Mammary Analogue Secretory Carcinoma: The First Submandibular Case Reported Including Findings on Fine Needle Aspiration Cytology

Abstract

We present the first case (male, 35 years old) of a mammary analogue secretory carcinoma occurring in a submandibular gland and document findings on fine needle aspiration cytology. On histology, the tumor displayed characteristic features: circumscribed nodules composed of bland, pink to light red neoplastic cells with low proliferative/mitotic activity arranged in tubular, vaguely cribriform, and microcystic structures containing Periodic acid Schiff-positive, diastase-resistant secretory material. Immunohistochemistry showed strong and diffuse positivity for cytokeratin 7, S100 protein, and vimentin, as well as moderate to strong immunoreactivity for c-kit in the majority of tumor cells. A rearrangement of the ETV6 gene on fluorescence in situ hybridization was documented. The patient underwent an ipsilateral selective (levels I-IV) neck dissection which showed metastasis in 3 out of 36 lymph nodes (levels 1-3). Adjuvant radiotherapy was administered. No local recurrence or metastatic disease has been detected during a follow up period of 28 months.

Unmasking MASC: Bringing to Light the Unique Morphologic, Immunohistochemical and Genetic Features of the Newly Recognized Mammary Analogue Secretory Carcinoma of Salivary Glands

“…In the initial description of MASC, the vast majority (13 of 16) of cases arose in the parotid gland [1], but subsequent publications have shown that while the parotid gland is the most common site of origin (n = 50), MASC may also arise in the oral cavity (n = 27), submandibular gland (n = 8), and accessory parotid gland (n = 1) [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]. In the oral cavity, the lip (n = 9), soft palate (n = 8), and buccal mucosa (n = 5) are the most commonly affected subsites [1,2,4,7,11,12].…”

“…In addition, the authors found that these salivary gland tumors shared the immunophenotype and, remarkably, the characteristic ETV6-NTRK3 translocation of secretory breast carcinoma, and they accordingly designated them ''mammary analogue secretory carcinomas'' (MASCs). Since the initial description, MASC has been increasingly recognized, with 70 additional reported cases [2][3][4][5][6][7][8][9][10][11][12][13][14][15].…”

Salivary Gland Tumor “Wishes” to Add to the Next WHO Tumor Classification: Sclerosing Polycystic Adenosis, Mammary Analogue Secretory Carcinoma, Cribriform Adenocarcinoma of the Tongue and Other Sites, and Mucinous Variant of Myoepithelioma

“…These tumors were termed mammary analogue secretory carcinomas (MASC). Since this initial report, there have been approximately 100 additional cases published [21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40]. These have been previously diagnosed as unusual variants of acinic cell carcinoma, adenoid cystic carcinoma, cystadenocarcinoma, mucoepidermoid carcinoma or adenocarcinoma, NOS.…”

Mammary Analogue Secretory Carcinoma of Salivary Gland Origin: An Update and Expanded Morphologic and Immunohistochemical Spectrum of Recently Described Entity

“…Moreover, 8 new cases of MASC have been documented as case reports within last two years [15][16][17][18][19][20].…”