subunit and transiently transfected either the wild-type alpha1B or the synprint deletion mutant into mouse pheochromocytoma (MPC) cell line 9/3L, a cell line that has the machinery required for rapid stimulated exocytosis but lacks ..
  82. ncbi Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma
    F Forrer
    Institute of Nuclear Medicine, University Hospital of Basel, Basel, Switzerland
    Q J Nucl Med Mol Imaging 52:334-40. 2008
    ..We evaluated the effectiveness and toxicity of radiolabeled DOTATOC in patients with metastatic paraganglioma and pheochromocytoma.
  83. ncbi Denaturing high performance liquid chromatography detection of SDHB, SDHD, and VHL germline mutations in pheochromocytoma
    Goswin Y Meyer-Rochow
    Kolling Institute of Medical Research, Royal North Shore Hospital and University of Sydney, Australia
    J Surg Res 157:55-62. 2009
    ..syndromes multiple endocrine neoplasia Type 2, von Hippel Lindau disease, Neurofibromatosis Type 1, and the pheochromocytoma/paraganglioma syndromes caused by mutations in the RET, VHL, NF1, SDHB, and SDHD genes, respectively...
  84. ncbi The role of Sdh4p Tyr-89 in ubiquinone reduction by the Saccharomyces cerevisiae succinate dehydrogenase
    Yuri Silkin
    Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada T6G 2H7
    Biochim Biophys Acta 1767:143-50. 2007
    ..Our results support a role for Tyr-89 in the protonation of ubiquinone and argue that the generation of reactive oxygen species is not causative of tumor formation...
  85. ncbi Retroperitoneoscopic adrenalectomy for pheochromocytoma in a morbidly obese
    J Esteban Varela
    Minimally Invasive and Bariatric Surgery, Surgical Service, VA North Texas Healthcare System, 4500 South Lancaster Road Ste 112, Dallas, TX 75216, USA
    Obes Surg 19:1180-2. 2009
    ..It is known that laparoscopic techniques in the morbidly obese may be challenging; therefore, alternative approaches such as the retroperitoneoscopic may prove to be of value in the morbidly obese...
  86. ncbi Synchronous presentation of primary non-small cell lung carcinoma and pheochromocytoma
    Edward P Chen
    Division of Cardiothoracic Surgery and General Surgery, Emory University Affiliated Hospitals, Atlanta, Georgia, USA
    Ann Thorac Surg 74:924-6. 2002
    ..5-cm right adrenal gland mass. Workup of the adrenal lesion revealed a pheochromocytoma. The patient underwent combined mediastinoscopy, laparoscopic right adrenalectomy, and right thoracotomy with ..
  87. pmc Integrative epigenomic and genomic analysis of malignant pheochromocytoma
    Johanna Sandgren
    Department of Surgical Sciences, Uppsala University, Uppsala University Hospital, SE 75185 Uppsala, Sweden
    Exp Mol Med 42:484-502. 2010
    ..Furthermore, genomic aberrations such as DNA copy number changes are common events in tumors. Pheochromocytoma is a rare endocrine tumor of the adrenal gland that mostly occurs sporadic with unknown epigenetic/genetic ..
  88. ncbi Phaeochromocytoma
    Jacques W M Lenders
    Department of Internal Medicine, Division of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen 6525GA, Netherlands
    Lancet 366:665-75. 2005
    ..If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours...
  89. ncbi Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma
    Anthony M Joshua
    Department of Medical Oncology, Princess Margaret Hospital, University Health Network, 610 University Avenue, M5G 2M9 Toronto, Ontario, Canada
    J Clin Endocrinol Metab 94:5-9. 2009
    ..The optimal systemic treatment for advanced disease is undefined, due in part to lack of effective agents. Here we report our experience suggesting that sunitinib is an effective agent in this malignancy...
  90. pmc Pheochromocytoma in rats with multiple endocrine neoplasia (MENX) shares gene expression patterns with human pheochromocytoma
    Sara Molatore
    Department of Pathology, Helmholtz Zentrum Munchen, 85764 Neuherberg, Germany
    Proc Natl Acad Sci U S A 107:18493-8. 2010
    ..crest origin arising from chromaffin cells of the adrenal medulla and sympathetic ganglia (extra-adrenal pheochromocytoma)...
  91. ncbi Pheochromocytoma
    Lee C Pederson
    Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Box 444, Houston, TX 77030, USA
    Curr Treat Options Oncol 4:329-37. 2003
    b>Pheochromocytoma is a rare tumor, but it represents a potentially curable form of hypertension. In patients with inherited pheochromocytoma, benign and bilateral tumors are more common...
  92. pmc Pharmacologic modulation of serine/threonine phosphorylation highly sensitizes PHEO in a MPC cell and mouse model to conventional chemotherapy
    Lucia Martiniova
    Reproductive and Adult Endocrinology Program, Eunice Kennedy Shriver, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 6:e14678. 2011
    ..PP2A), on its ability to inhibit a low growth fraction and highly drug-resistant solid neuroendocrine tumor, such as metastatic pheochromocytoma (PHEO). Subsequently, we evaluated the increased efficacy of chemotherapy combined with LB1.
  93. ncbi Pheochromocytoma: an imaging chameleon
    Michael A Blake
    Department of Radiology, Division of Abdominal Imaging and Intervention, Massachusetts General Hospital, White 270, 55 Fruit St, Boston, MA 02114, USA
    Radiographics 24:S87-99. 2004
    ..b>Pheochromocytoma is an important, often clinically occult neoplasm with devastating consequences if overlooked...
  94. ncbi Laparoscopic and retroperitoneoscopic treatment of pheochromocytomas and retroperitoneal paragangliomas: results of 161 tumors in 126 patients
    Martin K Walz
    Klinik für Chirurgie und Zentrum für Minimal Invasive Chirurgie, Kliniken Essen Mitte, Akademisches Lehrkrankenhaus der Universität Duisburg Essen, Henricistrasse 92, Essen, D 45136, Germany
    World J Surg 30:899-908. 2006
    ....
  95. ncbi Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001)
    M R Druce
    Department of Endocrinology, Barts and the London School of Medicine, London, UK
    Horm Metab Res 41:697-702. 2009
    ..We list current therapies used for malignant pheochromocytoma; however, these generally achieve relatively low success rates...
  96. ncbi Endoscopic adrenalectomy: an analysis of the transperitoneal and retroperitoneal approaches and results of a prospective follow-up study
    I Gockel
    Department of General and Abdominal Surgery, Johannes Gutenberg University, Langenbeckstrasse 1, 55101 Mainz, Germany
    Surg Endosc 19:569-73. 2005
    ..The aim of this study was to determine which of these is the optimal surgical technique in a prospectively designed analysis of a large series of patients operated on by a single team over a 10-year period...
  97. ncbi Stathmin as a marker for malignancy in pheochromocytomas
    P Bjorklund
    Department of Surgical Sciences, Uppsala University Hospital, Uppsala, Sweden
    Exp Clin Endocrinol Diabetes 118:27-30. 2010
    ..The results introduce stathmin as a possible diagnostic marker for malignant pheochromocytomas, and further evaluations are warranted...
  98. ncbi Phaeochromocytomas and sympathetic paragangliomas
    B J Petri
    Department of Pathology, Erasmus MC University Medical Centre, Rotterdam, The Netherlands
    Br J Surg 96:1381-92. 2009
    ..Surgical resection is the treatment of choice for both PCC and sPGL, but controversy exists about the management of patients with bilateral or multiple tumours...
  99. ncbi Stathmin expression in pheochromocytomas, paragangliomas, and in other endocrine tumors
    Peter M Sadow
    Department of Pathology, Brigham and Women s Hospital, Boston, MA 02115, USA
    Endocr Pathol 19:97-103. 2008
    ..These results show that stathmin is expressed at higher levels in more rapidly proliferating endocrine tumors. However, it is probably not useful as a stand-alone marker to determine malignancy in pheochromocytomas for individual tumors...
  100. ncbi Pheochromocytoma: state-of-the-art and future prospects
    Emmanuel L Bravo
    Department of Nephrology and Hypertension, Cleveland Clinic Foundation, Cleveland Ohio 44195, USA
    Endocr Rev 24:539-53. 2003
    This review provides current understanding of the pathophysiology of pheochromocytoma and the wide range of associated clinical manifestations that have led to earlier recognition of the disease...
  101. ncbi Pheochromocytoma in MEN 2A syndrome. Study of 54 patients
    Jose M Rodriguez
    Endocrine Surgery Unit, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain
    World J Surg 32:2520-6. 2008
    b>Pheochromocytoma occurs in nearly 50% of MEN 2A (multiple endocrine neoplasia, type 2A) cases...

Research Grants63

  1. Exocytosis and Coupled Endocytosis in Neuroendocrine Cells
    JOHN DAVID CASTLE; Fiscal Year: 2010
    ..roles of Secretory Carrier Membrane Proteins (SCAMPs) in exo-/endocytic coupling in adrenal medulla- derived pheochromocytoma (PC12) cells and mouse adrenal chromaffin cells...
  2. Epigenetic Regulation in Cranial Neural Crest Development
    Na Hu; Fiscal Year: 2012
    ..neural crest derived cells are prone to numerous types of cancer, including neurofibromatosis, melanoma, pheochromocytoma and neuroblastoma...
  3. MOLECULAR GENETICS OF SENSORY ORGAN DEVELOPMENT
    James W Posakony; Fiscal Year: 2013
    ..most lethal form of lung cancer), neuroblastoma (an aggressive childhood cancer), medullary thyroid cancer, pheochromocytoma (a tumor of the adrenal medulla), small cell neuroendocrine carcinoma of the prostate, and Merkel cell ..
  4. The role of the von-Hippel Lindau protein in developmental cell death in neurons
    KATHLEEN M MCAVOY; Fiscal Year: 2011
    ..in which affected individuals have an increased rate of renal clear cell carcinoma, hemangioblastoma, and pheochromocytoma. Lee et al...
  5. Optimizing 131I-mIBG Therapy for Children with Advanced Neuroblastoma
    Steven Dubois; Fiscal Year: 2013
    ..For example, 131I-mIBG is also used in the treatment of adults with pheochromocytoma. More generally, our work will inform other research focused on the use of radiation sensitizers and on ..
  6. STRUCTURES OF RNA BINDING PROTEINS FROM INFLUENZA VIRUS
    Gaetano Montelione; Fiscal Year: 2000
    ..Also, plasma NPY levels are elevated in patients with congestive heart failure (CHF) and pheochromocytoma, and in hypertensive rats...
  7. DEXAMETHASONE/NGF REGULATE CASEIN KINASE IN PC12 CELLS
    Robert Van Buskirk; Fiscal Year: 1990
    ..differentiation of several neural lines including sympathetic neurons, adrenal chromaffin cells and the rat pheochromocytoma cell line, PC12. The second messenger(s) for nerve growth factor has (have) not yet been identified...
  8. TUMOR CELL SYNTHESIS AND SECRETION OF PEPTIDE HORMONES
    David Orth; Fiscal Year: 1991
    ..Regulation of POMC secretion in normal rat adrenal medullary cells and PC12 rat adrenal pheochromocytoma cells will be examined in a dispersed cell perifusion column system...
  9. BLOOD-BRAIN BARRIER IN NORMAL & EXPERIMENTAL BRAIN
    RICHARD BROADWELL; Fiscal Year: 1990
    ..of fetal/neonatal CNS, adult non-neural tissue (anterior and posterior lobes of the pituitary gland), PC-12 (pheochromocytoma) cells, and pituitary fenestrated endothelia...
  10. MECHANISM OF PC12 CELL ADHESION TO COLLAGEN AND LAMININ
    David Turner; Fiscal Year: 1991
    ..PC12 cells, an established line of cells derived from a rat pheochromocytoma, resemble sympathetic neurons in many respects, including extension of neurites in response to nerve growth ..
  11. GLYCOCONJUGATES IN SYMPATHO-ADRENAL DEVELOPMENT
    GERALD SCHWARTING; Fiscal Year: 1990
    ..PC12 pheochromocytoma cells, which exhibit characteristics between chromaffin cells and neurons and can be induced to differentiate ..
  12. MIBG ANALOGUE RADIOPHARMACEUTICALS
    Ganesan Vaidyanathan; Fiscal Year: 2001
    ..MIBG) has been used in the detection and therapy of neuroendocrine tumors, especially neuroblastoma and pheochromocytoma. Although it is a satisfactory agent for diagnostic applications, the outcome of MIBG therapy is inadequate...
  13. REGULATION OF SODIUM CHANNEL EXPRESSION
    ROBERT MAUE; Fiscal Year: 2000
    ..The effect of NGF on rat pheochromocytoma (PC12) cells transfected or microinjected with genes encoding constitutively active or interfering forms of c-..
  14. CLINICAL PET AND LABORATORY STUDIES OF ADRENERGIC TUMORS
    Barry Shulkin; Fiscal Year: 2002
    ..evaluate the biodistribution of C-11epinephrine in patients with the neuroendocrine tumors neuroblastoma and pheochromocytoma, and to characterize the uptake and retention within tumors themselves...
  15. EFFECTS OF ORGANOTINS ON HIPPOCAMPAL NEURONAL DEVELOPMEN
    WILLIAM ATCHISON; Fiscal Year: 2000
    ..tension in developing hippocampal pyramidal neurons in primary culture, or transformed neuronal cells- rat pheochromocytoma (PC12) cells- a commonly-used model for neuronal growth, and a cell type which exhibits sensitivity to TMT...
  16. REGULATED EXPRESSION--CATECHOLAMINE BIOSYNTHESIS GENES
    Elaine Lewis; Fiscal Year: 1991
    ..gene required for stimulation of TH transcription by cyclic AMP, epidermal growth factor and cell density in pheochromocytoma cells...
  17. Nicotine Addiction: ACh Receptors and Secretion
    Aaron Fox; Fiscal Year: 2002
    ..My lab (in collaboration with Dr. Art Tischler's lab) recently identified a mouse pheochromocytoma (MPC) cell line that is secretion competent but which expresses no nicotinic receptors and few or no ..
  18. CATECHOLAMINE BIOSYNTHETIC ENZYMES
    Esther Sabban; Fiscal Year: 1991
    ..A Lambdagt11 expression library of cDNA prepared from PC12 pheochromocytoma cell mRNA is being screened with antibodies to DBH to isolate the cDNA for rat DBH. Ib...
  19. ADRENAL CATECHOLAMINE SECRETION
    ROBERT PERLMAN; Fiscal Year: 1990
    ..proposed in this application will investigate protein phosphorylation and phospholipid metabolism in PC12 pheochromocytoma cells and in the superior cervical ganglion of the rat...
  20. CHEMICAL MODELS OF PRESYNAPTIC NEUROMUSCULAR DISEASE
    WILLIAM ATCHISON; Fiscal Year: 1999
    ..terminals (synaptosomes) of rats or cultured mouse hybridoma neuroblastoma/glioma NG108-15 cells, or rat PC12 pheochromocytoma cells...
  21. Analytical Techniques for Exocytosis
    Andrew Ewing; Fiscal Year: 2009
    ..This work will be done with pheochromocytoma (PC12) cells in culture...
  22. ENCAPSULATED DOPAMINERGIC TUMOR CELLS AS BRAIN IMPLANTS
    CHRISTINE JAEGER; Fiscal Year: 1991
    ..Two DA cell lines: pheochromocytoma derived PC12 and the murine neuroblastoma NX31/T28 will be enclosed in polyvinylchloride acrylic copolymer ..
  23. MOLECULAR MECHANISMS OF PCP AND SIGMA DRUG ACTION
    LINDA WERLING; Fiscal Year: 2001
    ..human neuroblastoma cells SK-N-SH and SH-SY5Y for studies on regulation of norepinephrine release, and in rat pheochromocytoma (PC12) cells for regulation of dopamine release...
  24. Cholinergic and cognitive decline in response to TrkA knockdown using RNAi
    Vinay Parikh; Fiscal Year: 2009
    ..II promoter, and screening of TrkA miRNA sequences for suppression of TrkA receptor expression in rat pheochromocytoma cells...
  25. NERVE GROWTH FACTOR MODULATION OF THE EGF RECEPTOR
    Alice Brown; Fiscal Year: 1992
    The PC-12 pheochromocytoma and several neuroblastoma cell lines possess specific receptors for both nerve growth factor (NGF) and epidermal growth factor (EGF)...
  26. BIOPTERIN, CATECHOLAMINES, & NO IN REGULATING APOPTOSIS
    Robert Levine; Fiscal Year: 2002
    ..Cells to be studied include pheochromocytoma (PC12), sympathetic neurons, and fetal nigral neurons...
  27. MECHANISMS OF APOPTOTIC DEATH IN DOPAMINERGIC NEURONS
    Anna Liisa Nieminen; Fiscal Year: 2003
    ..In cultured rat pheochromocytoma-12 (PC12) dopaminergic cells, I will evaluate the following hypotheses: 1) MPP+ causes a mitochondrial ..
  28. Imaging agents of norepinephrine transporters
    HANK KUNG; Fiscal Year: 2007
    ..Imaging of norepinephrine neurons by I-131/ I-123 is commonly used for diagnosis and treatment of pheochromocytoma and neuroblastoma expressing NET and the same tracer is also useful for imaging norepinephrine neuronal ..
  29. Sphingosine 1 Phosphate Receptor and Neurite Retraction
    Rachelle Toman; Fiscal Year: 2003
    ..EDG-8, initially cloned by Dr. Norman Lee as a nerve growth factor receptor in rat pheochromocytoma (PC12) cells, is preferentially expressed in the brain...
  30. Functional and Tissue Specific Effects of VHL Mutations
    WENDY RATHMELL; Fiscal Year: 2007
    ..VHL type 2 disease is defined by the presence of pheochromocytoma and is subdivided into type 2A (pheochromocytoma and hemangioblastoma), 2B (pheochromocytoma, renal cell ..
  31. REGULATION OF NEURAL DEVELOPMENT BY PROTEIN KINASES
    John Wagner; Fiscal Year: 1992
    ..These studies use the PC12 cell line which is a cloned line derived from a transplantable pheochromocytoma. This line has proved to be a useful model system to study the regulation of neural differentiation...
  32. Hyperthermia: Uncoupling the agony from Ecstasy
    JON SPRAGUE; Fiscal Year: 2007
    ..of UCP3 activation will lead to improved targeted therapies for treatment of heat-related illnesses such as pheochromocytoma and hyperthyroidism...
  33. VASOACTIVE INTESTINAL & LUNG POLYPEPTIDES--TUMOR
    SAMI SAID; Fiscal Year: 1980
    ..and nervous systems, and in elevated concentrations in these tumors: pancreatic (islet-cell) adenomas, pheochromocytoma, neuroblastoma, medullary thyroid carcinoma, and bronchogenic carcinoma...
  34. DYNAMICS OF C-FOS PROTEIN INTERACTIONS
    EDWARD ZIFF; Fiscal Year: 2000
    ..We have also shown that when nerve growth factor (NGF) stimulates model PC12 pheochromocytoma cells to differentiate down a neuronal pathway, fos is induced. Peak fos transcription is 30 min. post NGF...
  35. ADRENAL CATECHOLAMINE SECRETION
    ROBERT PERLMAN; Fiscal Year: 1980
    ..We plan to study these processes in pheochromocytoma cells and in normal chromaffin cells...
  36. CYTOCHROME P450 2D6 VARIANTS IN NEUROTOXICITY
    Timothy Macdonald; Fiscal Year: 1999
    ..Finally, we propose to transfect a catecholaminergic cell line, rat pheochromocytoma PC12 cells, with the wild type and mutant enzymes...
  37. STRUCTURE/ACTIVITY STUDIES OF NPY
    Ambikaipakan Balasubramaniam; Fiscal Year: 2001
    ..NPY levels are elevated in the plasma of hypertensive rats, patients with congestive heart failure (CHF) and pheochromocytoma, and in the hypothalamus of obese rats suggest that NPY sequence may be modulated for therapeutic use...
  38. RESPONSE OF NEURONS TO ELECTRICAL FIELDS
    Kenneth Robinson; Fiscal Year: 1992
    ..Initial experiments will be done using PC 12 cells. These cells are derived from a rat pheochromocytoma, and in response to nerve growth factor (NGF) they stop dividing and differentiate into sympathetic-like ..
  39. ECTOPIC HORMONE SYNTHESIS IN PHEOCHROMOCYTOMA CELLS
    Richard Goodman; Fiscal Year: 1991
    ..Expression of vasoactive intestinal polypeptide (VIP) in pheochromocytoma and neuroblastoma cells is regulated by cAMP and protein kinase C-dependent pathways...
  40. SCRAPIE AGENT REPLICATION IN AN IN VITRO NEURONAL MODEL
    Richard Rubenstein; Fiscal Year: 1992
    ..We will use a cloned pheochromocytoma cell line, termed PC12, as an in vitro neuronal model to study scrapie...
  41. REGULATION OF VGF BY NEUROTROPHIC GROWTH FACTORS
    Stephen Salton; Fiscal Year: 2001
    ..regulate both neuronal differentiation and the response of the adult nervous system to injury, using the PC12 pheochromocytoma cell line as a model...
  42. Regulation of c-fos By Nerve Growth Factor
    Michael Pellegrino; Fiscal Year: 2006
    ..This phenomonon is best shown using the rat pheochromocytoma cell line, PC12 cells...
  43. CELLULAR MECHANISMS OF AXONAL REPAIR AND DEGENERATION
    Harvey Fishman; Fiscal Year: 2004
    ..in the repair of injured mammalian axons using neurites in nerve-model cultures of unmyelinated rat PNS pheochromocytoma (PC12), rat CNS (B104) cells, and in unmyelinated versus myelinated rat embryonic forebrain cultures at the ..
  44. HUMAN DOPAMINE-BETA-HYDROXYLASE
    DANIEL O CONNOR; Fiscal Year: 1980
    ..isolating the enzyme from human plasma, as well as other human tissues such as adrenal gland and pheochromocytoma, 2. conducting physicochemical comparisons of the molecular properties of these enzymes, and 3...
  45. ROLE OF IGF-11 IN NERVOUS SYSTEM DEVELOPMENT
    DOUGLAS ISHII; Fiscal Year: 1990
    ..be studied in cultured sensory and sympathetic neurons from chick and rat, and in human neuroblastoma and rat pheochromocytoma cell lines...
  46. MOLECULAR ANALYSIS OF MALIGNANT TRANSFORMATION
    ARTHUR PARDEE; Fiscal Year: 1992
    ..The genetic control differentiation of 3 malignant cellular systems will be studied: teratocarcinoma, pheochromocytoma, and colonic carcinoma...
  47. ECTOPIC HORMONE SYNTHESIS IN PHEOCHROMOCYTOMA CELLS
    Richard Goodman; Fiscal Year: 2009
    ..abstract_text> ..
  48. NO AND ESTRADIOL SUSTAINED NEURONAL DIFFERENTIATION
    Robin Farias Eisner; Fiscal Year: 2002
    ..which E2 sustains neuronal differentiation and neurite outgrowth of PCl2 cells, derived originally from a rat pheochromocytoma, by determining whether E2-induced NO is required for the continued differentiation program; and to identify ..
  49. Amyloid-Beta-induced ceramide production in neurons
    Kalipada Pahan; Fiscal Year: 2001
    ..we have found that Abeta induces the production of ceramide by more than 10 fold within 24 h in rat pheochromocytoma (PC 12) cells...
  50. Integrated MAP Kinase Signaling In Cell Differentiation
    Lynn Heasley; Fiscal Year: 2004
    ..Our studies with cultured PC12 pheochromocytoma cells, an established model for analysis of nerve growth factor (NGF)- dependent signaling pathways that ..
  51. REGULATION OF CALMODULIN DEPENDENT PROTEIN KINASES
    ARTHUR EDELMAN; Fiscal Year: 1999
    ..of extracellular and intracellular signals which induce phosphorylation and activation of CaM kinase I in pheochromocytoma PC12 cells; 2, Analysis of signal transduction mechanisms in the CaM kinase I cascade...
  52. GROWTH-ASSOCIATED PROTEIN-(GAP)-43 IN NEURONAL DEVELOPME
    Lawrence Baizer; Fiscal Year: 1993
    ..expression of GAP-43 coincides with nerve growth factor (NGF)-induced neurite outgrowth from cultured PC12 pheochromocytoma cells...
  53. CHROMAFFIN CELL PROLIFERATION IN NEOPLASTIC PROGRESSION
    Arthur Tischler; Fiscal Year: 2006
    ..The proposed studies will help to elucidate mechanisms that may be involved in the development and progression of pheochromocytomas. ..
  54. PHEOCHROMOCYTOMAS FROM NEUROFIBROMATOSIS KNOCKOUT MICE
    Arthur Tischler; Fiscal Year: 2003
    ..are developmentally related to neuroblastomas, which are common adrenal medullary neoplasms, and pheochromocytoma cells can undergo neuronal differentiation...
  55. Divalent Metal Transporter: Role in Manganese Toxicity
    JEROME ROTH; Fiscal Year: 2004
    ....
  56. MINORITY PREDOCTORAL FELLOWSHIP PROGRAM
    HECTOR MOBINE; Fiscal Year: 2008
    ..the cell biology, and the science of cardiomyopathy in particular in states of heightened stress, exposure to pheochromocytoma and pregnancy, may enable us to begin to answer some of these questions...
  57. PROCESSING OF CHROMOGRANIN A BY THE FIBRINOLYTIC SYSTEM
    ROBERT PARMER; Fiscal Year: 2001
    ....
  58. Homologous Recombination in a Secretory Mouse Cell Line
    AMY HARKINS; Fiscal Year: 2003
    ..We have identified a mouse pheochromocytoma cell line that exhibits Ca2+-dependent exocytosis of membrane-bound vesicles...
  59. Sympathetic Outflow to Catecholamine Storage Vesicles
    DANIEL O CONNOR; Fiscal Year: 2006
    ..Finally, these photoprotein and cDNA reagents can be widely employed by other investigators in elucidating biosynthetic, trafficking, and ion flux events in the sympathochromaffin system. ..
  60. Role of Natriuretic Peptides in the Cochlea
    George Trachte; Fiscal Year: 2004
    ..abstract_text> ..
  61. Chromaffin Cell physiology: Novel molecular approaches
    Sushil Mahata; Fiscal Year: 2009
    ..These studies, utilizing unique knockout, transgenic, and human variants of catestatin, are likely to establish novel catestatin mechanisms in the development of hypertension and its modulation through nicotinic-cholinergic receptors. ..
  62. Liberation of Intracellular Zinc and Neuronal Cell Death
    Elias Aizenman; Fiscal Year: 2007
    ..With this information, we hope to uncover novel therapeutic strategies to prevent or halt the progression of these and other neurodegenerative conditions. ..
  63. Imaging of O6-Alkylguanine-DNA Alkyltransferase
    Ganesan Vaidyanathan; Fiscal Year: 2005
    ..In summary, this proposal seeks to determine whether the development of a suitable agent for the imaging of tumor AGT will facilitate chemotherapy planning, thereby improving the outcome of alkylator chemotherapy. ..