Abstract：
:Pheochromocytomas and paragangliomas are neuroendocrine neoplasias of neural crest origin. Genetic mutations that are characterized in other human neoplasms are rarely seen in these tumors. About 10% of the patients with pheochromocytomas and paragangliomas present with a family history of von Hippel-Lindau disease (VHL), Multiple endocrine neoplasia type 2 (MEN2), one of the three familial paraganglioma syndromes (PGL; PGL1, PGL3, PGL4), or neurofibromatosis type 1 (NF1). In an even higher percentage, a genetic predisposition is involved in the development of these tumors. The genes of hereditary tumor syndromes such as the aforementioned ones are also ideal to study the molecular pathogenesis in the sporadic counterparts. Many studies have been undertaken to identify important secondary genetic events that contribute to the tumorigenesis of pheochromocytoma or paraganglioma, but a comprehensive review of these data is lacking. Recent findings of CGH and LOH studies provided new starting points to unravel the pathogenesis and progression of these tumors. This review presents an overview of our current understanding of the molecular pathogenesis of pheochromocytoma and paraganglioma.
journal_name
Endocr Patholjournal_title
Endocrine pathologyauthors
Dannenberg H,Komminoth P,Dinjens WN,Speel EJ,de Krijger RRdoi
10.1385/ep:14:4:329keywords:
subject
Has Abstractpub_date
2003-01-01 00:00:00pages
329-50issue
4eissn
1046-3976issn
1559-0097pii
EP:14:4:329journal_volume
14pub_type
杂志文章,评审abstract：:Thirteen cases of fatal sporadic medullary carcinoma of the thyroid (MCT) that killed the patient within 1 year after diagnosis were compared with 12 nonfatal MCTs with excellent prognosis. Males predominated in fatal cases whereas the reverse was true in MCTs with good prognosis. The two groups of tumors showed gener...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02914490
更新日期：1993-03-01 00:00:00
abstract：:Hurthle cell carcinoma of the thyroid is generally considered to be a subtype of follicular carcinoma. We report a case of a small solitary usual-type papillary carcinoma of the thyroid, with metastatic tumor in cervical lymph nodes. The lymph node tumor consisted of both tall-cell papillary carcinoma and Hurthle cell...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-002-0002-y
更新日期：2002-10-01 00:00:00
abstract：:We describe a consistently present, previously unrecognized, population of monocytes in pheochromocytomas and paragangliomas. Although sustentacular cells are generally recognized as a common component of these tumors, differential immunohistochemical staining for CD163 and S100 shows that monocytes can in fact be mor...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-019-9575-6
更新日期：2019-06-01 00:00:00
abstract：:Insulin-like growth factor II mRNA-binding protein 3 (IMP3) is an oncofetal protein highly expressed in fetal tissue and malignant tumors but rarely found in adult benign tissues. In various tumors, IMP3 expression is correlated with increased tumor aggressiveness and reduced overall survival. To our knowledge, IMP3 e...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-009-9096-9
更新日期：2010-03-01 00:00:00
abstract：:Hashimoto's thyroiditis is the archetype of organ-specific autoimmune disease. The key pathogenetic feature is the activation of thyroid-specific T-cells by properly presented endogenous thyroid antigens. There is strong indication that thyrocytes themselves present self-antigens, based on the finding of antigen prese...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739959
更新日期：1998-10-01 00:00:00
abstract：:Breast carcinomas often metastasize into various organs, most commonly into the lung and rather infrequently into the pineal gland. There were only 20 cases of the latter recorded until 1950. Currently, the total number of reported cases, including this report, is 74. At Sunnybrook Health Science Centre, between 1984 ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02921371
更新日期：1994-03-01 00:00:00
abstract：:CD44 and orthopedia homeobox protein (OTP) expressions have shown to be predictive of overall survival in pulmonary carcinoid (PC) tumours. The scope of the present study was to validate their role in PC patients and investigate potential application in clinical practice. Data was collected from patients presenting to...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-016-9459-y
更新日期：2017-03-01 00:00:00
abstract：:The recent increase in the detection of papillary thyroid carcinoma (PTC) has been influenced by the finding of incidental tumours. To this group, carcinomas measuring less than 1 cm (the so-called microcarcinomas) as well as those above 1 cm belong. Analyzing a case series from our own experience, this paper focuses ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-014-9323-x
更新日期：2014-09-01 00:00:00
abstract：:Breast metastases from nonmammary tumors are rare. We report here the first case of pancreatic neuroendocrine carcinoma metastatic to the breast in a patient with possible multiple endocrine neoplasia type 1. The diagnosis was supported by histological examination, immunohistochemistry, and ultrastructural analysis. T...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02738793
更新日期：1997-10-01 00:00:00
abstract：:Pleomorphic leiomyosarcoma (PLMS) of the adrenal gland is a rare tumor in an unusual location. A primary PLMS of the left adrenal gland is reported in a 59-yr-old Mexican woman who presented progressive flank pain and weight loss. The tumor measured 16 cm in diameter, showed markedly pleomorphic and osteoclast-like gi...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:16:1:075
更新日期：2005-04-01 00:00:00
abstract：:Folliculostellate (FS) cells are present in the anterior pituitary and have important regulatory functions including controlling hormone release from other anterior pituitary cells. FS cells do not usually express neuroendocrine genes such as chromogranin A (CgA). We analyzed transcriptional regulation and gene expres...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:16:3:173
更新日期：2005-10-01 00:00:00
abstract：:Neuroendocrine pancreatic tumors (PanNETs) are graded on the basis of their proliferative activity. Cytological samples are commonly the only samples available, but the determination of Ki-67 in cytology and its reliability as a measure of tumor mitotic activity is not well settled. We have retrospectively reviewed al...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-016-9455-2
更新日期：2016-12-01 00:00:00
abstract：:Although most papillary thyroid carcinomas behave as low-grade neoplasms and are generally associated with a good prognosis, some subgroups of these neoplasms represent more aggressive variants. In order to determine if differences in the behavior of these papillary carcinomas were related to expression of growth fact...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739931
更新日期：1997-01-01 00:00:00
abstract：:The presence, distribution, and morphological appearance of S-100 protein-immunoreactive cells in the human hypophysis were studied by immunocytochemistry. One hundred and twelve nonadenomatous pituitaries from fetuses to adults and pituitaries affected by several lesions including metastases, acute infarcts, and lymp...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02921341
更新日期：1992-03-01 00:00:00
abstract：:Objective. Information on angiogenesis in parathyroid pathology is scanty and in particular no data are available in parathyroid carcinomas. The aim of this study was to analyze angiogenesis as microvascular density (MVD) in parathyroid neoplastic progression from normal gland to adenoma and carcinoma. Methods. Sectio...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:17:2:175
更新日期：2006-07-01 00:00:00
abstract：:The functional differentiation of pituitary cells and adenomas follows the combination of transcription factors and co-factors in three cell lineages [growth hormone-prolactin-thyroid-stimulating hormone lineage, adrenocorticotrophic hormone (ACTH)/pro-opiomelanocortin (POMC) lineage, and follicular stimulating hormon...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-008-9014-6
更新日期：2008-04-01 00:00:00
abstract：:Somatostatin and its analogs have been included in experimental treatment protocols for advanced pancreatic adenocarcinoma based on their known antisecretory and antiproliferative properties. Somatostatin receptor type 2 (sstr2A) mediates antiproliferative actions of somatostatin and has the strongest affinity to the ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:12:2:147
更新日期：2001-07-01 00:00:00
abstract：:This study is aimed to investigate the BRAF (V600E) and TERT promoter mutation profile of 50 pediatric papillary thyroid carcinomas (PTCs) to refine their clinicopathological correlates. The median age at the time of surgery was 16 years (range, 6-18). No TERT promoter mutations were identified in this series. The BRA...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-016-9420-0
更新日期：2016-06-01 00:00:00
abstract：:Insulin-like growth factor mRNA binding protein 3 (IMP3) is an mRNA-binding protein that regulates transcription of insulin-like growth factor II affecting cell proliferation during embryogenesis. It is highly expressed in carcinomas of the pancreas, stomach, colon, rectum, kidneys, uterine cervix, lung, and ovary. Th...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-009-9079-x
更新日期：2009-10-01 00:00:00
abstract：:Activating mutations of GNAq protein in a hotspot at codon 209 have been recently described in uveal melanomas. Since these neoplasms share with thyroid carcinomas a high frequency of MAP kinase pathway-activating mutations, we hypothesized whether GNAq mutations could also play a role in the development of thyroid ca...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-010-9129-4
更新日期：2010-12-01 00:00:00
abstract：:Metallothionein-3 (MT-3) is an intracellular, low molecular weight protein mainly distributed in the central nervous system but also in various peripheral organs and several types of human neoplasms. However, details of MT-3 expression have not been examined in human adrenal cortex and its disorders. The mRNA levels o...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-013-9280-9
更新日期：2014-09-01 00:00:00
abstract：:Though most paragangliomas arise as sporadic tumors, the recent advantages in the genetic screening revealed that about 30 % of paragangliomas are linked to hereditary mutations, such as those involving SDH genes. A 22-year-old woman carrying a left main bronchus tumor underwent surgery in our institution. Her past me...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-016-9422-y
更新日期：2016-12-01 00:00:00
abstract：:Nodular corticotrope hyperplasia is a rare pathology causing Cushing's syndrome owing to a primary pituitary disease or ectopic CRH production. In this study, we evaluated the laboratory and pathological findings and results of transsphenoidal pituitary surgery in four patients with Cushing's disease. Dynamic tests of...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02914989
更新日期：1995-04-01 00:00:00
abstract：:A case of ectopic thymic tissue of the thyroid in a 30-year-old woman with Graves' disease is reported. The thyroidectomy specimen, removed because of failure of medical therapy, showed a continuous track of thymic tissue extending from the center of the gland to the capsule along an interiobular septum. This finding ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02915305
更新日期：1993-09-01 00:00:00
abstract：:A 31-year-old woman was incidentally found to have a large right adrenal mass by computed tomography imaging and underwent a workup that included endocrinological evaluation and positron emission tomography imaging. Laboratory results revealed the mass to be non-functioning. Imaging studies revealed a 9-cm heterogeneo...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-008-9013-7
更新日期：2007-01-01 00:00:00
abstract：BACKGROUND:The diffuse sclerosing variant of papillary thyroid carcinoma (DSV-PTC) is an uncommon tumor making up about 2% of all papillary thyroid carcinomas. Previous studies have not comprehensively evaluated these tumors in a large series of patients. DESIGN:Twenty-two cases of DSV-PTC diagnosed between 1970 and 2...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:16:4:331
更新日期：2005-01-01 00:00:00
abstract：:The purpose of this study is to describe a case of concurrent medullary and papillary thyroid carcinoma (MTC and PTC) and cutaneous melanoma and to analyze BRAF(V600E) mutation in plasma and tissues. We report the clinical history and the laboratory, imaging, and histopathological findings of a 47-year-old man affecte...
journal_title：Endocrine pathology
pub_type： 杂志文章,评审
doi：10.1007/s12022-014-9303-1
更新日期：2014-09-01 00:00:00
abstract：:In the majority of cases, Cushing's disease is the result of a small basophilic corticotroph microadenoma with an average size of less than 5 mm. Transsphenoidal microsurgery can cure patients with Cushing's disease; however, selective removal of the lesion requires precise preoperative localization. In this article, ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739828
更新日期：1999-06-01 00:00:00
abstract：:The cribriform morular variant of papillary thyroid carcinoma (CMV-PTC) is a rare morphologic entity that is associated with familial adenomatous polyposis (FAP). We report a case of a young lady with an incidentally discovered right-sided neck nodule on ultrasonography with a diagnosis of CMV-PTC confirmed on thyroid...
journal_title：Endocrine pathology
pub_type： 杂志文章,评审
doi：10.1007/s12022-013-9274-7
更新日期：2014-09-01 00:00:00
abstract：:A case of pancreatic acinar cell tumor (ACC) is presented in a 10-year-old boy. The tumor manifested clinically with Cushing's syndrome, high serum adrenocorticotropic hormone (ACTH) and cortisol concentrations. In addition, excessive serum levels of alpha-fetoprotein (AFP) were detected. Surgical resection was not po...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-007-0018-4
更新日期：2007-07-01 00:00:00
