CTNNB1 (?-Catenin)-altered Neoplasia: A Review Focusing on Soft Tissue Neoplasms and Parenchymal Lesions of Uncertain Histogenesis.

Agaimy A, Haller F (2016)


Publication Type: Journal article

Publication year: 2016

Journal

Publisher: Lippincott, Williams & Wilkins

Book Volume: 23

Pages Range: 1-12

Journal Issue: 1

DOI: 10.1097/PAP.0000000000000104

Abstract

?-catenin (CTNNB1) is a key regulatory molecule of the Wnt signaling pathway, which is important for tissue homeostasis and regulation of cell proliferation, differentiation, and function. Abnormal stabilization and nuclear accumulation of ?-catenin as a consequence of missense mutations or alternative molecular mechanisms occurs at a high frequency in a variety of epithelial cancers. In mesenchymal neoplasia, the role of ?-catenin has been traditionally considered limited to desmoid-type fibromatosis. However, the spectrum of ?-catenin-driven (?-catenin-altered) neoplasia of mesenchymal origin has been steadily widening to include, in addition to desmoid tumors, a variety of benign and intermediate-biology neoplasms of soft tissue (intranodal palisaded myofibroblastoma), head and neck (juvenile nasopharyngeal angiofibroma and sinonasal hemangiopericytoma/glomangiopericytoma), and ovarian (microcystic stromal tumor) origin. In addition, several old and newly reported distinctive site-specific ?-catenin-driven parenchymal neoplasms of uncertain histogenesis have been well characterized in recent studies, including solid-pseudopapillary neoplasm of the pancreas and its recently described ovarian counterpart, sclerosing hemangioma of lung and calcifying nested stromal-epithelial tumor of the liver. This review addresses the most relevant pathobiological and differential diagnostic aspects of ?-catenin-altered neoplasms with emphasis on site-specific histologic and biological variations. In addition, the morphologic overlap and analogy as well as distinctness between these uncommon tumors will be presented and discussed. Furthermore, a note is made on association of some of these lesions with hereditary tumor syndromes, in particular with the familial adenomatous polyposis coli.

How to cite

APA:

Agaimy, A., & Haller, F. (2016). CTNNB1 (?-Catenin)-altered Neoplasia: A Review Focusing on Soft Tissue Neoplasms and Parenchymal Lesions of Uncertain Histogenesis. Advances in Anatomic Pathology, 23(1), 1-12. https://dx.doi.org/10.1097/PAP.0000000000000104

MLA:

Agaimy, Abbas, and Florian Haller. "CTNNB1 (?-Catenin)-altered Neoplasia: A Review Focusing on Soft Tissue Neoplasms and Parenchymal Lesions of Uncertain Histogenesis." Advances in Anatomic Pathology 23.1 (2016): 1-12.

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