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Slow-Growing Painless Parotid Nodule in a Woman in Her 40s

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 44-year-old woman presented with an asymptomatic enlarging right parotid mass of about 6 years’ duration. She had no prior medical or surgical history and took no medications. Ultrasonography demonstrated a solid and hypoechoic mass, 1.5 × 1.0 × 0.9 cm, in the inferior right parotid gland with minimal vascularity and without extraparotid extension. No lymph node abnormality was observed. Fine-needle aspiration was performed, and the smear was extremely cellular and showed sheets and clusters of bland polygonal epithelial cells admixed with bright pink filamentous matrix. Because of the size and location, extracapsular dissection II was carried out.1 The final pathology of the lesion showed histologic findings compatible with salivary gland carcinoma, with microcystic and papillary architecture (Figure, A and B). Abundant bubbly secretion was present within these microcystic spaces, and this material was positive for periodic acid–Schiff (Figure, C). Immunohistochemically, there was positivity for epithelial membrane antigen, cytokeratin (CK) 7 and CK19, S100 protein, and gross cystic disease fluid protein 15 (GCDFP-15). Stains for gastrointestinal stromal tumors 1 (DOG1) and p63 were negative. The tumor appeared to be contained within the surgical excision margins. There was perineural invasion without evidence of neoplastic emboli in the peritumoral lymphovascular spaces. The patient underwent a right parotidectomy I to IV1 and a level II superselective neck dissection.

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C. Secretory carcinoma

Secretory carcinoma (SC) is a new tumor entity described in 20102 that harbors the recurrent translocation t(12;15)(p13;q25) resulting in the ETV6-NTRK3 gene fusion. The same cytogenetic abnormality was described earlier in secretory breast carcinoma.3

Although several case series have noted a male predominance, reported cases are divided fairly evenly by sex. The mean (range) patient age in these reported cases was 44.7 (14-77) years.4 The majority (70%) of tumors were located in the parotid gland, while the remainder occurred in a large diversity of locations, including the submandibular gland (7%), soft palate, buccal mucosa, base of tongue, and lips. The mean tumor size was 2.11 cm. The most common presentation was a slowly enlarging and painless nodule, and the median duration of symptoms was 4.25 years.4

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Article Information

Corresponding Author: Michele Ori, MD, Section of Otorhinolaryngology, Department of Surgery and Biomedical Sciences, University of Perugia, Sant’Andrea delle Fratte, 06129 Perugia (PG), Italy (michele.ori357@gmail.com).

Published Online: May 14, 2020. doi:10.1001/jamaoto.2020.0675

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also wish to thank Alessandra Calcinai, MD, Department of Pathology, Azienda USL Toscana Centro, St Joseph Hospital, Empoli, FI, Italy, for her scientific support. No compensation was received for this contribution.

References
1.
Quer  M , Guntinas-Lichius  O , Marchal  F ,  et al.  Classification of parotidectomies: a proposal of the European Salivary Gland Society.   Eur Arch Otorhinolaryngol. 2016;273(10):3307-3312. doi:10.1007/s00405-016-3916-6PubMedGoogle ScholarCrossref
2.
Skálová  A , Vanecek  T , Sima  R ,  et al.  Mammary analogue secretory carcinoma of salivary glands, containing the ETV6-NTRK3 fusion gene: a hitherto undescribed salivary gland tumor entity.   Am J Surg Pathol. 2010;34(5):599-608. doi:10.1097/PAS.0b013e3181d9efccPubMedGoogle ScholarCrossref
3.
Tognon  C , Knezevich  SR , Huntsman  D ,  et al.  Expression of the ETV6-NTRK3 gene fusion as a primary event in human secretory breast carcinoma.   Cancer Cell. 2002;2(5):367-376. doi:10.1016/S1535-6108(02)00180-0PubMedGoogle ScholarCrossref
4.
Sethi  R , Kozin  E , Remenschneider  A ,  et al.  Mammary analogue secretory carcinoma: update on a new diagnosis of salivary gland malignancy.   Laryngoscope. 2014;124(1):188-195. doi:10.1002/lary.24254PubMedGoogle ScholarCrossref
5.
Chênevert  J , Duvvuri  U , Chiosea  S ,  et al.  DOG1: a novel marker of salivary acinar and intercalated duct differentiation.   Mod Pathol. 2012;25(7):919-929. doi:10.1038/modpathol.2012.57PubMedGoogle ScholarCrossref
6.
Sams  RN , Gnepp  DR .  P63 expression can be used in differential diagnosis of salivary gland acinic cell and mucoepidermoid carcinomas.   Head Neck Pathol. 2013;7(1):64-68. doi:10.1007/s12105-012-0403-2PubMedGoogle ScholarCrossref
7.
Skálová  A .  Mammary analogue secretory carcinoma of salivary gland origin: an update and expanded morphologic and immunohistochemical spectrum of recently described entity.   Head Neck Pathol. 2013;7(1)(suppl 1):S30-S36. doi:10.1007/s12105-013-0455-yPubMedGoogle ScholarCrossref
8.
Majewska  H , Skálová  A , Stodulski  D ,  et al.  Mammary analogue secretory carcinoma of salivary glands: a new entity associated with ETV6 gene rearrangement.   Virchows Arch. 2015;466(3):245-254. doi:10.1007/s00428-014-1701-8PubMedGoogle ScholarCrossref
9.
Sun  L , Thorson  T , Zhu  R ,  et al.  A case report of parotid mammary analogue secretory carcinoma and reviews.   Int J Surg Case Rep. 2019;55:88-91. doi:10.1016/j.ijscr.2019.01.004PubMedGoogle ScholarCrossref
10.
Boon  E , Valstar  MH , van der Graaf  WTA ,  et al.  Clinicopathological characteristics and outcome of 31 patients with ETV6-NTRK3 fusion gene confirmed (mammary analogue) secretory carcinoma of salivary glands.   Oral Oncol. 2018;82:29-33. doi:10.1016/j.oraloncology.2018.04.022PubMedGoogle ScholarCrossref
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