Adamantinoma-like Ewing Sarcoma of the Salivary Glands: A Newly Recognized Mimicker of Basaloid Salivary Carcinomas

Lisa M. Rooper, Vickie Y. Jo, Cristina R. Antonescu, Vania Nose, William H. Westra, Raja R. Seethala, Justin A. Bishop

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
JournalAmerican Journal of Surgical Pathology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Adamantinoma
Ewing's Sarcoma
Salivary Glands
Carcinoma
Synaptophysin
Neoplasms
Cell Biology
Neuroendocrine Carcinoma
Rosette Formation
Large Cell Carcinoma
Adenoid Cystic Carcinoma
Glandular and Epithelial Neoplasms
Submandibular Gland
Parotid Gland
Growth
Keratins
Squamous Cell Carcinoma
Adenocarcinoma
Neck
Head

Keywords

  • adamantinoma-like Ewing sarcoma
  • Ewing sarcoma
  • EWSR1
  • FLI1
  • salivary gland

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Adamantinoma-like Ewing Sarcoma of the Salivary Glands : A Newly Recognized Mimicker of Basaloid Salivary Carcinomas. / Rooper, Lisa M.; Jo, Vickie Y.; Antonescu, Cristina R.; Nose, Vania; Westra, William H.; Seethala, Raja R.; Bishop, Justin A.

In: American Journal of Surgical Pathology, 01.01.2018.

Research output: Contribution to journalArticle

Rooper, Lisa M. ; Jo, Vickie Y. ; Antonescu, Cristina R. ; Nose, Vania ; Westra, William H. ; Seethala, Raja R. ; Bishop, Justin A. / Adamantinoma-like Ewing Sarcoma of the Salivary Glands : A Newly Recognized Mimicker of Basaloid Salivary Carcinomas. In: American Journal of Surgical Pathology. 2018.
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abstract = "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.",
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