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Intraductal carcinoma of the salivary gland with NCOA4-RET: expanding the morphologic spectrum and an algorithmic diagnostic approach.

Hum Pathol

August 2021

Pathology Service, Massachusetts General Hospital, 02114 USA; Department of Otolaryngology, Massachusetts Eye and Ear, 02114 USA; Department of Pathology, Harvard Medical School, Boston, MA, 02115 USA. Electronic address:

After the publication of the 2017 World Health Organization Classification of Head and Neck Tumours, there has been increasing interest in the classification of newly categorized intraductal carcinomas. Intraductal carcinoma (IC) is an indolent tumor, typically arising in the parotid gland, with an intact myoepithelial layer and a cystic, papillary, often cribriform architecture. Early studies of IC identified a heterogeneous group of molecular alterations driving neoplasia, and recent studies have defined three primary morphological/immunohistochemical variants, subsequently linking these morphologic variants with defined molecular signatures.

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Introduction: Warthin's tumor, also known as papillary cystadenoma lymphomatosum, monomorphic adenoma, or adenolymphoma, is a benign cystic tumor of the salivary glands containing abundant lymphocytes and lymph node-like stroma. It is named after the pathologist Aldred Scott Warthin, who described two cases in 1929.

Objective: The aim of this study is to analyze the pathogenesis of Warthin's tumor.

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To investigate the clinicopathological features of mammary analogue secretory carcinoma (MASC) of salivary glands, and its diagnosis, differential diagnosis, immunohistochemistry and molecular pathology. Seventeen cases of MASC were enrolled, with 9 cases of salivary acinar cell carcinoma and 18 cases of adenoid cystic carcinoma as control groups from Nanjing General Hospital from 1997 to 2014 were included in this retrospective study, combined with immunohistochemistry and molecular detection of ETV6-NTRK3 gene fusion. All cases were histologically reviewed with immunohistochemical staining (EnVision) for S-100 protein, SOX10, GATA3, CD117 expression in each group.

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Article Synopsis
  • Papillary cystadenocarcinoma (PCAC) is a rare tumor of the salivary glands that mainly grows in cystic forms and presents diagnostic challenges due to its similarities with other cystic papillary tumors.
  • A case of PCAC in a 55-year-old male was identified through fine needle aspiration cytology, showing mild atypical features, but further examination revealed more aggressive characteristics.
  • The report highlights both the cytological and histological features of PCAC, emphasizing the need for awareness of its potential for metastasis despite its classification as a low-grade malignant tumor.
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Psammoma bodies are infrequent in salivary gland aspirates. We present a case of papillary cystic acinic cell carcinoma with many psammoma bodies and discuss the diagnostic pitfalls with other salivary gland tumors. A 24-year-old woman presented with a 5-month history of a gradually enlarging, painless lump in the left side of the face.

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