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1. Nofech-Mozes S, Mackenzie R, Kahn HJ, Ehrlich L, Raphael SJ: Breast metastasis by medullary thyroid carcinoma detected by FDG positron emission tomography. Ann Diagn Pathol; 2008 Feb;12(1):67-71
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  • Medullary thyroid carcinoma (MTC) is an uncommon thyroid cancer comprising 5% to 8% of thyroid neoplasms.
  • In contrast to common thyroid tumors, this tumor originates from the calcitonin-producing C cells.
  • Common metastatic sites include the liver, bone, brain, and adrenal medulla.
  • (1) metastasis to the breast is an extremely rare occurrence and could be easily confused clinically and pathologically with a primary breast neoplasm and (2) this is the first reported case of detection of breast metastasis by an MTC using FDG ((18)F-fluoro-2-deoxy-D-glucose) positron emission tomography with an accompanying histologic description.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Cisplatin / therapeutic use. Combined Modality Therapy. Doxorubicin / therapeutic use. Female. Humans. Immunohistochemistry. Middle Aged. Palliative Care

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  • (PMID = 18164420.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
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2. Palaoğlu S, Sungur A, Cila A, Ozdemir N, Ruacan S: Diethylstilbestrol-induced prolactinoma: dose-related tumor growth and effect of catecholaminergic cells on prolactin tumor cells. Surg Neurol; 2005;64 Suppl 2:S42-7
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  • [Title] Diethylstilbestrol-induced prolactinoma: dose-related tumor growth and effect of catecholaminergic cells on prolactin tumor cells.
  • We transplanted, in rats, DES-induced prolactinoma cells into the adrenal medulla or under the renal capsule, two tissues rich and poor in catecholaminergic innervation, respectively.
  • METHODS: Prolactinoma was dose-dependently induced in ovariectomized female rats implanted with 10 and 20 mg DES, and tumor cells taken from prolactinoma induced by 20 mg DES were either transplanted under the renal capsule or into the adrenal medulla.
  • RESULTS: Although the adrenal medulla, with its high dopamine content to inhibit prolactin secretion, was devoid of any tumoral development, a significant tumoral development was evident under the renal capsule, seemingly because of no inhibitory control over prolactin secretion coexisting with the dopamine deficiency of the tissue.
  • [MeSH-major] Adrenal Medulla / pathology. Catecholamines / physiology. Kidney Cortex / pathology. Pituitary Neoplasms / pathology. Prolactinoma / pathology
  • [MeSH-minor] Animals. Carcinogens / administration & dosage. Diethylstilbestrol / administration & dosage. Dose-Response Relationship, Drug. Female. Neoplasm Transplantation. Rats

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  • (PMID = 16256840.001).
  • [ISSN] 0090-3019
  • [Journal-full-title] Surgical neurology
  • [ISO-abbreviation] Surg Neurol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Carcinogens; 0 / Catecholamines; 731DCA35BT / Diethylstilbestrol
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3. Opocher G, Schiavi F, Cicala MV, Patalano A, Mariniello B, Boaretto F, Zovato S, Pignataro V, Macino B, Negro I, Mantero F: Genetics of adrenal tumors. Minerva Endocrinol; 2009 Jun;34(2):107-21
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  • [Title] Genetics of adrenal tumors.
  • Endocrinology pioneered the development of molecular medicine, but also the study of adrenal tumors had a great impact in this field.
  • Particularly important was the detection of genetics of tumors derived from the adrenal medulla, as well as that of those derived from the sympathetic and parasympathetic paraganglia.
  • Less well understood is the genetics of adrenal cortex tumors, in particular adrenocortical carcinoma, a rare and particularly aggressive disease.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Biomarkers, Tumor / genetics. Mutation. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Cortex Neoplasms / genetics. Adrenocortical Carcinoma / genetics. Genetic Predisposition to Disease. Genomics. Humans. Neoplasm Proteins / genetics. Paraganglioma / genetics

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  • (PMID = 19471236.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Neoplasm Proteins
  • [Number-of-references] 81
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4. Mobine HR, Baker AB, Wang L, Wakimoto H, Jacobsen KC, Seidman CE, Seidman JG, Edelman ER: Pheochromocytoma-induced cardiomyopathy is modulated by the synergistic effects of cell-secreted factors. Circ Heart Fail; 2009 Mar;2(2):121-8
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  • BACKGROUND: Pheochromocytomas are rare tumors derived from the chromaffin cells of the adrenal medulla.

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  • (PMID = 19808327.001).
  • [ISSN] 1941-3297
  • [Journal-full-title] Circulation. Heart failure
  • [ISO-abbreviation] Circ Heart Fail
  • [Language] ENG
  • [Grant] United States / NHLBI NIH HHS / HL / HL083935-01A1; United States / NHLBI NIH HHS / HL / R01 HL084553; United States / NHLBI NIH HHS / HL / F31 HL083935-01A1; United States / PHS HHS / / R01 49039
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Catecholamines; X4W3ENH1CV / Norepinephrine
  • [Other-IDs] NLM/ NIHMS129904; NLM/ PMC2769512
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5. Saikali S, Paumier V, Garrelon JL, Le Gall F: [Facial primary cutaneous ganglioneuroma]. Ann Pathol; 2009 Apr;29(2):138-41
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  • [Transliterated title] Ganglioneurome cutané primitif de la face.
  • Ganglioneuroma is a benign neoplasm of the sympathetic nervous system most often arising in the posterior mediastinum, retroperitoneum, adrenal medulla and pelvis.

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  • (PMID = 19364589.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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6. Adler JT, Meyer-Rochow GY, Chen H, Benn DE, Robinson BG, Sippel RS, Sidhu SB: Pheochromocytoma: current approaches and future directions. Oncologist; 2008 Jul;13(7):779-93
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  • Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites.
  • In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease.
  • [MeSH-major] Adrenal Gland Neoplasms. Pheochromocytoma
  • [MeSH-minor] Adrenal Glands / surgery. Chemotherapy, Adjuvant. Genetic Predisposition to Disease. Humans. Neoplasm Metastasis / prevention & control. Radiotherapy, Adjuvant


7. Cadden IS, Atkinson AB, Johnston BT, Pogue K, Connolly R, McCance D, Ardill JE, Russell CF, McGinty A: Cyclooxygenase-2 expression correlates with phaeochromocytoma malignancy: evidence for a Bcl-2-dependent mechanism. Histopathology; 2007 Dec;51(6):743-51
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  • AIMS: Phaeochromocytomas are rare but potentially life-threatening neuroendocrine tumours of the adrenal medulla or sympathetic nervous system ganglia.
  • METHODS AND RESULTS: COX-2 and Bcl-2 expression were examined immunohistochemically in tissue from 41 sporadic phaeochromocytoma patients followed up for a minimum of 5 years after diagnosis.
  • There was a statistically significant association between COX-2 histoscore (intensity x proportion) and the development of tumour recurrence or metastases (P = 0.006).
  • A significant relationship was observed between coexpression of COX-2 and Bcl-2 in the primary tumour and the presence of recurrent disease (P = 0.034).
  • A highly significant association was observed between (i) tumour-associated expression of these two oncoproteins (P = 0.001) and (ii) COX-2 histoscore and the presence of Bcl-2 expression (P = 0.002).
  • CONCLUSIONS: COX-2 and Bcl-2 may promote phaeochromocytoma malignancy, and these oncoproteins may be valuable surrogate markers of an aggressive tumour phenotype.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Biomarkers, Tumor / analysis. Cyclooxygenase 2 / biosynthesis. Pheochromocytoma / metabolism. Proto-Oncogene Proteins c-bcl-2 / biosynthesis
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Humans. Immunohistochemistry. Kaplan-Meier Estimate. Male. Middle Aged. Neoplasm Recurrence, Local

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  • (PMID = 17916073.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Proto-Oncogene Proteins c-bcl-2; EC 1.14.99.1 / Cyclooxygenase 2
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8. Flores-Hernández SS, Ahumada Mendoza H, Santana-Montero BL, González Flores Mde L: [Early diagnosis of a newborn with a mediastinal mass]. Gac Med Mex; 2005 Nov-Dec;141(6):535-8
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  • [Title] [Early diagnosis of a newborn with a mediastinal mass].
  • Neuroblastoma is an embryonal tumour that evolves from the neural crest cell.
  • This neoplasm may arise at any site in the sympathetic nervous system, including the brain, the cervical region, the posterior mediastinum, the para-aortic sympathetic ganglia, the pelvis, and the adrenal medulla.
  • The clinical presentation in neonatal age is rare and the differential diagnosis includes congenital lung malformations, pneumoniae, atelectasia, etc.
  • This case illustrates how a patient with an X-ray image compatible with a thoracic tumor should be studied.
  • [MeSH-major] Mediastinal Neoplasms / diagnosis. Neuroblastoma / diagnosis
  • [MeSH-minor] Early Diagnosis. Female. Humans. Infant, Newborn

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  • (PMID = 16381510.001).
  • [ISSN] 0016-3813
  • [Journal-full-title] Gaceta médica de México
  • [ISO-abbreviation] Gac Med Mex
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Mexico
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9. Niederhuber JE, Fojo T: Treatment of metastatic disease in patients with neuroendocrine tumors. Surg Oncol Clin N Am; 2006 Jul;15(3):511-33, viii
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  • Their predominant site of origin is the gastrointestinal tract, where most involve the small intestine and appendix, but are also found in the adrenal medulla, bronchopulmonary system, pancreas, thyroid, parathyroid, and paraganglia cells.
  • [MeSH-minor] Algorithms. Antibiotics, Antineoplastic / administration & dosage. Antineoplastic Agents / administration & dosage. Chemoembolization, Therapeutic. Diagnostic Imaging. Hepatic Artery. Humans. Neoplasm Staging. Positron-Emission Tomography. Prognosis. Receptors, Somatostatin. Sensitivity and Specificity. Somatostatin / analogs & derivatives. Streptozocin / administration & dosage

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  • (PMID = 16882495.001).
  • [ISSN] 1055-3207
  • [Journal-full-title] Surgical oncology clinics of North America
  • [ISO-abbreviation] Surg. Oncol. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents; 0 / Receptors, Somatostatin; 51110-01-1 / Somatostatin; 5W494URQ81 / Streptozocin
  • [Number-of-references] 87
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10. Bronzino P, Abbo L, Barisone P, Dezzani C, Genovese AM, Iannucci P, Ippoliti M, Sacchi M, Aimo I: [Acute abdomen from ruptured adrenal pheochromocytoma: case report]. G Chir; 2005 Jan-Feb;26(1-2):25-8
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  • [Title] [Acute abdomen from ruptured adrenal pheochromocytoma: case report].
  • The pheochromocytoma is a very rare neoplasm, which originates in 98% of cases in the adrenal medulla; it is often bilateral in familial syndromes.
  • In this article the Authors report a case of a young woman with a large adrenal pheochromocytoma, that presented by an acute abdomen; the treatment was explorative laparotomy with unilateral adrenalectomy.
  • Therapy of this tumour is founded on surgery, plus chemiotherapy radiotherapy or treatment with 131I-MIBG (iodine-131-metaiodobenzylguanidine in malignant cases (10%).
  • [MeSH-major] Abdomen, Acute / etiology. Adrenal Gland Neoplasms / complications. Pheochromocytoma / complications


11. Namour F, Ayav A, Lu X, Klein M, Muresan M, Bresler L, Tramoy D, Guéant JL, Brunaud L: Lack of association between microsatellite instability and benign adrenal tumors. World J Surg; 2006 Jul;30(7):1240-6
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  • [Title] Lack of association between microsatellite instability and benign adrenal tumors.
  • BACKGROUND: The adrenal gland may give rise to pheochromocytomas, which are catecholamine-producing tumors originating from the adrenal medulla, or to adrenocortical tumors, which derive from the adrenocortical cortex and may be secreting or not.
  • AIM: The aim of this study was to investigate a third genetic mechanism by evaluating microsatellite instability using the reference markers (Bat25, Bat26, D2S123, D5S346, D17S250) validated by the National Cancer Institute.
  • No microsatellite instability was detected in any tumor.
  • A second patient with a MEN-2A syndrome and a two-sided pheochromocytoma exhibited a loss of heterozygosity for D2S123 in the right tumor only and a retention of heterozygosity for all markers in the left tumor.
  • CONCLUSIONS: These results suggest that microsatellite instability, evaluated by the five reference markers of the National Cancer Institute, is not a feature of benign adrenal tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Microsatellite Repeats / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Adult. Aged. Alleles. DNA, Neoplasm / analysis. Female. Humans. Loss of Heterozygosity. Male. Middle Aged. Polymerase Chain Reaction. Proto-Oncogene Proteins / genetics

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  • (PMID = 16715450.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Proto-Oncogene Proteins
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12. Klaunig JE, Kamendulis LM: Mechanisms of acrylamide induced rodent carcinogenesis. Adv Exp Med Biol; 2005;561:49-62
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  • It has been established that following chronic exposure, rats exhibited an increase in the incidence of adrenal pheochromocytomas, testicular mesotheliomas, thyroid adenomas and mammary neoplasms in F344 rats.
  • Acrylamide increased DNA synthesis in the target tissues (thyroid, testicular mesothelium, adrenal medulla) at all doses and time points examined.
  • In summary, acrylamide caused both an increase in DNA synthesis and DNA damage in mammalian tissues and cells suggesting that DNA reactivity and cell proliferation, in concert, may contribute to the observed acrylamide-induced carcinogenicity in the rat and has implication on the possible risk for human neoplasm development.

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  • (PMID = 16438288.001).
  • [ISSN] 0065-2598
  • [Journal-full-title] Advances in experimental medicine and biology
  • [ISO-abbreviation] Adv. Exp. Med. Biol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Carcinogens; 0 / DNA Adducts; 0 / Fluorescent Dyes; 20R035KLCI / Acrylamide; 9007-49-2 / DNA; G34N38R2N1 / Bromodeoxyuridine; GAN16C9B8O / Glutathione
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13. Vezzalini M, Mombello A, Menestrina F, Mafficini A, Della Peruta M, van Niekerk C, Barbareschi M, Scarpa A, Sorio C: Expression of transmembrane protein tyrosine phosphatase gamma (PTPgamma) in normal and neoplastic human tissues. Histopathology; 2007 Apr;50(5):615-28
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  • METHODS AND RESULTS: Membranous and/or cytoplasmic PTPgamma expression was detected in the majority of epithelial cell types and in endocrine cells, with the highest expression in adrenal medulla, endocrine cells of the gastrointestinal tract and pancreatic islets.
  • CONCLUSIONS: We have analysed PTPgamma expression in archival paraffin-embedded tissues for the first time, demonstrating particularly high expression in endocrine cells and both down- and up-regulation in neoplasia, the latter possibly reflecting the undifferentiated state of the neoplastic cells, suggesting a complex role for this phosphatase.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Neoplasms / enzymology. Nerve Tissue Proteins / metabolism. Protein Tyrosine Phosphatases / metabolism. Receptors, Cell Surface / metabolism
  • [MeSH-minor] Down-Regulation. Endocrine System / cytology. Endocrine System / enzymology. Female. Fluorescent Antibody Technique, Indirect. Gene Expression Regulation, Neoplastic. Humans. Immunoenzyme Techniques. In Situ Hybridization. Male. RNA, Messenger / metabolism. RNA, Neoplasm / analysis. Receptor-Like Protein Tyrosine Phosphatases, Class 5. Up-Regulation

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  • (PMID = 17394498.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Nerve Tissue Proteins; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / Receptors, Cell Surface; EC 3.1.3.48 / PTPRG protein, human; EC 3.1.3.48 / Protein Tyrosine Phosphatases; EC 3.1.3.48 / Receptor-Like Protein Tyrosine Phosphatases, Class 5
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14. Waldmann J, Fendrich V, Holler J, Buchholz M, Heinmöller E, Langer P, Ramaswamy A, Samans B, Walz MK, Rothmund M, Bartsch DK, Slater EP: Microarray analysis reveals differential expression of benign and malignant pheochromocytoma. Endocr Relat Cancer; 2010 Sep;17(3):743-56
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  • The diagnosis of a malignant pheochromocytoma (PC) can only be established by the presence of distant metastases, but a subset of apparently benign PCs develop metastases.
  • The reference consisted of laser microdissected tissue from normal adrenal medulla.
  • Comparative analysis by microarray of all ten PCs (benign/malignant) versus normal adrenal medulla revealed a more than twofold expression difference in 455/539 and 491/671 genes respectively.
  • Several of these genes are known to participate on adrenal tumorigenesis, potential tumor suppressor genes, and oncogenes.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Medulla / metabolism. Biomarkers, Tumor / metabolism. Gene Expression Profiling. Pheochromocytoma / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Case-Control Studies. Humans. Immunoenzyme Techniques. Middle Aged. Neoplasm Metastasis. Oligonucleotide Array Sequence Analysis. Prognosis. RNA, Messenger / genetics. Reverse Transcriptase Polymerase Chain Reaction. Young Adult


15. Kremens B: [Systemic therapy in children and adolescents]. Urologe A; 2007 Oct;46(10):1404-6
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  • National and supranational treatment studies are the standard of care for pediatric cancer in Germany; they yield 5-year survival rates of almost 90% for nephroblastoma and germ cell tumors and 60% for neuroblastoma (all stages) and rhabdomyosarcoma.
  • The principles of antineoplastic therapy are the same as in adult cancer medicine; the drugs used depend upon the disease.
  • [MeSH-minor] Adolescent. Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / mortality. Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Adrenal Medulla. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Humans. Infant. Kidney Neoplasms / drug therapy. Kidney Neoplasms / mortality. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Neoplasm Staging. Neoplasms, Germ Cell and Embryonal / drug therapy. Neoplasms, Germ Cell and Embryonal / mortality. Neoplasms, Germ Cell and Embryonal / pathology. Neoplasms, Germ Cell and Embryonal / surgery. Neuroblastoma / drug therapy. Neuroblastoma / mortality. Neuroblastoma / pathology. Neuroblastoma / surgery. Prognosis. Radiotherapy, Adjuvant. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / mortality. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / surgery. Survival Rate. Wilms Tumor / drug therapy. Wilms Tumor / mortality. Wilms Tumor / pathology. Wilms Tumor / surgery

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  • (PMID = 17823786.001).
  • [ISSN] 0340-2592
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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16. Minn AJ, Kang Y, Serganova I, Gupta GP, Giri DD, Doubrovin M, Ponomarev V, Gerald WL, Blasberg R, Massagué J: Distinct organ-specific metastatic potential of individual breast cancer cells and primary tumors. J Clin Invest; 2005 Jan;115(1):44-55
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  • [Title] Distinct organ-specific metastatic potential of individual breast cancer cells and primary tumors.
  • We used bioluminescence imaging to reveal patterns of metastasis formation by human breast cancer cells in immunodeficient mice.
  • Individual cells from a population established in culture from the pleural effusion of a breast cancer patient showed distinct patterns of organ-specific metastasis.
  • Single-cell progenies derived from this population exhibited markedly different abilities to metastasize to the bone, lung, or adrenal medulla, which suggests that metastases to different organs have different requirements.
  • Unsupervised classification using the transcriptomic data set supported the hypothesis that organ-specific metastasis by breast cancer cells is controlled by metastasis-specific genes that are separate from a general poor-prognosis gene expression signature.

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  • (PMID = 15630443.001).
  • [ISSN] 0021-9738
  • [Journal-full-title] The Journal of clinical investigation
  • [ISO-abbreviation] J. Clin. Invest.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA094060; United States / NIGMS NIH HHS / GM / T32 GM007739; United States / NIGMS NIH HHS / GM / GM07739; United States / NCI NIH HHS / CA / P01-CA94060
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC539194
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17. Niveditha SR, Suguna BV, Krishnamurthy, Rajnikanth, Babu KG, Shukla AK: Cytologic features of malignant cystic pheochromocytoma: a case report. Acta Cytol; 2007 Mar-Apr;51(2):200-2
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  • BACKGROUND: Image-guided fine needle aspiration (FNA) biopsy of deep-seated lesions (e.g., retroperitoneal) has aided preoperative diagnosis and treatment planning.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Medulla / pathology. Carcinoma, Renal Cell / diagnosis. Cysts / pathology. Paraganglioma / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] Biopsy, Fine-Needle. Diagnosis, Differential. Duodenum / pathology. Fatal Outcome. Humans. Hypertension / etiology. Kidney / pathology. Male. Middle Aged. Neoplasm Invasiveness. Predictive Value of Tests


18. Korpershoek E, Loonen AJ, Corvers S, van Nederveen FH, Jonkers J, Ma X, Ziel-van der Made A, Korsten H, Trapman J, Dinjens WN, de Krijger RR: Conditional Pten knock-out mice: a model for metastatic phaeochromocytoma. J Pathol; 2009 Mar;217(4):597-604
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  • Phaeochromocytomas (PCCs) are neuro-endocrine tumours of the adrenal medulla that are usually benign, but approximately 10% of patients develop metastases.
  • Here we describe adrenal tumours generated in a Pten conditional knock-out (KO) mouse model.
  • Thirty-two of 41 (78%) male Psa-Cre;Pten-loxP/loxP mice presented adrenal tumours that were shown to be PCC by histology and by immunohistochemical staining for enzymes in the catecholamine biosynthetic pathway.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Disease Models, Animal. Lung Neoplasms / secondary. Neoplasm Proteins / genetics. PTEN Phosphohydrolase / genetics. Pheochromocytoma / secondary


19. Luo Z, Li J, Qin Y, Ma Y, Liang X, Xian J, Lu D, Wei M, Yang JY, Yang MQ, He Z: Differential expression of human telomerase catalytic subunit mRNA by in situ hybridization in pheochromocytomas. Endocr Pathol; 2006;17(4):387-98
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  • While no statistical difference in p27kip1 expressions was observed among benign, malignant, and suspected malignant tumors, there was a statistical difference between the normal adrenal medulla samples and tumors (p < 0.001).
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics. RNA, Messenger / metabolism. Telomerase / genetics
  • [MeSH-minor] Adolescent. Adrenal Medulla / metabolism. Adrenal Medulla / pathology. Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Cyclin-Dependent Kinase Inhibitor p27 / metabolism. Female. Follow-Up Studies. Humans. In Situ Hybridization. Ki-67 Antigen / metabolism. Male. Middle Aged. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism

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  • (PMID = 17525487.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Neoplasm Proteins; 0 / RNA, Messenger; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27; EC 2.7.7.49 / Telomerase
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20. Khorram-Manesh A, Ahlman H, Nilsson O, Friberg P, Odén A, Stenström G, Hansson G, Stenquist O, Wängberg B, Tisell LE, Jansson S: Long-term outcome of a large series of patients surgically treated for pheochromocytoma. J Intern Med; 2005 Jul;258(1):55-66
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  • DESIGN: Retrospective review of patients with PC/PG regarding presenting symptoms, tumour characteristics, clinical management and long-term outcome after treatment.
  • At diagnosis 85% of the patients were hypertensive; one year after surgery more than half were still hypertensive.
  • We recommend life-long follow-up of patients treated for PC/PG with screening for recurrent tumour in sporadic cases and for associated tumours in hereditary cases.
  • [MeSH-major] Adrenal Gland Neoplasms / surgery. Pheochromocytoma / surgery
  • [MeSH-minor] Adrenal Medulla / pathology. Adult. Aged. Blood Pressure / physiology. Female. Humans. Hyperplasia. Hypertension / complications. Male. Middle Aged. Neoplasm Invasiveness. Paraganglioma / mortality. Paraganglioma / pathology. Paraganglioma / surgery. Postoperative Period. Preoperative Care / methods. Receptors, Adrenergic, alpha / administration & dosage. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 15953133.001).
  • [ISSN] 0954-6820
  • [Journal-full-title] Journal of internal medicine
  • [ISO-abbreviation] J. Intern. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Receptors, Adrenergic, alpha
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21. Korpershoek E, Petri BJ, van Nederveen FH, Dinjens WN, Verhofstad AA, de Herder WW, Schmid S, Perren A, Komminoth P, de Krijger RR: Candidate gene mutation analysis in bilateral adrenal pheochromocytoma and sympathetic paraganglioma. Endocr Relat Cancer; 2007 Jun;14(2):453-62
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  • [Title] Candidate gene mutation analysis in bilateral adrenal pheochromocytoma and sympathetic paraganglioma.
  • Pheochromocytomas (PCCs) are rare tumors that arise from chromaffin tissue in the adrenal medulla, but can also occur in the abdomen outside the adrenals and are then called sympathetic paragangliomas (sPGLs).
  • According to the literature, between 15 and 25% of apparently sporadic adrenal PCC and sPGL are caused by germline mutations in RET, von Hippel-Lindau disease (VHL), succinate dehydrogenase subunit B (SDHB), or subunit D SDHD.
  • However, few studies have addressed the mutationfrequency of these candidate genes in selected subgroups of PCC andsPGL, such as bilateral adrenal PCC or extra-adrenal sPGL, and none have looked at somatic mutations by analyzing tumor tissue.
  • Therefore, we have investigated the occurrence of germline and somatic mutations in RET, VHL, SDHB, and SDHD in comparatively large series of bilateral adrenal PCC (n = 33 patients) and sPGL (n = 26 patients), with the aim of determining the mutation frequency of each of these genes and to establish a genetic testing algorithm.
  • Twenty-one RET, two VHL germline, and one SDHD mutations were found in the patients with bilateral adrenal PCC.
  • We suggest that sequential mutation analysis should be directed first at RET, followed by VHL and SDHD for patients with bilateral adrenal PCC at diagnosis, and at SDHB and SDHD for patients with sPGL.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Genes, Neoplasm. Paraganglioma / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Adult. Aged. Amino Acid Sequence. Animals. Cattle. DNA Mutational Analysis. Female. Germ-Line Mutation. Humans. Iron-Sulfur Proteins / genetics. Male. Mice. Middle Aged. Molecular Sequence Data. Mutation. Proto-Oncogene Proteins c-ret / genetics. Rats. Succinate Dehydrogenase / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 17639058.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Iron-Sulfur Proteins; 0 / SDHD protein, human; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human; EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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22. Berthon A, Sahut-Barnola I, Lambert-Langlais S, de Joussineau C, Damon-Soubeyrand C, Louiset E, Taketo MM, Tissier F, Bertherat J, Lefrançois-Martinez AM, Martinez A, Val P: Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development. Hum Mol Genet; 2010 Apr 15;19(8):1561-76
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  • [Title] Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development.
  • Adrenocortical carcinoma is a rare but aggressive cancer with unknown aetiology.
  • Here, we show that constitutive activation of beta-catenin in the adrenal cortex of transgenic mice resulted in progressive steroidogenic and undifferentiated spindle-shaped cells hyperplasia as well as dysplasia of the cortex and medulla.
  • Altogether these observations demonstrate that constitutively active beta-catenin is an adrenal oncogene which triggers benign aldosterone-secreting tumour development and promotes malignancy.
  • [MeSH-major] Adrenal Cortex / pathology. Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / pathology. beta Catenin / metabolism
  • [MeSH-minor] Aldosterone / metabolism. Animals. Cell Proliferation. Disease Models, Animal. Humans. Hyperplasia. Mice. Mice, Inbred C57BL. Mice, Transgenic. Neoplasm Metastasis


23. Elston MS, Benn D, Robinson BG, Conaglen JV: An apparently sporadic paraganglioma with an SDHB gene germline mutation presenting at age 68 years. Intern Med J; 2006 Feb;36(2):129-31
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  • Paragangliomas (PGLs) are rare tumours arising from parasympathetic-associated paraganglia (particularly of the head and neck) or from sympathetic-associated paraganglia such as in the adrenal medulla when they are termed phaeochromocytomas and at extra-adrenal sites in the abdomen and thorax.
  • [MeSH-major] Abdominal Neoplasms / genetics. DNA, Neoplasm / genetics. Germ-Line Mutation. Iron-Sulfur Proteins / genetics. Paraganglioma, Extra-Adrenal / genetics. Protein Subunits / genetics. Succinate Dehydrogenase / genetics

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  • (PMID = 16472267.001).
  • [ISSN] 1444-0903
  • [Journal-full-title] Internal medicine journal
  • [ISO-abbreviation] Intern Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Iron-Sulfur Proteins; 0 / Protein Subunits; EC 1.3.5.1 / SDHB protein, human; EC 1.3.99.1 / Succinate Dehydrogenase
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