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Salivary Intraductal Carcinoma Arising within Intraparotid Lymph Node: A Report of 4 Cases with Identification of a Novel STRN-ALK Fusion

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Abstract

Intraductal carcinoma (IDC) is a rare salivary gland tumor that is considered analogous to ductal carcinoma in-situ of the breast, demonstrating a complex neoplastic epithelial proliferation surrounded by a continuous layer of presumed non-neoplastic myoepithelial cells. It is subcategorized into intercalated duct, apocrine, and hybrid subtypes based on morphologic and immunohistochemical features, with frequent NCOA4-RET and TRIM27-RET fusions, respectively, seen in intercalated duct and hybrid tumors. However, as an expanding clinicopathologic spectrum of IDC has been documented, controversy has emerged as to whether this tumor type is best defined by its intraductal growth pattern or distinctive molecular and immunophenotypic differentiation. Here, we further explore the nature of IDC by evaluating four cases that arose within intraparotid lymph nodes. These intercalated-duct phenotype tumors with diffuse S100 protein expression demonstrated a crowded and complex epithelial proliferation arranged in cystic, cribriform, and micropapillary architecture, surrounded by an intact myoepithelial cell layer, and were completely intranodal. Of two tumors with tissue available for molecular analysis, one demonstrated a NCOA4-RET fusion and one harbored a STRN-ALK fusion that is novel to IDC. Not only does the intranodal presence of IDC present a challenging differential diagnosis, but the complex nature of this proliferation within lymph node tissue raises questions as to whether the myoepithelial component of IDC is actually non-neoplastic in nature. Furthermore, identification of a STRN-ALK fusion expands the genetic spectrum of IDC and adds to evidence of an emerging role for ALK in salivary gland tumors. Further attention to the nature of the myoepithelial cells and documentation of alternate fusion events in IDC may inform continued discussion about its appropriate classification.

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Funding

This study was funded in part by the Jane B. and Edwin P. Jenevein, MD Endowment for Pathology at UT Southwestern Medical Center.

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Authors and Affiliations

  1. Departments of Pathology and Oncology, The Johns Hopkins Hospital, Baltimore, MD, USA

    Lisa M. Rooper

  2. Southern California Permanente Medical Group, Department of Pathology, Woodland Hills Medical Center, Woodland Hills, CA, USA

    Lester D. R. Thompson

  3. Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA

    Jeffrey Gagan & Justin A. Bishop

  4. ProPath, Dallas, TX, USA

    Bahram R. Oliai

  5. Department of Pathology, Department of Laboratory Medicine and Pathobiology, University Health Network, University of Toronto, Toronto, ON, Canada

    Ilan Weinreb

  6. Department of Pathology, MC 9073, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, TX, 75390-9073, USA

    Justin A. Bishop

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  1. Lisa M. Rooper
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Correspondence to Justin A. Bishop.

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Rooper, L.M., Thompson, L.D.R., Gagan, J. et al. Salivary Intraductal Carcinoma Arising within Intraparotid Lymph Node: A Report of 4 Cases with Identification of a Novel STRN-ALK Fusion. Head and Neck Pathol 15, 179–185 (2021). https://doi.org/10.1007/s12105-020-01198-0

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