A Study of gata3 and phox2b expression in tumors of the autonomic nervous system.
Affiliation
*Department of Histopathology, The Christie Hospital †Institute of Cancer Sciences, The University of Manchester, Manchester, UK ‡Department of Pathology, Continuum Health Partners, Beth Israel Medical Center §Department of Pathology, Albert Einstein College of Medicine, Manhattan Campus, New York, NY ∥Department of Pathology, University of Maryland School of Medicine ¶Department of Anatomic Pathology, University of Maryland Medical Center, Baltimore, MD.Issue Date
2013-05-24
Metadata
Show full item recordAbstract
Autonomic neurons and chromaffin cells, which constitute the autonomic nervous system, are derived from a common progenitor from the neural crest, and its development is controlled by a network of transcription factors, including the master regulator, Phox2b, and its downstream, Gata3. Anti-Phox2b and anti-Gata3 antibodies were applied to a total of 77 autonomic nervous system tumors, including 35 paragangliomas, 21 pheochromocytomas, 9 neuroblastomas, 4 ganglioneuroblastomas, and 8 ganglioneuromas, as well as their potential morphologic mimics, including tumors of the small round cell tumor group, neuroendocrine carcinomas of lung and gastrointestinal tract (carcinoid tumors/neuroendocrine tumors, large cell neuroendocrine carcinomas, and small cell carcinomas), Merkel cell carcinomas, benign and malignant tumors of thyroid, parathyroid, and adrenal cortex, and malignant melanomas. A variety of nonendocrine/neuroendocrine carcinomas were also studied. Gata3 expression was seen in 89% of paragangliomas, 95% of pheochromocytomas, and all neuroblastomas, ganglioneuroblastomas, and ganglioneuromas, as well as in all parathyroid tumors, a majority of urothelial and mammary carcinomas, and a subset of squamous cell carcinomas, but all other tumors were negative. Phox2b expression was seen in all neuroblastomas, ganglioneuroblastomas, and ganglioneuromas and in 40% of paragangliomas, but pheochromocytomas and all other tumors were negative. Gata3 is a highly reliable marker for paragangliomas, pheochromocytomas, and neuroblastic tumors to distinguish from their simulators. This is an additional utility for this marker, which is used for the diagnosis of urothelial and mammary carcinomas. Phox2b is also highly specific, but its low sensitivity to paragangliomas and pheochromocytomas would limit the utility only to neuroblastic tumors.Citation
A Study of gata3 and phox2b expression in tumors of the autonomic nervous system. 2013: Am J Surg PatholJournal
American Journal of Surgical PathologyDOI
10.1097/PAS.0b013e318289c765PubMed ID
23715162Type
ArticleLanguage
enISSN
1532-0979ae974a485f413a2113503eed53cd6c53
10.1097/PAS.0b013e318289c765