Sinonasal Tract Alveolar Rhabdomyosarcoma in Adults: A Clinicopathologic and Immunophenotypic Study of Fifty-Two Cases with Emphasis on Epithelial Immunoreactivity

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From: Head and Neck Pathology(Vol. 12, Issue 2)
Publisher: Springer
Document Type: Report
Length: 562 words

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To access, purchase, authenticate, or subscribe to the full-text of this article, please visit this link: Byline: Lester D. R. Thompson (1), Vickie Y. Jo (2), Abbas Agaimy (3), Antonio Llombart-Bosch (4), Gema Nieto Morales (4), Isidro Machado (5), Uta Flucke (6), Paul E. Wakely (7), Markku Miettinen (8), Justin A. Bishop (9) Keywords: Nasal cavity; Paranasal sinus neoplasms/pathology; Rhabdomyosarcoma, alveolar; Adult; Keratins; Immunohistochemistry Abstract: Sinonasal tract (SNT) alveolar rhabdomyosarcoma (ARMS) are frequently misdiagnosed, especially in adults. Fifty-two adult (a[yen]18 years) patients with SNT ARMS were reviewed and characterized by immunohistochemistry and molecular studies. Twenty-six females and 26 males (18--72 years mean 43.2 years), presented after a short duration (mean 2.6 months) with a large (mean 5.5 cm) destructive nasal cavity mass, involving multiple contiguous paranasal sites (n=46) and with cervical adenopathy (n=41). The tumors showed an alveolar, nested to solid growth pattern below an intact, but often involved (n=9) epithelium with frequent necrosis (n=37), destructive bone invasion (n=30), and lymphovascular invasion (n=25). The neoplastic cells were dyshesive and dilapidated, with crush artifacts. Rhabdoid features (n=36) and tumor cell multinucleation (n=28) were common. Mitotic counts were high (mean 17/10 HPFs). The neoplastic cells showed the following immunohistochemical positive findings: desmin (100%), myogenin (100%), MYOD1 (100%), MSA (96%), SMA (52%), CAM5.2 (50%), AE1/AE3 (36%) other positive markers included S100 protein (27%), CD56 (100%), synaptophysin (35%), and chromogranin (13%). Overall, 54% show epithelial marker reactivity. Molecular studies showed FOXO1 translocations (81%) with PCR demonstrating PAX3 in 72.7% tested. Patients presented with high stage (IV 24 III 26) and metastatic disease (lymph nodes n=41 distant metastases n=25) (IRSG grouping). Surgery (n=16), radiation (n=41) and chemotherapy (n=45) yielded an overall survival of 36.1 months (mean range 2.4--286) 18 alive without disease (mean 69.6 months) 7 alive with disease (mean 11.0 months) 1 dead without disease (63.7 months) and 26 dead with disease (mean 18.5 months). SNT ARMS frequently present in adults as a large, destructive midline mass of short symptom duration, with high stage disease. The alveolar to solid pattern of growth of cells with rhabdoid-plasmacytoid features suggests the diagnosis, but epithelial immunohistochemistry markers are present in 54% of cases, leading to misdiagnosis as carcinomas if muscle markers are not also performed. Overall survival of 36.1 months is achieved with multimodality therapy, but 64% have incurable disease (16.9 months). Mixed anatomic site (p=0.02) was a significant adverse prognostic indicator, while stage (0.06) and tumor size 5 cm (0.06) approached marginal significance. Author Affiliation: (1) 0000 0004 0445 0789, grid.417224.6, Southern California Permanente Medical Group, Department of Pathology, Woodland Hills Medical Center, 5601 De Soto Avenue, Woodland Hills, CA, 91365, USA (2) 0000 0004 0378 8294, grid.62560.37, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA (3) 0000 0000 9935 6525, grid.411668.c, Institute of Pathology, Friedrich-Alexander University Erlangen-Nuremberg, University Hospital, Erlangen, Germany (4) 0000 0001 2173 938X, grid.5338.d, University of Valencia, Valencia, Spain (5) 0000 0004 1771 144X, grid.418082.7, Instituto Valenciano de Oncologia, Valencia, Spain (6) 0000 0004 0444 9382, grid.10417.33, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands (7) 0000 0001 1545 0811, grid.412332.5, The Ohio State University Wexner Medical Center, Columbus, OH, USA (8) 0000 0004 1936 8075, grid.48336.3a, National Cancer Institute/National Institutes of Health, Bethesda, MD, USA (9) Southwestern Medical Center, University of Texas, Dallas, TX, USA Article History: Registration Date: 31/08/2017 Received Date: 13/07/2017 Accepted Date: 31/08/2017 Online Date: 05/09/2017

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Gale Document Number: GALE|A538874221