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1. Allen AT, Dress AF, Moore WF: Mirror syndrome resulting from metastatic congenital neuroblastoma. Int J Gynecol Pathol; 2007 Jul;26(3):310-2
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  • Neuroblastoma is a tumor of the sympathetic ganglia and adrenal medulla that rarely metastasizes to the placenta.

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  • (PMID = 17581417.001).
  • [ISSN] 0277-1691
  • [Journal-full-title] International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists
  • [ISO-abbreviation] Int. J. Gynecol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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2. McNicol AM: Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas. Endocr Pathol; 2006;17(4):329-36
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  • [Title] Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas.
  • The term pheochromocytoma is reserved for intra-adrenal tumors.
  • This short review discusses the gross and microscopic features, the immunohistochemical profile, the problem of recognizing malignant potential, and the rare instances where a differential diagnosis has to be considered.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Medulla / pathology. Paraganglioma, Extra-Adrenal / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Biomarkers, Tumor / analysis. Chromaffin Cells / chemistry. Chromaffin Cells / pathology. Humans. Hyperplasia. Immunohistochemistry / methods

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  • (PMID = 17525481.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 46
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3. Fritzsche FR, Bode PK, Koch S, Frauenfelder T: Radiological and pathological findings of a metastatic composite paraganglioma with neuroblastoma in a man: a case report. J Med Case Rep; 2010;4:374
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  • INTRODUCTION: Composite tumors of the adrenal medulla or paraganglia are extremely rare and present a diagnostic dilemma.
  • These tumors consist of a neuroendocrine component mixed with a neural component.We describe the imaging characteristics together with the corresponding pathological findings of a composite tumor.
  • An abdominal computed tomography scan and ultrasound examination detected a retroperitoneal tumor comprising two different tumor components.
  • Twenty-four-hour urine revealed high levels of normetanephrine, characteristic of a neuroendocrine tumor.
  • Our patient died ten months after the initial diagnosis from tumor-associated complications.
  • Such tumors should be considered in the differential diagnosis of retroperitoneal masses.

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  • (PMID = 21092109.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2997098
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4. Xia X, Pan Y, Zhang WY, Cheng G, Kong LD: Ethanolic extracts from Curcuma longa attenuates behavioral, immune, and neuroendocrine alterations in a rat chronic mild stress model. Biol Pharm Bull; 2006 May;29(5):938-44
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  • Male Sprague-Dawley rats subjected to the CMS procedure demonstrated increased serum interleukin-6 and tumor necrosis factor-alpha levels, as well as a reduction of natural killer cell activity in splenocytes.
  • In addition, CMS-treated rats exhibited elevated corticotropin-releasing factor in serum and medulla oblongata and cortisol levels in serum, with no significant change in serum adrenocorticotropin hormone levels.
  • These findings indicate that the alterations in immune and hypothalamic-pituitary-adrenal (HPA) axis systems could participate in the behavioral response to the CMS procedure in animals.
  • [MeSH-minor] Adrenocorticotropic Hormone / blood. Animals. Chronic Disease. Corticotropin-Releasing Hormone / blood. Corticotropin-Releasing Hormone / metabolism. Depression / drug therapy. Depression / psychology. Ethanol. Hydrocortisone / blood. Hypothalamo-Hypophyseal System / drug effects. Interleukin-6 / blood. Killer Cells, Natural / drug effects. Male. Medulla Oblongata / metabolism. Plant Extracts / pharmacology. Rats. Rats, Sprague-Dawley. Solvents. Spleen / cytology. Tumor Necrosis Factor-alpha / metabolism

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  • (PMID = 16651723.001).
  • [ISSN] 0918-6158
  • [Journal-full-title] Biological & pharmaceutical bulletin
  • [ISO-abbreviation] Biol. Pharm. Bull.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Interleukin-6; 0 / Plant Extracts; 0 / Solvents; 0 / Tumor Necrosis Factor-alpha; 3K9958V90M / Ethanol; 9002-60-2 / Adrenocorticotropic Hormone; 9015-71-8 / Corticotropin-Releasing Hormone; WI4X0X7BPJ / Hydrocortisone
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5. 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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6. Opocher G, Schiavi F: Genetics of pheochromocytomas and paragangliomas. Best Pract Res Clin Endocrinol Metab; 2010 Dec;24(6):943-56
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  • Pheochromocytoma is the tumor of the main sympathetic paraganglia, which is the adrenal medulla.
  • Both of them originate from neural crest cells and share similar mechanisms of tumor development.
  • The best known hereditary forms of pheochromocytoma and paraganglioma are the von Hippel-Lindau disease, in which pheochromocytoma may be associated with CNS hemangioblastoma, retinal angioma, pancreatic endocrine tumor/cysts and renal clear cell carcinoma/cysts; the multiple endocrine neoplasia type 2, in which pheochromocytoma is associated with medullary thyroid carcinoma and primary hyperparathyroidism, Type 1 neurofibromatosis, the most frequent hereditary cancer syndrome.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics


7. Plouin PF, Gimenez-Roqueplo AP: Pheochromocytomas and secreting paragangliomas. Orphanet J Rare Dis; 2006;1:49
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  • Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas) or in extraadrenal chromaffin cells (secreting paragangliomas).
  • Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass.
  • These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease.
  • The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines.
  • The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy.
  • Treatment requires resection of the tumor, generally by laparoscopic surgery.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Paraganglioma, Extra-Adrenal / diagnosis. Paraganglioma, Extra-Adrenal / secretion. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy
  • [MeSH-minor] Adrenal Gland Diseases / diagnosis. Catecholamines / biosynthesis. Catecholamines / secretion. Diagnosis, Differential. Genetic Testing / methods. Humans. Hypertension / etiology. Prognosis. Proto-Oncogene Proteins c-ret / genetics

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  • (PMID = 17156452.001).
  • [ISSN] 1750-1172
  • [Journal-full-title] Orphanet journal of rare diseases
  • [ISO-abbreviation] Orphanet J Rare Dis
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines; EC 2.7.10.1 / Proto-Oncogene Proteins c-ret; EC 2.7.10.1 / RET protein, human
  • [Number-of-references] 29
  • [Other-IDs] NLM/ PMC1702343
  • [General-notes] NLM/ Original DateCompleted: 20070718
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8. Morimoto R, Satoh F, Murakami O, Hirose T, Totsune K, Imai Y, Arai Y, Suzuki T, Sasano H, Ito S, Takahashi K: Expression of adrenomedullin 2/intermedin in human adrenal tumors and attached non-neoplastic adrenal tissues. J Endocrinol; 2008 Jul;198(1):175-83
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  • [Title] Expression of adrenomedullin 2/intermedin in human adrenal tumors and attached non-neoplastic adrenal tissues.
  • AM is expressed in various tumors including adrenocortical tumors and modulates tumor growth.
  • The AM2/IMD expression has not been studied, however, in adrenal tumors.
  • The expression of AM2/IMD and AM was therefore studied in human adrenal tumors and attached non-neoplastic adrenal tissues by immunocytochemistry (ICC).
  • AM2/IMD and AM immunoreactivities were detected in medulla of attached non-neoplastic tissues, while the degree of immunoreactivity for AM2/IMD and AM in cortices of attached adrenals was relatively weak or undetectable.
  • RIA detected IR-AM2/IMD in adrenal tumors (0.414+/-0.12 to 0.786+/-0.27 pmol/g wet weight, mean+/-S.E.M.) and attached adrenal tissues (0.397+/-0.052 pmol/g wet weight).
  • RT-PCR showed expression of AM2/IMD, CRLR, and RAMP1, RAMP2, and RAMP3 mRNA in tissues of adrenal tumors and attached adrenal glands.
  • In conclusion, AM2/IMD is expressed in human adrenal tumors and attached non-neoplastic adrenal tissues and may play (patho-)physiological roles in normal and neoplastic adrenals as an autocrine/paracrine regulator.
  • [MeSH-major] Adrenal Gland Neoplasms / chemistry. Adrenal Glands / chemistry. Peptide Hormones / analysis

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  • (PMID = 18460550.001).
  • [ISSN] 1479-6805
  • [Journal-full-title] The Journal of endocrinology
  • [ISO-abbreviation] J. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / ADM2 protein, human; 0 / Peptide Hormones; 0 / RNA, Messenger
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9. Korpershoek E, Van Nederveen FH, Dannenberg H, Petri BJ, Komminoth P, Perren A, Lenders JW, Verhofstad AA, De Herder WW, De Krijger RR, Dinjens WN: Genetic analyses of apparently sporadic pheochromocytomas: the Rotterdam experience. Ann N Y Acad Sci; 2006 Aug;1073:138-48
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  • They are usually located in the adrenal medulla, although in about 10% the tumors arise from extra-adrenal chromaffin tissue.
  • The majority of PCCs arise sporadically, but PCCs occur also in the context of hereditary cancer syndromes.
  • Familial PCC is inherited as an autosomal dominant trait alone or as a component of the multiple endocrine neoplasia Type 2 (MEN2) syndrome (RET gene), Von Hippel-Lindau (VHL) disease (VHL gene), neurofibromatosis Type 1 (NF1 gene), or familial pheochromocytoma-paraganglioma (PCC-PGL) syndrome (SDHD/B and C genes).
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Genetic Predisposition to Disease. Germ-Line Mutation. Humans. Polymerase Chain Reaction. Proto-Oncogene Proteins c-ret / genetics. Succinate Dehydrogenase / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics

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  • (PMID = 17102080.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 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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10. Hammer GD, Parker KL, Schimmer BP: Minireview: transcriptional regulation of adrenocortical development. Endocrinology; 2005 Mar;146(3):1018-24
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  • The adrenal glands are comprised of two distinct endocrine organs: the outer cortex, which is derived from mesoderm and synthesizes steroid hormones, and the inner medulla, which contains neuroectodermal cells derived from the neural crest and produces the catecholamine hormones norepinephrine and epinephrine.
  • The developmental program that gives rise to the adrenal gland begins early during embryogenesis and continues throughout gestation and well after birth.
  • In this article, we review the molecular mechanisms of adrenal differentiation and development, focusing on the contributions of genes responsible for the development of the adrenal cortex as identified from studies of experimental animal models and human subjects with clinical diseases.
  • These studies identify a hierarchical network of transcription factors, including Wilms' tumor-1, steroidogenic factor-1, dosage-sensitive sex reversal, adrenal hypoplasia congenita, X-linked-1, PBX1, and CITED2, that both give rise to the adrenal cortex and subsequently determine its subsequent function in steroidogenesis.
  • [MeSH-major] Adrenal Cortex / embryology. Adrenal Cortex / physiology. Adrenal Glands / embryology. Gene Expression Regulation, Developmental. Transcription, Genetic

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  • (PMID = 15604207.001).
  • [ISSN] 0013-7227
  • [Journal-full-title] Endocrinology
  • [ISO-abbreviation] Endocrinology
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CITED2 protein, human; 0 / DAX-1 Orphan Nuclear Receptor; 0 / DNA-Binding Proteins; 0 / Homeodomain Proteins; 0 / NR0B1 protein, human; 0 / NR5A1 protein, human; 0 / Nr0b1 protein, mouse; 0 / Proto-Oncogene Proteins; 0 / Receptors, Cytoplasmic and Nuclear; 0 / Receptors, Retinoic Acid; 0 / Repressor Proteins; 0 / Steroidogenic Factor 1; 0 / Steroids; 0 / Trans-Activators; 0 / Transcription Factors; 0 / WT1 Proteins; 0 / pbx1 protein, human; 0 / steroidogenic factor 1, mouse; X4W3ENH1CV / Norepinephrine; YKH834O4BH / Epinephrine
  • [Number-of-references] 71
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11. Nomikos IN, Zizi-Serbetzoglou A, Matsakis G, Elemenoglou J, Vamvakopoulos NC: Association of an oversized adrenal cortical adenoma with expression of pheochromocytoma-like neurosecretory features. J BUON; 2008 Jul-Sep;13(3):425-8
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  • [Title] Association of an oversized adrenal cortical adenoma with expression of pheochromocytoma-like neurosecretory features.
  • Abnormal stimulation of adrenal function may be either direct, affecting similarly cortical and medullary secretion, or indirect affecting primarily the medulla.
  • Indirect activation of clinically detectable adrenomedullary function may develop as a physical consequence of a non-functional adrenal tumor exerting pressure on the medulla by its size, location and direction of growth.
  • Our case of an oversized and overweight adrenal tumor associated with expression of late-onset pheochromocytoma-like clinical symptoms may be explained by the physical indirect rather than the biological direct activation of adrenomedullary function like hyperplasia or cancer.
  • [MeSH-major] Adenoma / pathology. Adrenal Cortex Neoplasms / pathology. Adrenal Medulla / pathology. Pheochromocytoma / pathology

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  • (PMID = 18979561.001).
  • [ISSN] 1107-0625
  • [Journal-full-title] Journal of B.U.ON. : official journal of the Balkan Union of Oncology
  • [ISO-abbreviation] J BUON
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 57285-09-3 / Inhibins
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12. 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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13. Miller AD, Masek-Hammerman K, Dalecki K, Mansfield KG, Westmoreland SV: Histologic and immunohistochemical characterization of pheochromocytoma in 6 cotton-top tamarins (Saguinus oedipus). Vet Pathol; 2009 Nov;46(6):1221-9
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  • Pheochromocytomas are uncommon neoplasms of the adrenal medulla that are most frequently reported in rats and select mouse strains.
  • In many cases, especially those in man, pheochromocytoma is associated with familial tumor syndromes, because of inherited mutations in a variety of proto-oncogenes and tumor suppressor genes.
  • The tumors in this population illustrate comparable histologic and immunohistochemical staining patterns with cases in other laboratory animals and humans, and, therefore, may indicate common underlying genetic alterations that precipitate tumor development.

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  • [Cites] Neuropeptides. 1999 Apr;33(2):159-63 [10657486.001]
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  • (PMID = 19605896.001).
  • [ISSN] 1544-2217
  • [Journal-full-title] Veterinary pathology
  • [ISO-abbreviation] Vet. Pathol.
  • [Language] ENG
  • [Grant] United States / NCRR NIH HHS / RR / RR000168-47; United States / NCRR NIH HHS / RR / T32 RR007000; United States / NCRR NIH HHS / RR / RR00168; United States / NCRR NIH HHS / RR / P51 RR000168; United States / NCRR NIH HHS / RR / K26 RR000168; United States / NCRR NIH HHS / RR / T32 RR007000-32; United States / NCRR NIH HHS / RR / RR007000-32; United States / NCRR NIH HHS / RR / P51 RR000168-47; United States / NCRR NIH HHS / RR / RR07000
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS173473; NLM/ PMC2825153
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14. Okutur K, Küçükler K, Öztekın E, Borlu F, Erdem L, Demır G: A rare cause of acute abdomen: ruptured adrenal pheochromocytoma. Turk J Gastroenterol; 2010 Dec;21(4):467-9
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  • [Title] A rare cause of acute abdomen: ruptured adrenal pheochromocytoma.
  • Pheochromocytoma is a tumor of the chromaffin cells which secretes catecholamines and 90% of it originates from adrenal medulla.
  • At laparotomy, ruptured adrenal mass was seen and excised successfully.
  • The histological evaluation confirmed the diagnosis as pheochromocytoma.
  • Ruptured adrenal pheochromocytoma is a mortal situation potentially and it should be considered in patients who present with an acute abdominal syndrome and hypertension or shock.
  • It should be known that early diagnosis and surgery with proper preoperative treatment is a life saver.
  • [MeSH-major] Abdomen, Acute / etiology. Adrenal Gland Neoplasms / complications. Hypertension / etiology. Pheochromocytoma / complications


15. Rufini V, Shulkin B: The evolution in the use of MIBG in more than 25 years of experimental and clinical applications. Q J Nucl Med Mol Imaging; 2008 Dec;52(4):341-50
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  • Radioiodinated metaiodobenzylguanidine (MIBG), or Iobenguane, was developed in the late 1970s at the Michigan University Medical Center for imaging the adrenal medulla and its diseases, and was rapidly extended to depict a wide range of tumors of neural crest origin.
  • A literature search in PubMed based on ''metaiodobenzylguanidine or MIBG'' was conducted; from this analysis, it appears that the use of MIBG evolved from nearly exclusively oncology (both for diagnosis and therapy) to new applications mainly aimed to study the sympathetic neuronal integrity of the heart.
  • Those currently exceed those about imaging of tumor diseases.
  • [MeSH-minor] Animals. Humans. Literature. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / metabolism. Neuroendocrine Tumors / pathology. Neuroendocrine Tumors / radiotherapy. Radiochemistry. Tissue Distribution

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  • (PMID = 19088689.001).
  • [ISSN] 1824-4785
  • [Journal-full-title] The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of Radiopharmaceutical Chemistry and Biology
  • [ISO-abbreviation] Q J Nucl Med Mol Imaging
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 105
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16. Kimura N, Watanabe T, Noshiro T, Shizawa S, Miura Y: 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. Endocr Pathol; 2005;16(1):23-32
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  • [Title] 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.
  • Pheochromocytomas and extra-adrenal sympathetic paragangliomas show varied histological patterns, and it is difficult to diagnose malignancy or predict the clinical course using current histological criteria.
  • In the present study, we reviewed 146 sympathetic paragangliomas including 116 adrenal (102 unilateral, 14 bilateral) and 30 extra-adrenal tumors including 38 metastatic tumors.
  • The frequency of these tumor types were 113 WD (77%), 27 MD (19%), and 6 PD (4%).
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Medulla / pathology. Paraganglioma, Extra-Adrenal / secondary. Pheochromocytoma / secondary
  • [MeSH-minor] Adult. Biomarkers, Tumor / metabolism. Catecholamines / metabolism. Female. Humans. Immunohistochemistry. Ki-67 Antigen / metabolism. Male. Middle Aged. Prognosis. Survival Rate

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  • (PMID = 16000843.001).
  • [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 / Catecholamines; 0 / Ki-67 Antigen
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17. Woods AM, Judd AM: Interleukin-4 increases cortisol release and decreases adrenal androgen release from bovine adrenal cells. Domest Anim Endocrinol; 2008 May;34(4):372-82
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  • [Title] Interleukin-4 increases cortisol release and decreases adrenal androgen release from bovine adrenal cells.
  • ACTH is the primary regulator of adrenal function during acute stress.
  • However, during chronic inflammatory stress additional factors play a major role in the regulation of adrenal secretion.
  • Many cytokines circulate in the blood and are synthesized and released from adrenal tissue.
  • Furthermore, these peptides modify adrenal function.
  • Recently, interleukin-4 (IL-4) was demonstrated to be released from a human adrenal tumor cell line.
  • Therefore, we hypothesized that normal bovine adrenocortical cells could express IL-4 and that this cytokine may modify adrenal function.
  • We determined that IL-4 and IL-4 receptors (IL-4R) are expressed in the bovine adrenal cortex whereas the expression of IL-4 and IL-4R in the adrenal medulla was not apparent.
  • However, the ACTH-stimulated release of cortisol from the bovine adrenal cells was augmented by IL-4.
  • IL-4 exposure had no affect on adrenal androgen release from bovine zona reticularis cells, but IL-4 inhibited the ACTH-stimulated release of adrenal androgens from these cells.
  • The effects of IL-4 on ACTH-stimulated cortisol and adrenal androgen release were dependent upon the IL-4 incubation interval and the IL-4 concentration.
  • Because communication between the immune and endocrine systems is important in inflammatory conditions, IL-4 may play a role in coordinating the adrenal response to inflammatory stress.
  • [MeSH-major] Adrenal Glands / drug effects. Adrenal Glands / metabolism. Androgens / secretion. Cattle / metabolism. Hydrocortisone / secretion. Interleukin-4 / pharmacology

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  • (PMID = 18055157.001).
  • [ISSN] 0739-7240
  • [Journal-full-title] Domestic animal endocrinology
  • [ISO-abbreviation] Domest. Anim. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Androgens; 0 / Receptors, Interleukin-4; 207137-56-2 / Interleukin-4; WI4X0X7BPJ / Hydrocortisone
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18. Yamaguchi K, Hara I, Takeda M, Tanaka K, Yamada Y, Fujisawa M, Kawabata G: Two cases of ganglioneuroma. Urology; 2006 Mar;67(3):622.e1-4
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  • Although ganglioneuroma is a relatively rare tumor of the sympathetic nervous system, detection of this tumor has increased as imaging techniques such as computed tomography and ultrasonography have become prevalent.
  • Magnetic resonance imaging in 1 patient showed a homogeneous mass with a low signal intensity on T1-weighted imaging and a heterogeneous mass with a high signal intensity on T2-weighted imaging, both characteristic of neurogenic tumor.
  • Histopathologic examination demonstrated that one tumor was located on the adrenal medulla, with the other in the extra-adrenal retroperitoneal space.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Ganglioneuroma / diagnosis. Retroperitoneal Neoplasms / diagnosis

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  • (PMID = 16504264.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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19. van Nederveen FH, Korpershoek E, deLeeuw RJ, Verhofstad AA, Lenders JW, Dinjens WN, Lam WL, de Krijger RR: Array-comparative genomic hybridization in sporadic benign pheochromocytomas. Endocr Relat Cancer; 2009 Jun;16(2):505-13
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  • Pheochromocytomas (PCC) are catecholamine-producing tumors arising from the adrenal medulla that occur either sporadically or in the context of hereditary cancer syndromes, such as multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau disease (VHL), neurofibromatosis type 1, and the PCC-paraganglioma syndrome.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosome Aberrations. Comparative Genomic Hybridization. Oligonucleotide Array Sequence Analysis. Pheochromocytoma / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Chromosomes, Human, Pair 1 / genetics. Chromosomes, Human, Pair 21 / genetics. Chromosomes, Human, Pair 22 / genetics. Chromosomes, Human, Pair 3 / genetics. Female. Humans. Loss of Heterozygosity. Male. Middle Aged. Multiple Endocrine Neoplasia Type 2a / genetics. Mutation / genetics. Von Hippel-Lindau Tumor Suppressor Protein / genetics. Young Adult

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  • (PMID = 19153209.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] EC 6.3.2.19 / VHL protein, human; EC 6.3.2.19 / Von Hippel-Lindau Tumor Suppressor Protein
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20. Herranz Antolín S, Pérez Pelayo M, González Losada T, de Icaya Ortiz de Ubina PM, Fuentes Tudanca S, Del Olmo García D: (123)I-mibg scintigraphy uptake in a hepatic lesion. Endocrinol Nutr; 2008 Mar;55(3):146-8
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  • Pheochromocytoma is a tumor derived from the chromaffin cells of the adrenal medulla.
  • When this type of tumor involves the sympathetic ganglia it is called paraganglioma.
  • Although infrequent, paraganglioma should be considered in the evaluation of hypertension, arrhythmias, and panic disorder.

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  • [Copyright] Copyright © 2008 Sociedad Española de Endocrinología y Nutrición. Published by Elsevier Espana. All rights reserved.
  • (PMID = 22967882.001).
  • [ISSN] 1575-0922
  • [Journal-full-title] Endocrinología y nutrición : órgano de la Sociedad Española de Endocrinología y Nutrición
  • [ISO-abbreviation] Endocrinol Nutr
  • [Language] eng; spa
  • [Publication-type] Journal Article
  • [Publication-country] Spain
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21. Lai EW, Joshi BH, Martiniova L, Dogra R, Fujisawa T, Leland P, de Krijger RR, Lubensky IA, Elkahloun AG, Morris JC, Puri RK, Pacak K: Overexpression of interleukin-13 receptor-alpha2 in neuroendocrine malignant pheochromocytoma: a novel target for receptor directed anti-cancer therapy. J Clin Endocrinol Metab; 2009 Aug;94(8):2952-7
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  • [Title] Overexpression of interleukin-13 receptor-alpha2 in neuroendocrine malignant pheochromocytoma: a novel target for receptor directed anti-cancer therapy.
  • CONTEXT: Pheochromocytomas and paragangliomas are rare catecholamine-secreting neuroendocrine tumors arising from the adrenal medulla and sympathetic tissues.
  • OBJECTIVE: The objective of the study was to identify and characterize overexpression of IL-13 receptor-alpha2 (IL-13Ralpha2) gene expression in human and murine tumors and verify xenograft mouse pheochromocytoma cell (MPC)-derived tumor's response to a selective cytotoxin.
  • INTERVENTION: The function of IL-13Ralpha2 in these tumor cells was examined by evaluating tumor sensitivity to a recombinant IL-13-Pseudomonas exotoxin (IL-13PE).
  • MAIN OUTCOME MEASURES: IC(50) and tumor size were measured.
  • Our results showed a statistically significant decrease in tumor size as early as 3 d after initial treatment and further suppressed growth of MPC tumors.

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  • (PMID = 19491224.001).
  • [ISSN] 1945-7197
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bacterial Proteins; 0 / Immunotoxins; 0 / Interleukin-13 Receptor alpha2 Subunit; 0 / pseudomonas exoprotein A protein, Pseudomonas aeruginosa
  • [Other-IDs] NLM/ PMC2730867
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22. Neto AC, Ball BA, Browne P, Conley AJ: Cellular localization of androgen synthesis in equine granulosa-theca cell tumors: immunohistochemical expression of 17alpha-hydroxylase/17,20-lyase cytochrome P450. Theriogenology; 2010 Aug;74(3):393-401
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  • This study was conducted to localize and thereby define the cellular expression of 17alpha-hydroxylase/17,20-lyase cytochrome P450 (P450c17), the enzyme most directly responsible for androgen synthesis, in 30 GTCTs and control tissues (gonads and adrenal glands) using immuno-histochemistry (IHC).
  • Testicular interstitial cells and islands of adreno-cortical cells located in the adrenal medulla of the adrenal cortex further established the specificity of the antisera used.
  • [MeSH-major] Androgens / biosynthesis. Granulosa Cell Tumor / veterinary. Horse Diseases / enzymology. Ovarian Neoplasms / veterinary. Steroid 17-alpha-Hydroxylase / metabolism. Thecoma / veterinary

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20416939.001).
  • [ISSN] 1879-3231
  • [Journal-full-title] Theriogenology
  • [ISO-abbreviation] Theriogenology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Androgens; EC 1.14.99.9 / Steroid 17-alpha-Hydroxylase
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23. Geli J, Kiss N, Lanner F, Foukakis T, Natalishvili N, Larsson O, Kogner P, Höög A, Clark GJ, Ekström TJ, Bäckdahl M, Farnebo F, Larsson C: The Ras effectors NORE1A and RASSF1A are frequently inactivated in pheochromocytoma and abdominal paraganglioma. Endocr Relat Cancer; 2007 Mar;14(1):125-34
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  • NORE1A (RASSF5) and RASSF1A are newly described Ras effectors with tumour suppressor functions.
  • Significantly suppressed NORE1A and RASSF1A mRNA levels were detected in primary tumours compared with normal adrenal medulla (P<0.001).
  • [MeSH-major] Abdominal Neoplasms / genetics. Monomeric GTP-Binding Proteins / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. Tumor Suppressor Proteins / genetics

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  • (PMID = 17395981.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RASSF1 protein, human; 0 / RASSF5 protein, human; 0 / RNA, Messenger; 0 / Sulfites; 0 / Tumor Suppressor Proteins; EC 3.6.5.2 / Monomeric GTP-Binding Proteins; OJ9787WBLU / hydrogen sulfite
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24. Longo L, Borghini S, Schena F, Parodi S, Albino D, Bachetti T, Da Prato L, Truini M, Gambini C, Tonini GP, Ceccherini I, Perri P: PHOX2A and PHOX2B genes are highly co-expressed in human neuroblastoma. Int J Oncol; 2008 Nov;33(5):985-91
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  • In this light, we have carried out a quantitative expression analysis of PHOX2B and its paralogue PHOX2A on a panel of NB cell lines and NB tumour samples to identify a possible differential expression between NB cells and their normal counterpart (adrenal medulla cells).
  • Our results revealed that both PHOX2A and PHOX2B are over-expressed in tumour samples and NB cell lines.
  • [MeSH-minor] Adrenal Medulla / metabolism. Cell Line, Tumor. DNA Mutational Analysis. Humans. Pedigree. Up-Regulation

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  • (PMID = 18949361.001).
  • [ISSN] 1019-6439
  • [Journal-full-title] International journal of oncology
  • [ISO-abbreviation] Int. J. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Homeodomain Proteins; 0 / NBPhox protein; 0 / PHOX2A protein, human; 0 / Transcription Factors
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25. Huebener N, Fest S, Strandsby A, Michalsky E, Preissner R, Zeng Y, Gaedicke G, Lode HN: A rationally designed tyrosine hydroxylase DNA vaccine induces specific antineuroblastoma immunity. Mol Cancer Ther; 2008 Jul;7(7):2241-51
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  • Therapeutic vaccination against tumor antigens without induction of autoimmunity remains a major challenge in cancer immunotherapy.
  • Importantly, no cell infiltration was detectable in TH-expressing adrenal medulla, indicating the absence of autoimmunity.
  • [MeSH-minor] Amino Acid Sequence. Animals. Antibody Specificity / immunology. COS Cells. Cercopithecus aethiops. Cytotoxicity, Immunologic. Histocompatibility Antigens Class I / immunology. Lymphocyte Activation. Lymphocytes, Tumor-Infiltrating / immunology. Mice. Models, Molecular. Molecular Sequence Data. Peptides / chemistry. T-Lymphocytes / immunology. Ubiquitin / metabolism. Vaccination

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  • (PMID = 18645033.001).
  • [ISSN] 1535-7163
  • [Journal-full-title] Molecular cancer therapeutics
  • [ISO-abbreviation] Mol. Cancer Ther.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Histocompatibility Antigens Class I; 0 / Peptides; 0 / Ubiquitin; 0 / Vaccines, DNA; EC 1.14.16.2 / Tyrosine 3-Monooxygenase
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26. Toni García M, Anda Apiñániz E, Pablo J, de Esteban M, Munárriz Alcuaz P, Goñi Iriarte MJ, Forga Llenas L: An unusual association: pheochromocytoma on an atrophied adrenal gland due to addison's disease. Endocrinol Nutr; 2008 Dec;55(10):510-3
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  • [Title] An unusual association: pheochromocytoma on an atrophied adrenal gland due to addison's disease.
  • APS-II is defined by the development of two or more of the following entities: primary adrenal insufficiency (Addison's disease), Graves' disease, type 1A diabetes mellitus, autoimmune thyroiditis, primary hypogonadism, celiac disease, and myasthenia gravis.
  • Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed by autoantibodies against 21-hydroxylase.
  • Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved.
  • Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla.
  • In this patient, the adrenal gland cortex was atrophied and the tumor was attached to the adrenal medulla.

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  • [Copyright] Copyright © 2008 Sociedad Española de Endocrinología y Nutrición. Published by Elsevier Espana. All rights reserved.
  • (PMID = 22980466.001).
  • [ISSN] 1575-0922
  • [Journal-full-title] Endocrinología y nutrición : órgano de la Sociedad Española de Endocrinología y Nutrición
  • [ISO-abbreviation] Endocrinol Nutr
  • [Language] eng; spa
  • [Publication-type] Journal Article
  • [Publication-country] Spain
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27. Cayo MA, Cayo AK, Jarjour SM, Chen H: Sodium butyrate activates Notch1 signaling, reduces tumor markers, and induces cell cycle arrest and apoptosis in pheochromocytoma. Am J Transl Res; 2009 Jan 31;1(2):178-83
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  • [Title] Sodium butyrate activates Notch1 signaling, reduces tumor markers, and induces cell cycle arrest and apoptosis in pheochromocytoma.
  • BACKGROUND: Pheochromocytoma is a neuroendocrine (NE) tumor of the adrenal medulla for which surgical resection is the only therapy.
  • Our lab has demonstrated the importance of the Notch1 signaling pathway in NE neoplasia, indicating that this pathway could be a target for emergent treatments in pheochromocytoma.
  • We hypothesized that the HDAC inhibitor Sodium Butyrate (NaB) might activate Notch1 in pheochromocytoma resulting in altered tumor cell proliferation.

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  • (PMID = 19956429.001).
  • [ISSN] 1943-8141
  • [Journal-full-title] American journal of translational research
  • [ISO-abbreviation] Am J Transl Res
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA109053; United States / NCI NIH HHS / CA / R21 CA117117; United States / NIDDK NIH HHS / DK / T35 DK062709
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2776315
  • [Keywords] NOTNLM ; Butyrate / HDAC inhibitor / Notch1 / PC-12 / neuroendocrine / pheochromocytoma
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28. Puc J, Placha G, Wocial B, Podsypanina K, Parsons R, Gaciong Z: Analysis of PTEN mutation in non-familial pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:317-31
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  • PTEN, a tumor suppressor gene, is frequently mutated in a variety of human tumors.
  • In mice, monoallelic inactivation of this gene predisposes animals to neoplasia of multiple organs.
  • Interestingly, Pten heterozygous mice develop bilateral hyperplasia of the adrenal medulla.
  • Examination of protein expression by immunohistochemistry using 8 normal adrenals and 11 sporadic pheochromocytomas showed no decrease in the PTEN protein expression in the tumor tissue, but upregulation of insulin-like growth factor II, a peptide implicated in growth of adrenal tissue, was observed in four cases (36%).
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Mutation. PTEN Phosphohydrolase / genetics. Pheochromocytoma / genetics


29. Kinoshita Y, Kuratsukuri K, Landas S, Imaida K, Rovito PM Jr, Wang CY, Haas GP: Expression of prostate-specific membrane antigen in normal and malignant human tissues. World J Surg; 2006 Apr;30(4):628-36
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  • BACKGROUND: Prostate-specific membrane antigen (PSMA) is upregulated in androgen-dependent prostate carcinoma and it has been targeted for immunotherapy and diagnosis of this cancer.
  • RESULTS: Prostate-specific membrane antigen was detected in the epithelium of prostate, urinary bladder, proximal tubules of kidney, liver, esophagus, stomach, small intestine, colon, breast, fallopian tubes and testicular seminiferous tubules, hippocampal neurons and astrocytes, ependyma, cortex and medulla of the adrenal gland, and ovary stroma.
  • It was also detected in neoplasms of the prostate, kidney, urinary bladder, stomach, small intestine, colon, lung, adrenal gland, and testis.
  • The broad distribution of PSMA may make it suitable for the diagnosis and therapy of a wide variety of tumors.
  • [MeSH-major] Antigens, Surface / analysis. Biomarkers, Tumor / analysis. Glutamate Carboxypeptidase II / analysis. Neoplasms / pathology. Prostate / pathology. Prostatic Neoplasms / pathology. Tumor Cells, Cultured / pathology

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  • (PMID = 16555021.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Grant] United States / NIA NIH HHS / AG / 1R01AG21389-01
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Surface; 0 / Biomarkers, Tumor; EC 3.4.17.21 / Glutamate Carboxypeptidase II; EC 3.4.17.21 / glutamate carboxypeptidase II, human
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30. Sandgren J, Andersson R, Rada-Iglesias A, Enroth S, Akerstrom G, Dumanski JP, Komorowski J, Westin G, Wadelius C: Integrative epigenomic and genomic analysis of malignant pheochromocytoma. Exp Mol Med; 2010 Jul 31;42(7):484-502
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  • Epigenomic and genomic changes affect gene expression and contribute to tumor development.
  • The histone modifications trimethylated histone H3 lysine 4 (H3K4me3) and lysine 27 (H3K27me3) are epigenetic regulators associated to active and silenced genes, respectively and alterations of these modifications have been observed in cancer.
  • Pheochromocytoma is a rare endocrine tumor of the adrenal gland that mostly occurs sporadic with unknown epigenetic/genetic cause.
  • The integrated analysis of the tumor expression levels, in relation to normal adrenal medulla, indicated that either histone modifications or chromosomal alterations, or both, have great impact on the expression of a substantial fraction of the genes in the investigated sample.
  • Candidate tumor suppressor genes identified with decreased expression, a H3K27me3 mark and/or in regions of deletion were for instance TGIF1, DSC3, TNFRSF10B, RASSF2, HOXA9, PTPRE and CDH11.
  • Our approach to associate histone methylations and DNA copy number changes to gene expression revealed apparent impact on global gene transcription, and enabled the identification of candidate tumor genes for further exploration.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Epigenesis, Genetic. Genome, Human / genetics. Genomics. Pheochromocytoma / genetics. Pheochromocytoma / pathology
  • [MeSH-minor] Female. Gene Dosage / genetics. Gene Expression Regulation, Neoplastic. Gene Regulatory Networks / genetics. Histones / metabolism. Humans. Lysine / metabolism. Methylation. Protein Processing, Post-Translational. Tumor Suppressor Proteins / genetics. Tumor Suppressor Proteins / metabolism

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  • (PMID = 20534969.001).
  • [ISSN] 2092-6413
  • [Journal-full-title] Experimental & molecular medicine
  • [ISO-abbreviation] Exp. Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Histones; 0 / Tumor Suppressor Proteins; K3Z4F929H6 / Lysine
  • [Other-IDs] NLM/ PMC2912476
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31. Björklund P, Cupisti K, Fryknäs M, Isaksson A, Willenberg HS, Akerström G, Hellman P, Westin G: Stathmin as a marker for malignancy in pheochromocytomas. Exp Clin Endocrinol Diabetes; 2010 Jan;118(1):27-30
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  • Pheochromocytomas of the adrenal medulla may be life-threatening catecholamine-producing tumors which are malignant in about 10% of cases.
  • Differential diagnosis between malignant and benign tumors is dependent on the development of metastasis or extensive local invasion.
  • We applied an expression microarray containing 7770 cDNA clones and analysed the expression profiles in eleven tumors compared to normal adrenal medulla.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Biomarkers, Tumor / metabolism. Pheochromocytoma / diagnosis. Stathmin / metabolism
  • [MeSH-minor] Adrenal Medulla / metabolism. Adrenal Medulla / pathology. Adult. Aged. Blotting, Western. Diagnosis, Differential. Female. Gene Expression Profiling. Humans. Immunohistochemistry. Male. Middle Aged. Oligonucleotide Array Sequence Analysis. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Young Adult

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  • [Copyright] J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart, New York.
  • (PMID = 19449284.001).
  • [ISSN] 1439-3646
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / RNA, Messenger; 0 / STMN1 protein, human; 0 / Stathmin
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32. Bonar CJ, Lewandowski AH, Arafah B, Capen CC: Pheochromocytoma in an aged female African elephant (Loxodonta africana). J Zoo Wildl Med; 2005 Dec;36(4):719-23
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  • Necropsy revealed an enlarged right adrenal medulla.
  • Special stains and electron microscopy demonstrated chromaffin granules, suggesting that the tumor was derived from catecholamine secreting cells of the adrenal medulla, and may have been functionally secretory.
  • Serum levels of both norepinephrine and epinephrine were elevated at time of death, supporting the functional nature of the tumor.
  • Pheochromocytoma should be considered as a differential diagnosis in cases of suspected hypertension and acute death in elephants.
  • [MeSH-major] Adrenal Gland Neoplasms / veterinary. Elephants. Pheochromocytoma / veterinary
  • [MeSH-minor] Animals. Death, Sudden / veterinary. Diagnosis, Differential. Fatal Outcome. Female. Immunohistochemistry / veterinary

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  • (PMID = 17312735.001).
  • [ISSN] 1042-7260
  • [Journal-full-title] Journal of zoo and wildlife medicine : official publication of the American Association of Zoo Veterinarians
  • [ISO-abbreviation] J. Zoo Wildl. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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33. Jakowski JD, Wakely PE Jr, Jimenez RE: An uncommon type of adrenal incidentaloma: a case report of a schwannoma of the adrenal medulla with cytological, histological, and ultrastructural correlation. Ann Diagn Pathol; 2008 Oct;12(5):356-61
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  • [Title] An uncommon type of adrenal incidentaloma: a case report of a schwannoma of the adrenal medulla with cytological, histological, and ultrastructural correlation.
  • Benign nerve sheath tumors of the adrenal gland are an extremely uncommon cause of an incidentaloma.
  • We report a case of a schwannoma of the adrenal medulla in an asymptomatic 51-year-old woman, which was discovered incidentally on a computed tomography scan after routine workup for her degenerative joint diseases of the lumbar spine.
  • Because of the large size and unknown biologic nature of the tumor by clinical and radiographic studies alone, an adrenalectomy was performed.
  • The gross specimen featured a well-circumscribed medullary based tumor with cystic degeneration.
  • The diagnosis of a nerve sheath tumor was based on classic histological findings, supported by S-100 positivity, and ultrastructurally by the finding of typical Schwann cells.
  • The cytological diagnosis from the fine-needle aspiration biopsy material obtained at the time of gross examination was much more challenging on retrospective review.
  • A review of the histogenesis and differential diagnosis of this common nerve sheath tumor in this unusual location is discussed.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Adrenal Medulla / pathology. Neurilemmoma / pathology
  • [MeSH-minor] Adrenalectomy. Biomarkers, Tumor / analysis. Biopsy. Female. Humans. Incidental Findings. Middle Aged. S100 Proteins / analysis. Schwann Cells / ultrastructure. Tomography, X-Ray Computed

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  • (PMID = 18774499.001).
  • [ISSN] 1532-8198
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / S100 Proteins
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34. Plouin PF, Gimenez-Roqueplo AP, Bertagna X: [COMETE, a network for the study and management of hypersecreting adrenal tumors]. Bull Acad Natl Med; 2008 Jan;192(1):73-82; discussion 83-5
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  • [Title] [COMETE, a network for the study and management of hypersecreting adrenal tumors].
  • [Transliterated title] Le réseau national COMETE sur les tumeurs de la surrénale.
  • Patients with adrenal tumors are at risk of the consequences of tumor growth (including metastasis) and of hormone hypersecretion.
  • Pheochromocytomas and paragangliomas arise from the adrenal medulla and produce catecholamines; they may be benign or malignant, and sporadic or familial.
  • Adrenal adenomas and carcinomas arise from the adrenal cortex.
  • The overall objective of COMETE is to promote basic and clinical research into adrenal tumors.
  • This implies - in cross-sectional studies: collecting adrenal tumors, maintaining a collection of tumor and leukocyte DNA samples, keeping a computerized record of relevant biological and clinical data, and distributing biological samples and bioclinical information anonymously to collaborating research laboratories; in prospective studies: ensuring indefinite follow-up of patients with tumors at risk of malignancy or recurrence, which means establishing and maintaining a cohort of patients with large adrenocortical tumors or carcinomas anda cohort of patients with pheochromocytomas or paragangliomas.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Societies, Medical / organization & administration

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  • (PMID = 18663983.001).
  • [ISSN] 0001-4079
  • [Journal-full-title] Bulletin de l'Académie nationale de médecine
  • [ISO-abbreviation] Bull. Acad. Natl. Med.
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Netherlands
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35. Guérin M, Guillemot J, Thouënnon E, Pierre A, El-Yamani FZ, Montero-Hadjadje M, Dubessy C, Magoul R, Lihrmann I, Anouar Y, Yon L: Granins and their derived peptides in normal and tumoral chromaffin tissue: Implications for the diagnosis and prognosis of pheochromocytoma. Regul Pept; 2010 Nov 30;165(1):21-9
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  • [Title] Granins and their derived peptides in normal and tumoral chromaffin tissue: Implications for the diagnosis and prognosis of pheochromocytoma.
  • Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites.
  • The diagnosis of pheochromocytomas depends mainly upon the demonstration of catecholamine excess by 24-h urinary catecholamines and metanephrines or plasma metanephrines.
  • In this context, we will focus here on reviewing the distribution and characterization of granins and their processing products in normal and tumoral chromaffin cells, and their clinical usefulness for the diagnosis and prognosis of pheochromocytomas.
  • In most cases, elevated levels of these entities were found, in correlation with tumor occurrence, while rarely discriminating between benign and malignant neoplasms.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / pathology. Chromaffin Cells / metabolism. Chromaffin Cells / pathology. Chromogranins / metabolism. Pheochromocytoma / diagnosis. Pheochromocytoma / pathology


36. Cascón A, Montero-Conde C, Ruiz-Llorente S, Mercadillo F, Letón R, Rodríguez-Antona C, Martínez-Delgado B, Delgado M, Díez A, Rovira A, Díaz JA, Robledo M: Gross SDHB deletions in patients with paraganglioma detected by multiplex PCR: a possible hot spot? Genes Chromosomes Cancer; 2006 Mar;45(3):213-9
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  • Pheochromocytoma and paraganglioma are rare neuroendocrine tumors that arise in the adrenal medulla and the extra-adrenal paraganglia, respectively.
  • Although this is the first report describing the presence of gross deletions in patients with apparently sporadic paragangliomas, the extra-adrenal location of the tumor seems to constitute a determining factor for whether to include these patients in genetic testing for gross deletions in the SDHB gene.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Deletion. Iron-Sulfur Proteins / genetics. Paraganglioma / genetics. Pheochromocytoma / genetics. Protein Subunits / genetics. Succinate Dehydrogenase / genetics


37. Bayley JP, van Minderhout I, Hogendoorn PC, Cornelisse CJ, van der Wal A, Prins FA, Teppema L, Dahan A, Devilee P, Taschner PE: Sdhd and SDHD/H19 knockout mice do not develop paraganglioma or pheochromocytoma. PLoS One; 2009;4(11):e7987
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  • SDHD is remarkable in showing an 'imprinted' tumor suppressor phenotype.
  • We also crossed this mouse with a knockout of H19, a postulated imprinted modifier gene of Sdhd tumorigenesis, to evaluate if loss of these genes together would lead to the initiation or enhancement of tumor development.
  • No paraganglioma or other tumor development was seen in Sdhd KO mice followed for their entire lifespan, in sharp contrast to the highly penetrant phenotype in humans.
  • Heterozygous Sdhd KO mice did not show hyperplasia of paraganglioma-related tissues such as the carotid body or of the adrenal medulla, or any genotype-related pathology, with similar body and organ weights to wildtype mice.
  • [MeSH-minor] Animals. Female. Gene Expression Regulation, Neoplastic. Genes, Tumor Suppressor. Genotype. Heterozygote. Male. Mice. Mice, Knockout. Phenotype. RNA, Long Noncoding


38. Ait-Ali D, Turquier V, Tanguy Y, Thouënnon E, Ghzili H, Mounien L, Derambure C, Jégou S, Salier JP, Vaudry H, Eiden LE, Anouar Y: Tumor necrosis factor (TNF)-alpha persistently activates nuclear factor-kappaB signaling through the type 2 TNF receptor in chromaffin cells: implications for long-term regulation of neuropeptide gene expression in inflammation. Endocrinology; 2008 Jun;149(6):2840-52
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  • [Title] Tumor necrosis factor (TNF)-alpha persistently activates nuclear factor-kappaB signaling through the type 2 TNF receptor in chromaffin cells: implications for long-term regulation of neuropeptide gene expression in inflammation.
  • Chromaffin cells of the adrenal medulla elaborate and secrete catecholamines and neuropeptides for hormonal and paracrine signaling in stress and during inflammation.
  • TNF-alpha-dependent signaling in neuroendocrine cells thus leads to a unique, persistent mode of NF-kappaB activation that features long-lasting transcription of both IkappaB and MIG-6, which may play a role in the long-lasting effects of TNF-alpha in regulating neuropeptide output from the adrenal, a potentially important feedback station for modulating long-term cytokine effects in inflammation.

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  • (PMID = 18292192.001).
  • [ISSN] 0013-7227
  • [Journal-full-title] Endocrinology
  • [ISO-abbreviation] Endocrinology
  • [Language] ENG
  • [Grant] United States / NIMH NIH HHS / MH / Z01 MH002386
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / NF-kappa B; 0 / Neuropeptides; 0 / Recombinant Proteins; 0 / TNF Receptor-Associated Factor 2; 0 / Tumor Necrosis Factor-alpha; 63231-63-0 / RNA
  • [Other-IDs] NLM/ PMC2408812
  •  go-up   go-down


39. Molatore S, Liyanarachchi S, Irmler M, Perren A, Mannelli M, Ercolino T, Beuschlein F, Jarzab B, Wloch J, Ziaja J, Zoubaa S, Neff F, Beckers J, Höfler H, Atkinson MJ, Pellegata NS: Pheochromocytoma in rats with multiple endocrine neoplasia (MENX) shares gene expression patterns with human pheochromocytoma. Proc Natl Acad Sci U S A; 2010 Oct 26;107(43):18493-8
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  • [Title] Pheochromocytoma in rats with multiple endocrine neoplasia (MENX) shares gene expression patterns with human pheochromocytoma.
  • Pheochromocytomas are rare neoplasias of neural crest origin arising from chromaffin cells of the adrenal medulla and sympathetic ganglia (extra-adrenal pheochromocytoma).
  • Pheochromocytoma that develop in rats homozygous for a loss-of-function mutation in p27Kip1 (MENX syndrome) show a clear progression from hyperplasia to tumor, offering the possibility to gain insight into tumor pathobiology.
  • We compared the gene-expression signatures of both adrenomedullary hyperplasia and pheochromocytoma with normal rat adrenal medulla.
  • Hyperplasia and tumor show very similar transcriptome profiles, indicating early determination of the tumorigenic signature.
  • Overexpression of these genes precedes histological changes in affected adrenal glands.
  • Adrenal and extra-adrenal pheochromocytoma development clearly follows diverged molecular pathways in MENX rats.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Multiple Endocrine Neoplasia / genetics. Pheochromocytoma / genetics
  • [MeSH-minor] Adrenal Medulla / metabolism. Adrenal Medulla / pathology. Animals. Base Sequence. Biomarkers, Tumor / genetics. Cyclin-Dependent Kinase Inhibitor p27 / genetics. DNA Primers / genetics. Disease Models, Animal. Gene Expression Profiling. Homeodomain Proteins / genetics. Humans. Hyperplasia. Neural Cell Adhesion Molecule L1 / genetics. PC12 Cells. Paraganglioma / genetics. Rats. Rats, Mutant Strains. Species Specificity

  • Genetic Alliance. consumer health - Multiple Endocrine Neoplasia.
  • Genetic Alliance. consumer health - Pheochromocytoma.
  • MedlinePlus Health Information. consumer health - Adrenal Gland Cancer.
  • MedlinePlus Health Information. consumer health - Pheochromocytoma.
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  • (PMID = 20937862.001).
  • [ISSN] 1091-6490
  • [Journal-full-title] Proceedings of the National Academy of Sciences of the United States of America
  • [ISO-abbreviation] Proc. Natl. Acad. Sci. U.S.A.
  • [Language] eng
  • [Databank-accession-numbers] GEO/ GSE21006
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cdkn1b protein, rat; 0 / DNA Primers; 0 / Homeodomain Proteins; 0 / Neural Cell Adhesion Molecule L1; 0 / PHOX2A protein, human; 147604-94-2 / Cyclin-Dependent Kinase Inhibitor p27
  • [Other-IDs] NLM/ PMC2972990
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40. Onuki T, Yamagishi T, Teranishi J, Suzuki K, Kondo K, Nakaigawa N, Saito K, Noguchi K, Kubota Y: [Clinical study of 38 cases of pheochromocytoma --correlation between the instability of intraoperative blood pressure and 24-hour urinary vanillylmandelic acid]. Hinyokika Kiyo; 2007 Jul;53(7):449-54
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  • Pheochromocytoma is a rare tumor of chromaffin tissues most commonly arising from the adrenal medulla.
  • One patient had bilateral adrenal tumors and pathological examination revealed malignant pheochromocytoma.
  • Six patients had an extra-adrenal tumor and in 2 patients the tumor occurred in the urinary bladder.
  • The 24-h urinary total metanephrines and vanillylmandelic acid (VMA) were the most sensitive biochemical tests for the diagnosis of pheochromocytoma.
  • Fifteen patients had intraoperative hypertensive reactions in the surgical manipulation or hypotension after tumor resection.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Hypertension / diagnosis. Intraoperative Complications / diagnosis. Pheochromocytoma / diagnosis. Vanilmandelic Acid / urine


41. 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


42. Adams MS, Bronner-Fraser M: Review: the role of neural crest cells in the endocrine system. Endocr Pathol; 2009;20(2):92-100
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  • According to current understanding, neural crest cells give rise to the chromaffin cells of the adrenal medulla, chief cells of the extra-adrenal paraganglia, and thyroid C cells.
  • The endocrine tumors that correspond to these cell types are pheochromocytomas, extra-adrenal paragangliomas, and medullary thyroid carcinomas.
  • Although controversies concerning embryological origin appear to have mostly been resolved, questions persist concerning the pathobiology of each tumor type and its basis in neural crest embryology.

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  • (PMID = 19377845.001).
  • [ISSN] 1046-3976
  • [Journal-full-title] Endocrine pathology
  • [ISO-abbreviation] Endocr. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 141
  •  go-up   go-down


43. Gorsane I, Zouaghi K, Goucha R, Bacha MM, Hedri H, Khiari K, Abderrahim E, Abdallah TB, Abdallah NB, Moussa FB, Maiz HB, Kheder A: [Pheochromocytoma in end-stage renal disease patient treated by peritoneal dialysis]. Nephrol Ther; 2008 Dec;4(7):597-601
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  • Pheochromocytoma is a rare tumor responsible for paroxysmal hypertension which is difficult to control.
  • Diagnosis is important because it represents a curable form of hypertension.
  • These cases are specially responsible for diagnosis and therapeutic problems.
  • Radiographic diagnosis tests were negative but MIBG scintigraphy was able to localise the tumor in the left suprarenal gland.
  • He had coelioscopic left adrenalectomy without complications, microscopic studies showed an hyperplasia of the adrenal medulla.
  • We conclude that refractory hypertension, as a possible diagnosis, is uncommon in peritoneal dialysis patients.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Kidney Failure, Chronic / complications. Kidney Failure, Chronic / therapy. Nephritis / complications. Pheochromocytoma / diagnosis


44. Cotesta D, Petramala L, Serra V, Giustini S, Divona L, Calvieri S, De Toma G, Ciardi A, Corsi A, Massa R, Reale MG, Letizia C: Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review. Minerva Endocrinol; 2006 Jun;31(2):183-9
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  • [Title] Pheochromocytoma associated with adrenocortical tumor in the same gland. Two case reports and literature review.
  • Pheochromocytomas are catecholamine-producing neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglionic system that show 2 distinctive features, rarity and clinical variability.
  • Pheochromocytoma occasionally is associated with pathological lesions of the adrenal cortex.
  • The diagnosis of pheochromocytoma was confirmed in both cases with laboratory analysis and the lesion was achieved by employing 3 imaging techniques: computed tomography (CT), magnetic resonance imaging (MRI) and scintigraphy with (123)I-metaiodobenzilguanidine (MIBG).
  • The patients underwent adrenalectomy and in the same adrenal gland we found a pheochromocytoma associated with a nonfunctioning cortical adenoma.
  • [MeSH-major] Adrenal Cortex Neoplasms / diagnosis. Adrenal Gland Neoplasms / diagnosis. Adrenocortical Adenoma / diagnosis. Neoplasms, Multiple Primary / diagnosis. Pheochromocytoma / diagnosis
  • [MeSH-minor] 3-Iodobenzylguanidine. Adrenalectomy. Adult. Humans. Male. Middle Aged. Neurofibromatosis 1 / diagnosis. Radiopharmaceuticals. Treatment Outcome

  • Genetic Alliance. consumer health - Pheochromocytoma.
  • MedlinePlus Health Information. consumer health - Adrenal Gland Cancer.
  • MedlinePlus Health Information. consumer health - Pheochromocytoma.
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  • (PMID = 16682942.001).
  • [ISSN] 0391-1977
  • [Journal-full-title] Minerva endocrinologica
  • [ISO-abbreviation] Minerva Endocrinol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine
  • [Number-of-references] 41
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45. Guillemot J, Compagnon P, Cartier D, Thouennon E, Bastard C, Lihrmann I, Pichon P, Thuillez C, Plouin PF, Bertherat J, Anouar Y, Kuhn JM, Yon L, Lefebvre H: Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors. Endocr Relat Cancer; 2009 Mar;16(1):281-90
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  • Tissue explants, obtained from 18 pheochromocytomas including the tumor removed from a 46-year-old female patient who experienced life-threatening hypertension crisis after metoclopramide administration and 17 additional pheochromocytomas (9 benign and 8 malignant) were studied.
  • RESULTS: Metoclopramide and the 5-HT(4) receptor agonist cisapride were found to activate catecholamine- and granin-derived peptide secretions by cultured tumor cells.
  • 5-HT(4) receptor mRNAs were detected in the patient's tumor and the series of 17 additional pheochromocytomas.
  • [MeSH-major] Adrenal Gland Neoplasms / drug therapy. Dopamine Antagonists / pharmacology. Metoclopramide / pharmacology. Pheochromocytoma / drug therapy. Receptors, Serotonin, 5-HT4 / genetics
  • [MeSH-minor] Adrenal Medulla / cytology. Adrenal Medulla / drug effects. Catecholamines / secretion. Chromogranins / secretion. Cisapride / pharmacology. Domperidone / pharmacology. Female. Humans. Middle Aged. RNA, Messenger / metabolism. Retrospective Studies. Reverse Transcriptase Polymerase Chain Reaction. Serotonin Receptor Agonists / pharmacology. Tumor Cells, Cultured

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  • (PMID = 18948374.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Catecholamines; 0 / Chromogranins; 0 / Dopamine Antagonists; 0 / RNA, Messenger; 0 / Serotonin Receptor Agonists; 158165-40-3 / Receptors, Serotonin, 5-HT4; 5587267Z69 / Domperidone; L4YEB44I46 / Metoclopramide; UVL329170W / Cisapride
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46. Zhou M, Shen D, Head JE, Chew EY, Chévez-Barrios P, Green WR, Chan CC: Ocular clusterin expression in von Hippel-Lindau disease. Mol Vis; 2007;13:2129-36
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  • Its mRNA is ubiquitously expressed, with high levels in von Hippel-Lindau (VHL) target organs such as the brain, liver, kidney, and adrenal medulla.
  • RESULTS: All retinal hemangioblastoma were composed of typical VHL tumor cells admixed with small vascular channels as well as glial cells.
  • CONCLUSIONS: Clusterin shows possible important functions in tumor suppression by the VHL gene product (pVHL) and the potential to be a novel biomarker in retinal hemangioblastoma associated VHL disease.

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  • (PMID = 18079682.001).
  • [ISSN] 1090-0535
  • [Journal-full-title] Molecular vision
  • [ISO-abbreviation] Mol. Vis.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000222-22; United States / Intramural NIH HHS / / Z99 EY999999
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Clusterin; 0 / RNA, Messenger
  • [Other-IDs] NLM/ NIHMS36212; NLM/ PMC2173882
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47. 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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48. Kelliher K, Santiago A, Estrada DE, Campbell BT: Laparoscopic excision of a familial paraganglioma. J Laparoendosc Adv Surg Tech A; 2009 Apr;19 Suppl 1:S155-8
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  • Pheochromocytomas are rare neuroendocrine tumors that secrete catecholamines and usually arise from the adrenal medulla.
  • Catecholamine-producing tumors that arise from extra-adrenal chromaffin tissue are referred to as paragangliomas, or extra-adrenal pheochromocytomas.
  • Contrary to the traditional "Rule of Tens," as many as 25% of pheochromocytomas occur in hereditary tumor syndromes, such as multiple endocrine neoplasia-2, von Hippel-Lindau disease, neurofibromatosis-1, or hereditary or familial paraganglioma syndrome.
  • This case report and the accompanying video demonstrate that the laparoscopic approach to retroperitoneal paraganglioma resection provides excellent exposure of the tumor and surrounding structures.
  • [MeSH-major] Laparoscopy. Paraganglioma / surgery. Paraganglioma, Extra-Adrenal / surgery. Retroperitoneal Neoplasms / surgery

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  • (PMID = 19260793.001).
  • [ISSN] 1092-6429
  • [Journal-full-title] Journal of laparoendoscopic & advanced surgical techniques. Part A
  • [ISO-abbreviation] J Laparoendosc Adv Surg Tech A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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49. Ohno N, Terada N, Komada M, Saitoh S, Costantini F, Pace V, Germann PG, Weber K, Yamakawa H, Ohara O, Ohno S: Dispensable role of protein 4.1B/DAL-1 in rodent adrenal medulla regarding generation of pheochromocytoma and plasmalemmal localization of TSLC1. Biochim Biophys Acta; 2009 Mar;1793(3):506-15
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  • [Title] Dispensable role of protein 4.1B/DAL-1 in rodent adrenal medulla regarding generation of pheochromocytoma and plasmalemmal localization of TSLC1.
  • Protein 4.1B is a membrane skeletal protein expressed in various organs, and is associated with tumor suppressor in lung cancer-1 (TSLC1) in vitro.
  • Although involvement of 4.1B in the intercellular junctions and tumor-suppression was suggested, some controversial results posed questions to the general tumor-suppressive function of 4.1B and its relation to TSLC1 in vivo.
  • In this study, the expression of 4.1B and its interaction with TSLC1 were examined in rodent adrenal gland, and the involvement of 4.1B in tumorigenesis and the effect of 4.1B deficiency on TSLC1 distribution were also investigated using rodent pheochromocytoma and 4.1B-knockout mice.
  • Although plasmalemmal immunolocalization of 4.1B was shown in chromaffin cells of rodent adrenal medulla, expression of 4.1B was maintained in developed pheochromocytoma, and morphological abnormality or pheochromocytoma generation could not be found in 4.1B-deficient mice.
  • Furthermore, molecular interaction and colocalization of 4.1B and TSLC1 were observed in mouse adrenal gland, but the immunolocalization of TSLC1 along chromaffin cell membranes was not affected in the 4.1B-deficient mice.
  • These results suggest that the function of 4.1B as tumor suppressor might significantly differ among organs and species, and that plasmalemmal retention of TSLC1 would be maintained by molecules other than 4.1B interacting in rodent chromaffin cells.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Medulla / metabolism. Immunoglobulins / metabolism. Membrane Proteins / metabolism. Pheochromocytoma / metabolism. Tumor Suppressor Proteins / metabolism


50. Pan Z, Repertinger S, Deng C, Sharma P: A giant cystic pheochromocytoma of the adrenal gland. Endocr Pathol; 2008;19(2):133-8
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  • [Title] A giant cystic pheochromocytoma of the adrenal gland.
  • Adrenal pheochromocytomas are rare catecholamine-secreting tumors that originate from chromaffin cells in the adrenal medulla, and giant pheochromocytomas with cystic changes are particularly rare.
  • Radiographic studies showed an 18-cm cystic mass in the left upper quadrant of the abdomen; further workups, which included light microscopy, immunohistochemical, and electron microscopic analysis, revealed a pheochromocytoma of the left adrenal gland.
  • Cytogenetic analysis and genetic mutation analyses for von-Hippel-Lindau (VHL), rearranged during transfection (RET), and succinate dehydrogenase complex subunit B (SDHB) genes were also performed but failed to reveal any abnormalities within the tumor cells.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Adrenalectomy. DNA Mutational Analysis. Headache / diagnosis. Headache / etiology. Humans. Immunohistochemistry. Male. Microscopy, Electron. Middle Aged. Paraffin Embedding. Tissue Fixation. Tomography, X-Ray Computed


51. Mukai M, Takao T, Yoshida T, Inoue H, Miyagawa Y, Yoshimura K, Okuyama A, Aozasa K, Fujii T, Takatera H: [Adrenal ganglioneuroma in a 14-year-old girl: a case report]. Hinyokika Kiyo; 2006 Aug;52(8):619-21
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  • [Title] [Adrenal ganglioneuroma in a 14-year-old girl: a case report].
  • A 14-year-old girl was referred to our hospital for examination of a right adrenal tumor, incidentally found by abdominal ultrasound sonography.
  • Computed tomographic scan and magnetic resonance imaging showed a 44 x 20 mm solid tumor in the right adrenal region.
  • Laparoscopic adrenalectomy was performed and the tumor was histologically diagnosed as ganglioneuroma originated from the right adrenal medulla.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Ganglioneuroma / diagnosis
  • [MeSH-minor] Adolescent. Adrenal Medulla. Adrenalectomy. Female. Humans. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 16972624.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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52. 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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53. Babińska A, Gnacińska A, Swiatkowska-Stodulska R, Sworczak K: Myocardial infarction in a 30-year-old patient with pheochromocytoma and type 1 neurofibromatosis. Pol Arch Med Wewn; 2008 Sep;118(9):517-23
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  • Chromaffinoma of the adrenal medulla (pheochromocytoma--PHEO) is a rare cause of arterial hypertension which is diagnosed incidentally or run in a family as a component of disease syndromes of the genetic origin.
  • A correct diagnosis of PHEO allows the right treatment to be administered.
  • The evaluation of the secondary arterial hypertension led to the detection of the adrenal tumor.
  • Based on the clinical presentation and the tumor characteristics, on computed tomography, PHEO was suspected.
  • The patient underwent laparoscopic, right-sided adrenalectomy, and the histopathological examination definitely concurred with the diagnosis of PHEO.
  • The diagnosis toward PHEO is recommended if the patient with NF1 shows arterial hypertension.
  • Proper diagnosis and treatment protects the patient against life-threatening cardiovascular complications.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Myocardial Infarction / diagnosis. Myocardial Infarction / etiology. Neurofibromatosis 1 / complications. Pheochromocytoma / complications


54. Lai EW, Rodriguez OC, Aventian M, Cromelin C, Fricke ST, Martiniova L, Lubensky IA, Lisanti MP, Picard KL, Powers JF, Tischler AS, Pacak K, Albanese C: ErbB-2 induces bilateral adrenal pheochromocytoma formation in mice. Cell Cycle; 2007 Aug 01;6(15):1946-50
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  • [Title] ErbB-2 induces bilateral adrenal pheochromocytoma formation in mice.
  • Pheochromocytoma (PCC) is a rare catecholamine-producing tumor that arises from the adrenal medulla and is often familial.
  • In the present study, ectopic expression of an activated ErbB-2 transgene resulted in bilateral adrenal PCC.
  • Analyses of tumor samples and normal adrenal tissue revealed that levels of the Pten tumor suppressor protein were greatly reduced in PCCs, while levels of the cell cycle regulatory protein cyclin D1 were usually increased.
  • In addition, levels of phospo-AKT were increased in PCCs versus normal adrenal tissue.
  • These data establish that increased ErbB-2 growth factor receptor signaling in the adrenal medulla can lead to PCC through combined influences on Pten, AKT andcyclin D1.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Adrenal Gland Neoplasms / pathology. Cell Transformation, Neoplastic / metabolism. Cell Transformation, Neoplastic / pathology. Pheochromocytoma / metabolism. Pheochromocytoma / pathology. Receptor, ErbB-2 / metabolism


55. Cardoso CC, Bornstein SR, Hornsby PJ: Optimizing orthotopic cell transplantation in the mouse adrenal gland. Cell Transplant; 2010;19(5):565-72
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  • [Title] Optimizing orthotopic cell transplantation in the mouse adrenal gland.
  • Orthotopic cell transplantation models are important for a complete understanding of cell-cell interactions as well as tumor biology.
  • In published studies of orthotopic transplantation in the mouse adrenal gland, human neuroblastoma cells have been shown to invade and occupy the adrenal, but in these investigations a true orthotopic model was not established.
  • Here we show an orthotopic model in which transplanted cells are retained within the adrenal gland by formation of a fibrin clot.
  • To establish an appropriate technique, we used brightly fluorescent 10 microm polystyrene microspheres injected into the mouse adrenal gland.
  • When the microspheres were injected in a fibrinogen/thrombin mixture, fluorescence was confined to the adrenal gland.
  • When 3 x 10(5) cells were implanted orthotopically, by 16 days the cell mass had expanded and had invaded the cortex, whereas when 1 x 10(5) cells were used, tumor masses were much smaller.
  • When mice were sacrificed at different time points, we found that tumor growth resulting was progressive and that by 26 days cells there was extensive invasion into the cortex or almost complete replacement of the cortex with tumor cells.
  • In summary, the present orthotopic model for intra-adrenal cell transplantation is valuable for investigation of growth of neoplastic cells of both cortical and medullary origin and should be useful for future studies of cortex-medulla interactions.

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  • (PMID = 20525431.001).
  • [ISSN] 1555-3892
  • [Journal-full-title] Cell transplantation
  • [ISO-abbreviation] Cell Transplant
  • [Language] ENG
  • [Grant] United States / NIA NIH HHS / AG / AG012287-14; United States / NIA NIH HHS / AG / P01 AG020752-020006; United States / NIA NIH HHS / AG / AG020752-020006; United States / NIA NIH HHS / AG / P01 AG020752; United States / NIA NIH HHS / AG / R37 AG012287-14; United States / NIA NIH HHS / AG / R37 AG012287
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 9001-31-4 / Fibrin; 9001-32-5 / Fibrinogen; EC 3.4.21.5 / Thrombin
  • [Other-IDs] NLM/ NIHMS246503; NLM/ PMC3735364
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56. Charfi S, Ayadi L, Ellouze S, Ghorbel R, Khabir A, Gouiaa N, Bahri I, Fakhfakh I, Makni S, Sellami-Boudawra T: [Composite pheochromocytoma associated with multiple endocrine neoplasia type 2B]. Ann Pathol; 2008 Jun;28(3):225-8
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  • [Title] [Composite pheochromocytoma associated with multiple endocrine neoplasia type 2B].
  • [Transliterated title] Phéochromocytome composite associé à une néoplasie endocrine multiple de type 2B.
  • Composite tumors of the adrenal medulla are rare and have been reported in both the presence and the absence of phacomatosis.
  • Composite pheochromocytoma of the adrenal gland in multiple endocrine neoplasia 2B has not been reported so far.
  • Clinical investigations revealed a left adrenal medullary tumor and bilateral thyroid nodules.
  • Our report is the first to describe composite pheochromocytoma with multiple endocrine neoplasia 2B; this report underlines the diversity of neoplasms that could be encountered in this disease and the complex mechanisms involved in its pathogenesis.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Multiple Endocrine Neoplasia Type 2b / pathology. Pheochromocytoma / pathology


57. Elkahloun AG, Powers JF, Nyska A, Eisenhofer G, Tischler AS: Gene expression profiling of rat pheochromocytoma. Ann N Y Acad Sci; 2006 Aug;1073:290-9
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  • To compare the molecular profiles of rat and human pheochromocytomas and to identify pathways potentially involved in pathogenesis of rat pheochromocytomas, we conducted a gene expression profiling study comparing 31 pheochromocytomas obtained from the National Toxicology Program to normal adult rat adrenal medulla.
  • Downregulated genes included receptors and tumor-suppressor genes, including NF2 and Dmbt1.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Gene Expression Profiling. Pheochromocytoma / genetics

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  • (PMID = 17102098.001).
  • [ISSN] 0077-8923
  • [Journal-full-title] Annals of the New York Academy of Sciences
  • [ISO-abbreviation] Ann. N. Y. Acad. Sci.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA48017; United States / Intramural NIH HHS / /
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
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58. Iwase K, Nagasaka A, Kato K, Itoh A, Jimbo S, Hibi Y, Kobayashi N, Yamamoto H, Seko T, Miura K: Cu/Zn- and Mn-superoxide dismutase distribution and concentration in adrenal tumors. J Surg Res; 2006 Sep;135(1):150-5
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  • [Title] Cu/Zn- and Mn-superoxide dismutase distribution and concentration in adrenal tumors.
  • The tissue distribution of Cu/Zn- and Mn-superoxide dismutases (SOD) in adrenal tumors was studied by an immunohistochemical technique, and the concentrations of both SODs were measured by a sensitive sandwich enzyme immunoassay technique.
  • In the normal adrenal gland, both Cu/Zn- and Mn-SODs were localized predominantly in the reticular zone of the cortex.
  • Cu/Zn-SOD was stained clearly in the inner fascicular zone of the cortex, but not in the medulla, whereas Mn-SOD was stained weakly in the medulla.
  • In different adrenal tumors, the localization of both stained SODs reflected the origin of the tumor cell.
  • The concentrations of both SODs in the tissues of medullary tumors were lower than those in the normal adrenal gland and adrenocortical adenomas.
  • The concentration of Cu/Zn-SOD in the tumor tissue of Cushing's syndrome adenoma was higher, and that of Mn-SOD was lower than the concentrations in the normal adrenal gland.
  • The ratio of the tissue concentrations of Mn-SOD to Cu/Zn-SOD was lower in adrenal medullary tumors and Cushing's syndrome adenomas than in the normal adrenal gland and primary aldosteronism adenomas, indicating the predominance of Cu/Zn-SOD in the former, and Mn-SOD in the latter.
  • These data suggest that the localization of Cu/Zn- and Mn-SODs in adrenal tissues reflects the specificity of the adrenal cells that produce the tissue-specific hormones.
  • An investigation of changes in these enzymes in adrenal tumors may also provide useful information on adrenal tumor cell differentiation.
  • [MeSH-major] Adrenal Cortex / enzymology. Adrenal Cortex Neoplasms / metabolism. Adrenocortical Adenoma / metabolism. Superoxide Dismutase / metabolism

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  • (PMID = 16780879.001).
  • [ISSN] 0022-4804
  • [Journal-full-title] The Journal of surgical research
  • [ISO-abbreviation] J. Surg. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 1.15.1.1 / Superoxide Dismutase
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59. Ait-Ali D, Stroth N, Sen JM, Eiden LE: PACAP-cytokine interactions govern adrenal neuropeptide biosynthesis after systemic administration of LPS. Neuropharmacology; 2010 Jan;58(1):208-14
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  • [Title] PACAP-cytokine interactions govern adrenal neuropeptide biosynthesis after systemic administration of LPS.
  • We have examined induction of neuropeptide expression in adrenal medulla after treatment of mice with lipopolysaccharide (LPS), a model for septic shock, which activates both immune and stress responses in vivo.
  • Messenger RNAs encoding vasoactive intestinal polypeptide (VIP) and galanin, both modulators of steroidogenesis in neighboring adrenal cortex, are up-regulated at 24 h (eight-fold for VIP and two-fold for galanin) after LPS injection, and remain elevated for the following 24 h.
  • Up-regulation of VIP and galanin by LPS is abrogated in pituitary adenylate cyclase-activating polypeptide (PACAP)-deficient mice, suggesting an interaction between LPS, or LPS-induced cytokines, and PACAP released in adrenal medulla from the splanchnic nerve.
  • Treatment of cultured chromaffin cells with 100 nM PACAP and 10 nM tumor necrosis factor-alpha (TNF-alpha), a cytokine whose production is elevated by LPS, results in long-term synergistic up-regulation of VIP and galanin mRNA.

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  • (PMID = 19647754.001).
  • [ISSN] 1873-7064
  • [Journal-full-title] Neuropharmacology
  • [ISO-abbreviation] Neuropharmacology
  • [Language] ENG
  • [Grant] United States / Intramural NIH HHS / / Z01 AG000768-04; United States / Intramural NIH HHS / / Z01 AG000772-01; United States / NIMH NIH HHS / MH / Z01 MH002386; United States / Intramural NIH HHS / / Z01 MH002386-21
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Cytokines; 0 / Lipopolysaccharides; 0 / Neuropeptides; 0 / Pituitary Adenylate Cyclase-Activating Polypeptide; 0 / Tumor Necrosis Factor-alpha; 37221-79-7 / Vasoactive Intestinal Peptide; 88813-36-9 / Galanin
  • [Other-IDs] NLM/ NIHMS141772; NLM/ PMC2783598
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60. Kim J, Yamamoto F, Gondo S, Yanase T, Mukai T, Maeda M: 6-Deoxy-6-[131I]iodo-L-ascorbic acid for the in vivo study of ascorbate: autoradiography, biodistribution in normal and hypolipidemic rats, and in tumor-bearing nude mice. Biol Pharm Bull; 2009 Nov;32(11):1906-11
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  • [Title] 6-Deoxy-6-[131I]iodo-L-ascorbic acid for the in vivo study of ascorbate: autoradiography, biodistribution in normal and hypolipidemic rats, and in tumor-bearing nude mice.
  • Normal female rat distribution studies showed high and specific uptake of 6-deoxy-6-[(131)I]iodo-L-ascorbic acid (6-(131)IAsA) into the adrenal glands, known to highly express the ascorbate sodium-dependent vitamin C transporter-2 (SVCT-2), and the adrenal gland was clearly visualized by whole-body autoradiography.
  • Preinjection of sulfinpyrazone, a known blocker of ascorbate transport, with 6-(131)IAsA resulted in decreased uptake of radioactivity in rat adrenal glands compared to the control group, seemingly illustrating the participation of the SVCT transporter (probably the SVCT-2 subtype) in the uptake process in vivo.
  • 4-Aminopyrazolo[3,4-d]pyrimidine-induced hypolipidemic rats showed a 1.7-fold increase in adrenal uptake of radioactivity at 30 min postinjection of 6-(131)IAsA, compared to the control, with increased adrenal-to-liver and adrenal-to-kidney ratios.
  • To further characterize 6-(131)IAsA for its tumor uptake properties, biodistribution studies were also performed using male nude mice implanted with either Y-1 adrenocortical tumor cells or adrenal medulla-derived PC12 cells.
  • None of these tumors exhibited relevant uptake of 6-(131)IAsA while normal adrenal glands showed high uptake of radioactivity, suggesting that these tumors in this model have only a poor transport capacity for this agent.
  • The present study demonstrates that the use of radioiodinated 6-IAsA may help to obtain information about functional alterations in diseased adrenal glands, but it does not exhibit desirable properties as a tumor-seeking agent for ascorbic acid bioactivity.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Iodine Radioisotopes / pharmacokinetics. Lipids / blood. Neoplasms, Experimental / metabolism

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  • (PMID = 19881306.001).
  • [ISSN] 1347-5215
  • [Journal-full-title] Biological & pharmaceutical bulletin
  • [ISO-abbreviation] Biol. Pharm. Bull.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / 6-deoxy-6-iodoascorbic acid; 0 / Iodine Radioisotopes; 0 / Lipids; PQ6CK8PD0R / Ascorbic Acid
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61. 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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62. Hofland J, van Nederveen FH, Timmerman MA, Korpershoek E, de Herder WW, Lenders JW, Verhofstad AA, de Krijger RR, de Jong FH: Expression of activin and inhibin subunits, receptors and binding proteins in human pheochromocytomas: a study based on mRNA analysis and immunohistochemistry. Clin Endocrinol (Oxf); 2007 Mar;66(3):335-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: Pheochromocytomas are uncommon tumours arising from chromaffin cells of the adrenal medulla and related paraganglia.
  • [MeSH-major] Adrenal Gland Neoplasms / chemistry. Biomarkers, Tumor / analysis. Inhibin-beta Subunits / analysis. Pheochromocytoma / chemistry
  • [MeSH-minor] Activin Receptors, Type I / analysis. Activin Receptors, Type I / genetics. Activin Receptors, Type II / analysis. Activin Receptors, Type II / genetics. Adult. Blotting, Northern / methods. Chi-Square Distribution. Diagnosis, Differential. Female. Follistatin / analysis. Follistatin / genetics. Gene Expression. Humans. Immunohistochemistry. Inhibins / analysis. Inhibins / genetics. Male. Middle Aged. Proteoglycans / analysis. Proteoglycans / genetics. RNA, Messenger / analysis. Receptors, Transforming Growth Factor beta / analysis. Receptors, Transforming Growth Factor beta / genetics. Reverse Transcriptase Polymerase Chain Reaction. Statistics, Nonparametric

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  • (PMID = 17302865.001).
  • [ISSN] 0300-0664
  • [Journal-full-title] Clinical endocrinology
  • [ISO-abbreviation] Clin. Endocrinol. (Oxf)
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Follistatin; 0 / Proteoglycans; 0 / RNA, Messenger; 0 / Receptors, Transforming Growth Factor beta; 0 / inhibin beta A subunit; 0 / inhibin-alpha subunit; 145170-29-2 / betaglycan; 57285-09-3 / Inhibins; 93443-12-0 / Inhibin-beta Subunits; EC 2.7.11.30 / ACVR1B protein, human; EC 2.7.11.30 / Activin Receptors, Type I; EC 2.7.11.30 / Activin Receptors, Type II; EC 2.7.11.30 / activin receptor type II-A; EC 2.7.11.30 / activin receptor type II-B
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63. Evinger MJ, Mathew E, Cikos S, Powers JF, Lee YS, Sheikh S, Ross RA, Tischler AS: Nicotine stimulates expression of the PNMT gene through a novel promoter sequence. J Mol Neurosci; 2005;26(1):39-55
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  • Cholinergic stimulation through neuronal nicotinic receptors constitutes the primary means for neural regulation of PNMT expression in the adrenal medulla (AM).
  • [MeSH-minor] Adrenal Glands / enzymology. Animals. Base Sequence. Cattle. Cell Line, Tumor. Cells, Cultured. DNA Footprinting. Deoxyribonuclease I. Humans. Neurons / physiology. Phosphatidylethanolamine N-Methyltransferase. Receptors, Nicotinic / physiology. Recombinant Proteins / metabolism. Sequence Deletion. Transcription, Genetic / drug effects. Transfection

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  • (PMID = 15968085.001).
  • [ISSN] 0895-8696
  • [Journal-full-title] Journal of molecular neuroscience : MN
  • [ISO-abbreviation] J. Mol. Neurosci.
  • [Language] eng
  • [Grant] United States / NIGMS NIH HHS / GM / GM 46588; United States / NINDS NIH HHS / NS / NS 37685
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Receptors, Nicotinic; 0 / Recombinant Proteins; 6M3C89ZY6R / Nicotine; EC 2.1.1.- / Methyltransferases; EC 2.1.1.17 / Phosphatidylethanolamine N-Methyltransferase; EC 3.1.21.1 / Deoxyribonuclease I
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64. Huang YC, Chang CH, Wang CH, Chang JS: Pheochromocytoma complicated with severe ventricular tachycardia: report of one case. Acta Paediatr Taiwan; 2007 Sep-Oct;48(5):280-4
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  • Later, a 4x4.5x2.5 cm tumor in the right adrenal gland area was found by computed axial tomogram study.
  • Assessment of the urine catecholamine metabolites showed high levels of vanillylmandelic acid, normetanephrine and norepinephrine indicating an active adrenal pheochromocytoma produced mainly norepinephrine.
  • Although several antihypertensive drugs were used, ventricular tachycardia and Torsade de pointe still occurred on her for 3 times, each was preceded by a period of blood pressure fluctuation and burst out concomitantly at the peak of a hypertension crisis.
  • Her right adrenal gland was resected smoothly when BP was well under control.
  • Histological examination showed the adrenal medulla was full of pheochromocytoma cells.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Pheochromocytoma / complications. Tachycardia, Ventricular / etiology

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  • (PMID = 18254579.001).
  • [ISSN] 1608-8115
  • [Journal-full-title] Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi
  • [ISO-abbreviation] Acta Paediatr Taiwan
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
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65. Pollard PJ, El-Bahrawy M, Poulsom R, Elia G, Killick P, Kelly G, Hunt T, Jeffery R, Seedhar P, Barwell J, Latif F, Gleeson MJ, Hodgson SV, Stamp GW, Tomlinson IP, Maher ER: Expression of HIF-1alpha, HIF-2alpha (EPAS1), and their target genes in paraganglioma and pheochromocytoma with VHL and SDH mutations. J Clin Endocrinol Metab; 2006 Nov;91(11):4593-8
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  • These similarities between the downstream consequences of VHL inactivation and HIF dysregulation in renal cell carcinoma and PCC may explain how inactivation of the ubiquitously expressed VHL protein results in susceptibility to specific tumor types.
  • [MeSH-major] Adrenal Gland Neoplasms / metabolism. Head and Neck Neoplasms / metabolism. Hypoxia-Inducible Factor 1, alpha Subunit / metabolism. Paraganglioma / metabolism. Pheochromocytoma / metabolism. Succinate Dehydrogenase / genetics. Transcription Factors / metabolism. Von Hippel-Lindau Tumor Suppressor Protein / genetics
  • [MeSH-minor] Adrenal Medulla / metabolism. Basic Helix-Loop-Helix Transcription Factors. Gene Expression Regulation, Neoplastic. Germ-Line Mutation. Humans. Immunohistochemistry


66. Imrich R, Lukac J, Rovensky J, Radikova Z, Penesova A, Kvetnansky R, Huckova M, Vigas M, Macho L, Koska J: Lower adrenocortical and adrenomedullary responses to hypoglycemia in premenopausal women with systemic sclerosis. J Rheumatol; 2006 Nov;33(11):2235-41
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  • OBJECTIVES: To evaluate function of the hypothalamic-pituitary-adrenal (HPA) axis, adrenomedullary hormonal system (AMHS), and sympathetic noradrenergic system (SNS) in premenopausal women with systemic sclerosis (SSc).
  • Concentrations of glucose, adrenocorticotrophic hormone (ACTH), cortisol, androstenedione (ASD), dehydroepiandrosterone (DHEA), dehydroepiandrosterone sulfate (DHEAS), 17a-hydroxyprogesterone (17OHP), epinephrine (EPI), norepinephrine (NE), interleukin 1ss (IL-1ss), IL-6, and tumor necrosis factor-a (TNF-a) were analyzed in plasma.
  • [MeSH-major] Adrenal Cortex / physiology. Adrenal Medulla / physiology. Androgens / metabolism. Hypoglycemia / metabolism. Premenopause / metabolism. Progesterone / blood. Scleroderma, Systemic / metabolism


67. Erem C, Kocak M, Cinel A, Erso HO, Reis A: Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks. Saudi Med J; 2008 Jan;29(1):122-5
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  • [Title] Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks.
  • Adrenal ganglioneuromas are rare tumors originating from the neural crest tissue of the sympathetic nervous system.
  • We report a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient.
  • Abdominal CT and MRI revealed a solid round tumor approximately 4 cm in diameter on the upper pole of the right kidney.
  • Histological diagnosis of the tumor was a ganglioneuroma originating from the adrenal medulla.
  • In conclusion, this is a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient.
  • [MeSH-major] Adrenal Gland Neoplasms / complications. Dopamine / secretion. Ganglioneuroma / complications. Hypertension / etiology
  • [MeSH-minor] Adrenalectomy. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Tomography, X-Ray Computed


68. Reisch N, Walz MK, Erlic Z, Neumann HP: [Pheochromocytoma - still a challenge]. Internist (Berl); 2009 Jan;50(1):27-35
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  • Pheochromocytomas are rare, mostly benign catecholamine-producing tumors arising from the chromaffin cells of the adrenal medulla or in the paraganglia.
  • Computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen as well as functional imaging with (123)Iod-MIBG scintigraphy and (18)F-dopa positron emission tomography are used for tumor localization.
  • The therapy of choice is the endoscopic adrenal sparing surgery following preoperative alpha-blockade.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Hypertension / diagnosis. Hypertension / prevention & control. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy


69. Shyla A, Hölzlwimmer G, Calzada-Wack J, Bink K, Tischenko O, Guilly MN, Chevillard S, Samson E, Graw J, Atkinson MJ, Pellegata NS: Allelic loss of chromosomes 8 and 19 in MENX-associated rat pheochromocytoma. Int J Cancer; 2010 May 15;126(10):2362-72
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  • Pheochromocytomas are neoplasias of neural crest origin that arise from the chromaffin cells of the adrenal medulla.
  • We also analyzed additional candidate genes, that is, rat homologues of genes predisposing to human pheochromocytoma and known tumor-suppressor genes, but we found no AI.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosomes, Human, Pair 19. Chromosomes, Human, Pair 8. Cyclin-Dependent Kinase Inhibitor p27 / genetics. Frameshift Mutation. Loss of Heterozygosity. Pheochromocytoma / genetics


70. Babinska A, Sworczak K, Wisniewski P, Nałecz A, Jaskiewicz K: The role of immunohistochemistry in histopathological diagnostics of clinically "silent" incidentally detected adrenal masses. Exp Clin Endocrinol Diabetes; 2008 Apr;116(4):246-51
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  • [Title] The role of immunohistochemistry in histopathological diagnostics of clinically "silent" incidentally detected adrenal masses.
  • BACKGROUND: The detectability of adrenal incidentalomas (incidentally found adrenal tumours) in the whole population is estimated at 0.1%; 0.42% in non-endocrine patients and at 4.3% in oncologically diagnosed ones.
  • Even up to 16% of incidentalomas of adrenal glands can be malignant lesions.
  • The issue of crucial importance is the histopathological differentiation between benign lesions and malignant tumours of the adrenal cortex and medulla.
  • OBJECTIVES: To evaluate whether the immunohistochemical analysis of the expression of p53, p21, PCNA and Ki67 in the tumour's tissue can be useful in the histopathological diagnostics of adrenal incidentalomas and whether it is important for prognosis.
  • MATERIAL AND METHODS: Our series consisted of 74 tumour samples from 164 patients operated for incidentalomas.
  • RESULTS: We found a statistically significant correlation between the expression of p53, p21, Ki67 and the differential diagnosis of adrenal cortical adenoma and adrenocortical carcinoma (for proteins: p53 p=0.010, for p21 p=0.010, for Ki67 p<0.001).
  • The statistical significant correlation between PCNA protein and diagnosis of adrenal cortical adenoma and adrenocortical carcinoma was not found.
  • The statistically significant correlation between p21, PCNA proteins and the diagnosis of benign and malignant PHEOs was not estimated.
  • [MeSH-major] Adenoma / pathology. Adrenal Gland Neoplasms / pathology. Pheochromocytoma / pathology
  • [MeSH-minor] Gene Expression Regulation, Neoplastic. Humans. Immunohistochemistry. Ki-67 Antigen / genetics. Proliferating Cell Nuclear Antigen / genetics. Proto-Oncogene Proteins c-bcl-2 / genetics. Tumor Suppressor Protein p53 / genetics. p21-Activated Kinases / genetics

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  • (PMID = 18393131.001).
  • [ISSN] 0947-7349
  • [Journal-full-title] Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association
  • [ISO-abbreviation] Exp. Clin. Endocrinol. Diabetes
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / Proliferating Cell Nuclear Antigen; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Tumor Suppressor Protein p53; EC 2.7.11.1 / p21-Activated Kinases
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71. Li JS, Li SY, Zhang YX, Qiao CX, Zhou HY: [A comparative study of biological indicators of phlegm-heat syndrome and phlegm-dampness syndrome model with acute exacerbation of chronic obstructive pulmonary disease]. Zhongguo Wei Zhong Bing Ji Jiu Yi Xue; 2010 May;22(5):267-70
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  • The level of white blood cell (WBC) count, neutrophil ratio, free triiodothyronine (FT(3)), free thyroxine (FT(4)), epinephrine (E), norepinephrine (NE), cortisol (COR), C-reactive protein (CRP), tumor necrosis factor-alpha (TNF-alpha), interleukin-8 (IL-8) in blood, and TNF-alpha, IL-8, intercellular adhesion molecule-1 (ICAM-1) in broncho-alveolar lavage fluid (BALF) were determined with radioimmunology.
  • The changes in systemic inflammatory response, decrease in functional indicators of thyroid and adrenal medulla, and decline in lung function were more marked in PDs group than in PHs group.
  • [MeSH-minor] Animals. C-Reactive Protein / metabolism. Disease Models, Animal. Intercellular Adhesion Molecule-1 / metabolism. Interleukin-8 / metabolism. Lung / pathology. Lung / physiopathology. Rats. Rats, Wistar. Tumor Necrosis Factor-alpha / metabolism

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  • (PMID = 20519073.001).
  • [ISSN] 1003-0603
  • [Journal-full-title] Zhongguo wei zhong bing ji jiu yi xue = Chinese critical care medicine = Zhongguo weizhongbing jijiuyixue
  • [ISO-abbreviation] Zhongguo Wei Zhong Bing Ji Jiu Yi Xue
  • [Language] chi
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Interleukin-8; 0 / Tumor Necrosis Factor-alpha; 126547-89-5 / Intercellular Adhesion Molecule-1; 9007-41-4 / C-Reactive Protein
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72. Paik KY: [Paraganglioma of the pancreas metastasized to the adrenal gland: a case report]. Korean J Gastroenterol; 2009 Dec;54(6):409-12
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  • [Title] [Paraganglioma of the pancreas metastasized to the adrenal gland: a case report].
  • Paraganglioma is a rare neuroendocrine tumor arising from the neural crest, which includes tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and other unnamed paraganglia.
  • A well defined ovoid shape mass in left adrenal gland was suggested adenoma.
  • Microscopic examination with pancreas and adrenal gland revealed that the cells were arranged in a characteristic Zellballen pattern.
  • On the basis of these findings, we diagnosed the tumor as a paraganglioma of the pancreas and adrenal gland.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Pancreatic Neoplasms / diagnosis. Paraganglioma / diagnosis
  • [MeSH-minor] Adenoma / diagnosis. Adenoma / surgery. Aged. Chromogranin A / metabolism. Female. Humans. Phosphopyruvate Hydratase / metabolism. S100 Proteins / metabolism. Synaptophysin / metabolism. Tomography, X-Ray Computed

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  • (PMID = 20026898.001).
  • [ISSN] 1598-9992
  • [Journal-full-title] The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
  • [ISO-abbreviation] Korean J Gastroenterol
  • [Language] kor
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Chromogranin A; 0 / S100 Proteins; 0 / Synaptophysin; EC 4.2.1.11 / Phosphopyruvate Hydratase
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73. Nakamura M, Han B, Nunobiki O, Kakudo K: Adrenomedullin: a tumor progression factor via angiogenic control. Curr Cancer Drug Targets; 2006 Nov;6(7):635-43
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  • [Title] Adrenomedullin: a tumor progression factor via angiogenic control.
  • ADM is synthesized and is secreted from many mammalian tissues, including the adrenal medulla, endothelial and vascular smooth muscle cells, as well as the myocardium and central nervous system.
  • ADM has been implicated as a mediator of several diseases such as cardiovascular and renal disorders, sepsis, inflammation, diabetes and cancer.
  • ADM is also expressed in a variety of tumors, including breast, endometrial and prostate cancer.
  • ADM has been shown to be a mitogenic factor capable of stimulating growth of several cancer cell types.
  • In addition, ADM is a survival factor for certain cancer cells and an indirect suppressor of the immune response.
  • The major focus of this review will be on the role of ADM in cancer, with emphasis on its utility in diagnostic and prognostic terms, along with its relevance as a therapeutic target.

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  • (PMID = 17100569.001).
  • [ISSN] 1873-5576
  • [Journal-full-title] Current cancer drug targets
  • [ISO-abbreviation] Curr Cancer Drug Targets
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Receptors, Adrenomedullin; 0 / Receptors, Peptide; 148498-78-6 / Adrenomedullin
  • [Number-of-references] 111
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74. Machens A, Dralle H: Multiple endocrine neoplasia type 2 and the RET protooncogene: from bedside to bench to bedside. Mol Cell Endocrinol; 2006 Mar 9;247(1-2):34-40
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  • [Title] Multiple endocrine neoplasia type 2 and the RET protooncogene: from bedside to bench to bedside.
  • Although the initial characterization of the various MEN-2 associated phenotypes (familial medullary thyroid cancer, multiple endocrine neoplasia 2A and 2B) evolved at the bedside, it was at the bench where the underlying RET (REarranged during Transfection) germline mutations were identified.
  • Molecular information has revolutionized our understanding and continues to transform the clinical management of this fascinating endocrine tumor syndrome of neural crest derivation, which consists of medullary thyroid cancer, pheochromocytoma, and parathyroid hyperplasia/adenoma.
  • With the continuing expansion of our knowledge about the underlying molecular mechanisms and our growing therapeutic abilities, multiple endocrine neoplasia type 2 is gradually returning home to the bedside, closing the loop from bedside to bench to bedside.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 2a / genetics. Proto-Oncogene Proteins c-ret / genetics
  • [MeSH-minor] Adenoma / genetics. Adenoma / pathology. Adrenal Gland Neoplasms / genetics. Adrenal Gland Neoplasms / pathology. Adrenal Medulla / pathology. Age Factors. Animals. Carcinoma, Medullary / genetics. Carcinoma, Medullary / pathology. Genotype. Germ-Line Mutation. Humans. Hyperplasia. Multiple Endocrine Neoplasia Type 2b / genetics. Multiple Endocrine Neoplasia Type 2b / pathology. Neural Crest / pathology. Parathyroid Neoplasms / genetics. Parathyroid Neoplasms / pathology. Phenotype. Pheochromocytoma / genetics. Pheochromocytoma / pathology. Syndrome. Thyroid Neoplasms / genetics. Thyroid Neoplasms / pathology

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  • (PMID = 16343738.001).
  • [ISSN] 0303-7207
  • [Journal-full-title] Molecular and cellular endocrinology
  • [ISO-abbreviation] Mol. Cell. Endocrinol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Ireland
  • [Chemical-registry-number] EC 2.7.10.1 / Proto-Oncogene Proteins c-ret
  • [Number-of-references] 58
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75. Aarts M, Dannenberg H, deLeeuw RJ, van Nederveen FH, Verhofstad AA, Lenders JW, Dinjens WN, Speel EJ, Lam WL, de Krijger RR: Microarray-based CGH of sporadic and syndrome-related pheochromocytomas using a 0.1-0.2 Mb bacterial artificial chromosome array spanning chromosome arm 1p. Genes Chromosomes Cancer; 2006 Jan;45(1):83-93
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  • Pheochromocytomas (PCC) are relatively rare neuroendocrine tumors, mainly of the adrenal medulla.
  • They arise sporadically or occur secondary to inherited cancer syndromes, such as multiple endocrine neoplasia type II (MEN2), von Hippel-Lindau disease (VHL), or neurofibromatosis type I (NF1).
  • In conclusion, these data strongly suggest that chromosome arm 1p is the site for multiple tumor suppressor genes, although the potential candidate genes CDKN2C and PTPRF/LAR are not included in these regions.
  • [MeSH-major] Adrenal Gland Neoplasms / genetics. Chromosome Deletion. Chromosomes, Artificial, Bacterial. Chromosomes, Human, Pair 1 / genetics. Pheochromocytoma / genetics

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  • [Copyright] Copyright 2005 Wiley-Liss, Inc
  • (PMID = 16215979.001).
  • [ISSN] 1045-2257
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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76. 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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77. Takahashi K, Totsune K, Saruta M, Fukuda T, Suzuki T, Hirose T, Imai Y, Sasano H, Murakami O: Expression of urocortin 3/stresscopin in human adrenal glands and adrenal tumors. Peptides; 2006 Jan;27(1):178-82
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  • [Title] Expression of urocortin 3/stresscopin in human adrenal glands and adrenal tumors.
  • In the present study, we studied expression of Ucn3/SCP in the normal adrenal and adrenal tumors by radioimmunoassay and reverse transcriptase-polymerase chain reaction (RT-PCR).
  • High concentrations of immunoreactive (IR)-Ucn3 were present in the normal portions of adrenal glands (4.2+/-0.51 pmol/g wet weight, mean+/-S.E.M., n = 14), and the levels were higher than those in the brain.
  • IR-Ucn3 was also detected in the tumor tissues of aldosterone-secreting adenomas (6.2+/-0.6 pmol/g wet weight, n = 10), cortisol-secreting adenomas (5.0+/-1.2 pmol/g wet weight, n = 4), and pheochromocytomas (1.9+/-0.4 pmol/g wet weight, n = 7).
  • Reverse phase high performance liquid chromatography showed that IR-Ucn3 in normal portions of adrenal glands and aldosterone-secreting adenomas was eluted mainly in the positions of Ucn3 and SCP with several minor peaks eluting earlier.
  • The RT-PCR showed expression of Ucn3 mRNA in normal portions of adrenal gland (positive ratio; 4/4), aldosterone-secreting adenomas (3/4), cortisol-secreting adenomas (1/3) and pheochromocytomas (6/7).
  • These findings indicate that Ucn3 is produced in normal adrenal and adrenal tumors (both adrenocortical tumors and pheochromocytomas), and suggest that Ucn3 acts as an autocrine or paracrine regulator in normal adrenal and adrenal tumors.
  • [MeSH-major] Adenoma / metabolism. Adrenal Cortex / metabolism. Adrenal Gland Neoplasms / metabolism. Adrenal Medulla / metabolism. Corticotropin-Releasing Hormone / biosynthesis. Pheochromocytoma / metabolism. Urocortins / biosynthesis

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  • (PMID = 16095756.001).
  • [ISSN] 0196-9781
  • [Journal-full-title] Peptides
  • [ISO-abbreviation] Peptides
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA, Messenger; 0 / UCN3 protein, human; 0 / Urocortins; 9015-71-8 / Corticotropin-Releasing Hormone
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78. 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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79. 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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80. Al-Shammari NF, Redha E, Al Hajeri MH: Cervical neonatal neuroblastoma with recurrent SVT. Gulf J Oncolog; 2009 Jul;(6):45-57
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  • Neuroblastoma is the most common extracranial solid tumor of childhood and the third most common paediatric malignancy after leukemia and central nervous system (CNS) tumors.
  • The median age at diagnosis is 22 months.
  • They occur most commonly in the adrenal medulla (35%).
  • Neuroblastomas also occur as primary tumors in the extra-adrenal retroperitoneum in 30% of cases, in the posterior mediastinum in 20% of cases , in the neck up to 5% of cases and in the pelvis in 5% of cases.


81. Yanagisawa M, Kachi T: Inhibitory effects of adrenomedullary hormone on the induction and growth of fibrosarcoma by methylcholanthrene. Neuro Endocrinol Lett; 2005 Apr;26(2):113-20
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  • RESULTS: The incidence of tumor at 90 days after the MC injection was 8 per 35 cases (22.9%) in the control group, 12 per 36 cases (33.3%) in the AMX + AMT group, 8 per 28 cases (28.6%) in the Bil.
  • DISCUSSION: The mechanisms of effects of AMX, AMT and/or epinephrine on the tumor incidence have been discussed with reference to tumor promotion, vascular neoplasia, etc.
  • [MeSH-major] Adrenal Medulla / physiology. Epinephrine / physiology. Fibrosarcoma / pathology. Fibrosarcoma / physiopathology
  • [MeSH-minor] Adrenalectomy. Animals. Hormones / physiology. Hormones / secretion. Hormones / therapeutic use. Male. Methylcholanthrene. Rats. Rats, Wistar. Transplantation, Autologous. Tumor Burden / physiology

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  • (PMID = 15855881.001).
  • [ISSN] 0172-780X
  • [Journal-full-title] Neuro endocrinology letters
  • [ISO-abbreviation] Neuro Endocrinol. Lett.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Sweden
  • [Chemical-registry-number] 0 / Hormones; 56-49-5 / Methylcholanthrene; YKH834O4BH / Epinephrine
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82. Keating DJ, Rychkov GY, Giacomin P, Roberts ML: Oxygen-sensing pathway for SK channels in the ovine adrenal medulla. Clin Exp Pharmacol Physiol; 2005 Oct;32(10):882-7
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  • [Title] Oxygen-sensing pathway for SK channels in the ovine adrenal medulla.
  • The aim of the present study was to identify the oxygen-sensing pathway in the oxygen-sensitive sheep adrenal medullary chromaffin cell (AMCC).
  • [MeSH-major] Adrenal Medulla / physiology. Chromaffin Cells / physiology. Potassium Channels / physiology. Signal Transduction / physiology
  • [MeSH-minor] Animals. Biphenyl Compounds / pharmacology. Cell Hypoxia / physiology. Cell Line, Tumor. Dithiothreitol / pharmacology. Humans. Hydrogen Peroxide / pharmacology. Membrane Potentials / drug effects. Membrane Potentials / physiology. Mice. Onium Compounds / pharmacology. Oxidants / pharmacology. Patch-Clamp Techniques. Plasmids / genetics. Rotenone / pharmacology. Sheep. Transfection

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  • (PMID = 16173951.001).
  • [ISSN] 0305-1870
  • [Journal-full-title] Clinical and experimental pharmacology & physiology
  • [ISO-abbreviation] Clin. Exp. Pharmacol. Physiol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biphenyl Compounds; 0 / Onium Compounds; 0 / Oxidants; 0 / Potassium Channels; 03L9OT429T / Rotenone; 10182-84-0 / diphenyliodonium; BBX060AN9V / Hydrogen Peroxide; T8ID5YZU6Y / Dithiothreitol
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83. Fukumitsu N, Ashida H, Ogi S, Uchiyama M, Mori Y, Ikemoto I, Sakamoto N, Tojo K, Kawakami M: A case of ganglioneuroma in which 131I-6beta-iodomethyl-19-norcholest-5(10)-en-3beta-ol scintigraphy showed high uptake in the adrenal gland leading to a misdiagnosis. Ann Nucl Med; 2006 Jan;20(1):69-73
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  • [Title] A case of ganglioneuroma in which 131I-6beta-iodomethyl-19-norcholest-5(10)-en-3beta-ol scintigraphy showed high uptake in the adrenal gland leading to a misdiagnosis.
  • We experienced a case in which 131I-6beta-iodomethyl-19-norcholest-5(10)-en-3beta-ol (131I-adosterol) scintigraphy showed high uptake in the right adrenal gland.
  • Unexpectedly, pathological finding showed the right adrenal gland was occupied with a large ganglioneuroma.
  • This is an instructive case in which 131I-adosterol scintigraphy showed abnormal high uptake in the adrenal gland, in spite of the fact that the adrenal gland was occupied by a tumor derived from adrenal medulla.
  • [MeSH-major] 19-Iodocholesterol / analogs & derivatives. Adenoma / radionuclide imaging. Adrenal Gland Neoplasms / radionuclide imaging. Diagnostic Errors / prevention & control. Ganglioneuroma / radionuclide imaging

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  • (PMID = 16485578.001).
  • [ISSN] 0914-7187
  • [Journal-full-title] Annals of nuclear medicine
  • [ISO-abbreviation] Ann Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 30461-91-7 / 19-Iodocholesterol; 68232-36-0 / 6-iodomethylcholesterol
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84. Choi EK, Kim WH, Park KY: A case of a composite adrenal medullary tumor of pheochromocytoma and ganglioneuroma masquerading as acute pancreatitis. Korean J Intern Med; 2006 Jun;21(2):141-5
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  • [Title] A case of a composite adrenal medullary tumor of pheochromocytoma and ganglioneuroma masquerading as acute pancreatitis.
  • Composite adrenal medullary tumors, composed of both pheochromocytoma and ganglioneuroma, are extremely rare, as are pheochromocytomas masquerading as acute relapsing pancreatitis.
  • An abdominal computed tomographic scan revealed an enlarged pancreas and a 3-cm left adrenal incidentaloma.
  • An adrenalectomy was performed and a composite adrenal medullary tumor of pheochromocytoma and ganglioneuroma was confirmed during a pathologic examination.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Medulla. Ganglioneuroma / diagnosis. Neoplasms, Multiple Primary / diagnosis. Pancreatitis / etiology. Pheochromocytoma / diagnosis


85. Ilias I, Pacak K: Diagnosis and management of tumors of the adrenal medulla. Horm Metab Res; 2005 Dec;37(12):717-21
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  • [Title] Diagnosis and management of tumors of the adrenal medulla.
  • The adrenal medulla consists of chromaffin cells, the site of catecholamine biosynthesis.
  • Pheochromocytomas are chromaffin-cell tumors; 80-85 % arise from the adrenal medulla and 15-20 % arise from extra-adrenal chromaffin tissues (paragangliomas).
  • Pheochromocytomas account for 6.5 % of incidentally discovered adrenal tumors; they are found in 50 % of patients with multiple endocrine neoplasia 2A (MEN 2A) and 5-25 % of patients with von Hippel-Lindau (VHL) syndrome.
  • The diagnosis of pheochromocytoma should first be established biochemically by measuring plasma free metanephrines (the measurement of urinary fractionated metanephrines is the second choice).
  • Primary treatment for both types of tumor is surgical; chemotherapy is used for inoperable disease.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Adrenal Gland Neoplasms / therapy. Adrenal Medulla / physiopathology. Neuroendocrine Tumors / diagnosis. Neuroendocrine Tumors / therapy
  • [MeSH-minor] Humans. Multiple Endocrine Neoplasia Type 2a / diagnosis. Multiple Endocrine Neoplasia Type 2a / therapy. Neuroblastoma / diagnosis. Neuroblastoma / therapy. Pheochromocytoma / diagnosis. Pheochromocytoma / therapy. Prognosis

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  • (PMID = 16372223.001).
  • [ISSN] 0018-5043
  • [Journal-full-title] Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et métabolisme
  • [ISO-abbreviation] Horm. Metab. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 39
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86. Matoso A, Zhou Z, Hayama R, Flesken-Nikitin A, Nikitin AY: Cell lineage-specific interactions between Men1 and Rb in neuroendocrine neoplasia. Carcinogenesis; 2008 Mar;29(3):620-8
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  • [Title] Cell lineage-specific interactions between Men1 and Rb in neuroendocrine neoplasia.
  • Inactivation of multiple endocrine neoplasia (MEN) type 1 gene (Men1) results in development of multiple endocrine tumors in Men1(+/-) mice and in humans.
  • Men1 and Rb did not cooperate in tumor suppression, as demonstrated by comparable survival rates of Rb(+/-) and Men1(+/-)Rb(+/-) mice, absence of tumor growth acceleration and lack of novel neoplasms.
  • Notably, the loss of the remaining copy of the wild-type Men1 and Rb was mutually exclusive in all tumors of Men1(+/-)Rb(+/-) mice, including pituitary anterior lobe and adrenal medulla neoplasms shared by Rb- and Men1-deficient phenotypes.


87. Erem C, Ucuncu O, Nuhoglu I, Cinel A, Cobanoglu U, Demirel A, Koc E, Kocak M, Guvendi GF: Adrenal ganglioneuroma: report of a new case. Endocrine; 2009 Jun;35(3):293-6
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  • [Title] Adrenal ganglioneuroma: report of a new case.
  • Although adrenal ganglioneuroma (GN) is a rare tumor originating from the neural crest tissue of the sympathetic nervous system, detection of this tumor has increased, as imaging procedures such as ultrasonography (US) and computed tomography (CT) have become prevalent.
  • We describe a case of adrenal GN incidentally diagnosed in a 68-year-old female patient.
  • Abdominal CT and magnetic resonance imaging showed a solid oval tumor approximately 6 x 4 cm in the left adrenal gland without remarkable signs of malignancy.
  • Histological diagnosis of the tumor was a ganglioneuroma originating from the adrenal medulla.
  • Adrenal GN occurs rarely in adults and preoperative diagnosis is difficult, especially in asymptomatic cases.
  • According to our knowledge, this is the fifth case of adrenal GN in an adult patient from Turkey in English literature.
  • [MeSH-major] Adrenal Gland Neoplasms / diagnosis. Ganglioneuroma / diagnosis

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  • (PMID = 19367379.001).
  • [ISSN] 1355-008X
  • [Journal-full-title] Endocrine
  • [ISO-abbreviation] Endocrine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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88. Fukuda T, Takahashi K, Suzuki T, Saruta M, Watanabe M, Nakata T, Sasano H: Urocortin 1, urocortin 3/stresscopin, and corticotropin-releasing factor receptors in human adrenal and its disorders. J Clin Endocrinol Metab; 2005 Aug;90(8):4671-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Urocortin 1, urocortin 3/stresscopin, and corticotropin-releasing factor receptors in human adrenal and its disorders.
  • OBJECTIVE, DESIGN, AND SETTING: We examined in situ expression of Ucn and CRF receptors in nonpathological human adrenal gland and its disorders using immunohistochemistry and mRNA in situ hybridization.
  • RESULTS: Ucn immunoreactivity was localized in the cortex and medulla of nonpathological adrenal glands.
  • Ucn1 immunoreactivity was marked in the medulla, whereas Ucn3 was immunostained mostly in the cortex.
  • Both CRF type 1 and CRF2 were expressed in the cortex, particularly in the zonae fasciculata and reticularis but very weakly or undetectably in the medulla.
  • Ucn and CRF receptors were all expressed in the tumor cells of pheochromocytomas, adrenocortical adenomas, and carcinomas, but its positivity was less than that in nonpathological adrenal glands, suggesting that Ucn1, Ucn3, and CRF receptors were down-regulated in these adrenal neoplasms.
  • CONCLUSIONS: Ucn1, Ucn3, and CRF receptors are all expressed in human adrenal cortex and medulla and may play important roles in physiological adrenal functions.
  • [MeSH-major] Adrenal Cortex / physiology. Adrenal Cortex Neoplasms / physiopathology. Corticotropin-Releasing Hormone / genetics. Pheochromocytoma / physiopathology. Receptors, Corticotropin-Releasing Hormone / genetics

  • MedlinePlus Health Information. consumer health - Pheochromocytoma.
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  • (PMID = 15914529.001).
  • [ISSN] 0021-972X
  • [Journal-full-title] The Journal of clinical endocrinology and metabolism
  • [ISO-abbreviation] J. Clin. Endocrinol. Metab.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CRF receptor type 1; 0 / CRF receptor type 2; 0 / RNA, Messenger; 0 / Receptors, Corticotropin-Releasing Hormone; 0 / UCN3 protein, human; 0 / Urocortins; 9015-71-8 / Corticotropin-Releasing Hormone
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89. Corti A: Chromogranin A and the tumor microenvironment. Cell Mol Neurobiol; 2010 Nov;30(8):1163-70
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  • [Title] Chromogranin A and the tumor microenvironment.
  • Chromogranin A (CgA) is an acidic glycoprotein belonging to a family of regulated secretory proteins stored in the dense core granules of the adrenal medulla and of many other neuroendocrine cells and neurons.
  • A growing body of evidence suggests that CgA is more than a diagnostic/prognostic marker for cancer patients.
  • Indeed, results of in vitro experiments and in vivo studies in animal models suggest that this protein and its fragments can affect several elements of the tumor microenvironment, including fibroblasts and endothelial cells.
  • In this article, recent findings implicating CgA as a modulator of the tumor microenvironment and suggesting that abnormal secretion of CgA could play important roles in tumor progression and response to therapy in cancer patients are reviewed and discussed.
  • [MeSH-major] Chromogranin A / metabolism. Neoplasms / metabolism. Tumor Microenvironment

  • NCI CPTC Antibody Characterization Program. NCI CPTC Antibody Characterization Program .
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  • (PMID = 21080056.001).
  • [ISSN] 1573-6830
  • [Journal-full-title] Cellular and molecular neurobiology
  • [ISO-abbreviation] Cell. Mol. Neurobiol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromogranin A; 0 / Peptide Fragments; 126729-24-6 / vasostatin I
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90. Chen ML, Xu PZ, Peng XD, Chen WS, Guzman G, Yang X, Di Cristofano A, Pandolfi PP, Hay N: The deficiency of Akt1 is sufficient to suppress tumor development in Pten+/- mice. Genes Dev; 2006 Jun 15;20(12):1569-74
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The deficiency of Akt1 is sufficient to suppress tumor development in Pten+/- mice.
  • The tumor suppressor PTEN is frequently inactivated in human cancers.
  • Here we showed that the deficiency of Akt1 is sufficient to dramatically inhibit tumor development in Pten+/- mice.
  • Akt1 deficiency had a profound effect on endometrium and prostate neoplasia, two types of human cancer, in which PTEN is frequently mutated, and also affected thyroid and adrenal medulla tumors and intestinal polyps.
  • Even haplodeficiency of Akt1 was sufficient to markedly attenuate the development of high-grade prostate intraepithelial neoplasia (PIN) and endometrial carcinoma.
  • These results have significant implications for cancer therapy.


91. Nevo I, Sagi-Assif O, Edry Botzer L, Amar D, Maman S, Kariv N, Leider-Trejo LE, Savelyeva L, Schwab M, Yron I, Witz IP: Generation and characterization of novel local and metastatic human neuroblastoma variants. Neoplasia; 2008 Aug;10(8):816-27
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Neuroblastoma (NB) is the most commonly occurring solid tumor in children.
  • The disease usually arises in the adrenal medulla, and it is characterized by a remarkable heterogeneity in its progression.
  • Most NB patients with an advanced disease have massive bone marrow infiltration at diagnosis.
  • Currently, models consisting of metastatic and nonmetastatic cell variants of the same genetic background exist for several types of cancer; however, none exists for NB.
  • SH-SY5Y and MHH-NB-11 NB cells were inoculated orthotopically into the adrenal glands of athymic nude mice.
  • Neuroblastoma cells metastasizing to the lungs were isolated from mice bearing adrenal tumors.
  • [MeSH-major] Adrenal Gland Neoplasms / pathology. Disease Models, Animal. Lung Neoplasms / secondary. Neoplasms, Experimental / secondary. Neuroblastoma / secondary
  • [MeSH-minor] Animals. Cell Line, Tumor. Cell Proliferation / drug effects. Deferoxamine / pharmacology. Doxorubicin / therapeutic use. Drug Screening Assays, Antitumor. Flow Cytometry. Humans. Immunophenotyping. Karyotyping. Male. Matrix Metalloproteinase 2 / secretion. Matrix Metalloproteinase 9 / secretion. Mice. Mice, Inbred BALB C. Mice, Nude. Survival Rate. Xenograft Model Antitumor Assays