Endobronchial pulmonary angiomatoid fibrous histiocytoma: two cases with EWSR1-CREB1 and EWSR1-ATF1 fusions

The American Journal of Surgical Pathology
Khin ThwayCyril Fisher

Abstract

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biological potential, predominantly occurring in the extremities of children and young adults. It has only recently been reported as a primary lung tumor. We describe 2 cases arising endobronchially harboring EWSR1 gene rearrangements by fluorescence in situ hybridization and, respectively, EWSR1-CREB1 and EWSR1-ATF1 gene fusions by reverse transcription polymerase chain reaction. Histologically, both tumors showed classical features of AFH, comprising multiple nodules of bland spindle to epithelioid cells surrounded by lymphoplasmacytic inflammation and at least a partial fibrous capsule. Both tumors showed focal but strong desmin immunoreactivity, with focal pancytokeratin and epithelial membrane antigen in 1 case. The lung is now a recognized site of AFH occurrence, but tumors arising here can be associated with different gene fusions. It is important to recognize AFH in the pulmonary region, as its behavior at other sites is generally relatively indolent; however, it may be mistaken for metastatic or more aggressive primary lung tumors. It is likely that cases of AFH in the lung may have been previously missed because of their morphologic ...Continue Reading

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Citations

Jan 19, 2013·Medical Oncology·Monica CantileGerardo Botti
May 1, 2015·Archives of Pathology & Laboratory Medicine·Khin Thway, Cyril Fisher
May 16, 2017·The American Journal of Surgical Pathology·Patrice DesmeulesCristina R Antonescu
Apr 8, 2014·Histopathology·Maria-Rosa GhignaVincent Thomas-de-Montpreville
Jun 8, 2018·The American Journal of Surgical Pathology·Alison L CheahJohn R Goldblum
Jul 28, 2019·Journal of Clinical Pathology·Geoffroy BoulleAudrey Mansuet-Lupo
Nov 30, 2019·Virchows Archiv : an International Journal of Pathology·Christian KoelscheGunhild Mechtersheimer
Sep 24, 2020·International Journal of Surgical Pathology·Raima A MemonSameer Al Diffalha
Apr 30, 2017·International Journal of Surgical Pathology·Carlos N Prieto-GranadaJames Mueller
Aug 14, 2012·Advances in Anatomic Pathology·Rajiv M Patel, Steven D Billings
Mar 2, 2019·Journal of Cardiothoracic Surgery·Wobbe BoumaTheo J Klinkenberg
Dec 8, 2020·British Journal of Neurosurgery·Jiangwei DingTao Sun
Mar 14, 2021·Thoracic Cancer·Shuji MishimaKimihiro Shimizu
Aug 21, 2019·Pediatric Neurosurgery·Michael D WhiteStephanie Greene

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