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Adamantinoma-like Ewing Sarcoma of the Salivary Glands

A Newly Recognized Mimicker of Basaloid Salivary Carcinomas

Rooper, Lisa M., MD*; Jo, Vickie Y., MD; Antonescu, Cristina R., MD; Nose, Vania, MD, PhD§; Westra, William H., MD; Seethala, Raja R., MD; Bishop, Justin A., MD*,#

The American Journal of Surgical Pathology: February 2019 - Volume 43 - Issue 2 - p 187–194
doi: 10.1097/PAS.0000000000001171
Original Articles

Adamantinoma-like Ewing sarcoma (ALES) is a rare tumor that demonstrates the EWSR1-FLI1 translocation characteristic of Ewing sarcoma despite overt epithelial differentiation including diffuse expression of cytokeratins and p40. Most cases of ALES described to date have occurred in the head and neck where they can mimic a wide range of small round blue cell tumors. Because distinguishing ALES from basaloid salivary gland carcinomas can be particularly difficult, we analyzed a series of 10 ALESs that occurred in the salivary glands with the aim of identifying features that allow for better recognition of this entity. The salivary ALESs included 8 parotid gland and 2 submandibular gland tumors in patients ranging from 32 to 77 years (mean: 52 y). Nine were initially misclassified as various epithelial neoplasms. Although these tumors displayed the basaloid cytology, rosette formation, infiltrative growth, and nuclear monotony characteristic of ALES, peripheral palisading and overt keratinization were relatively rare in this site. Salivary ALESs not only displayed positivity for AE1/AE3, p40, and CD99, but also demonstrated a higher proportion of synaptophysin reactivity than has been reported for nonsalivary ALESs. These morphologic and immunohistochemical findings make ALES susceptible to misclassification as various other tumors including basal cell adenocarcinoma, adenoid cystic carcinoma, squamous cell carcinoma, NUT carcinoma, large cell neuroendocrine carcinoma and myoepithelial carcinoma. Nevertheless, monotonous cytology despite highly infiltrative growth and concomitant positivity for p40 and synaptophysin can provide important clues for consideration of ALES, and identification of the defining EWSR1-FLI1 translocations can confirm the diagnosis.

*Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD

Department of Pathology, Brigham and Women’s Hospital

§Department of Pathology, Massachusetts General Hospital, Boston, MA

Department of Pathology, Memorial Sloan-Kettering Cancer Center

Department of Pathology, Icahn School of Medicine at Mount Sinai Hospital, New York, NY

Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA

#Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Justin A. Bishop, MD, Department of Pathology, University of Texas Southwestern Medical Center, MC 9073, 5323 Harry Hines Blvd., Dallas, TX 75390-9073 (e-mail:

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