BACKGROUND: The diagnosis of salivary gland secretory carcinoma (SC) in fine-needle aspiration specimens is challenging because its low-grade nature makes it difficult to differentiate it from various benign or malignant salivary gland neoplasms. Currently, the gold standard is demonstration of ETV6-NTRK3 fusion gene. However, the decision for ordering this costly molecular testing can be facilitated by the correct recognition of its cytomorphological features. The aim of the review was to determine the accuracy of fine-needle aspiration cytology (FNAC) in diagnosis of salivary gland SC. The secondary objective was to recognize varied cytomorphological patterns, characteristic features of SC and differentiate it from other neoplasms.
METHODS: PubMed/MEDLINE, Science Direct, Embase, Cochrane review, and PROSPERO databases were searched for studies having the following key search terms: (\"secretory carcinoma of salivary gland\" OR \"mammary analogue secretory carcinoma of salivary gland\") AND (\"Cytology\" OR \"Cytological features\" OR \"aspirate\" OR \"cytodiagnosis\") published in the time frame of 2010 to June 2023. Studies reporting cytological features of the salivary gland tumors which were confirmed/diagnosed as SC on molecular investigation, were included in the systematic review. Finally, seventeen studies reporting a total of 45 cases were included in the metanalysis.
RESULTS: The sensitivity of the FNAC in diagnosing SC in salivary gland is 27.7% (95% CI: 16.6-42.5%). The LR+ (positive likelihood ratio) was 0.654 (0.344-1.245), LR- (negative likelihood ratio) was 1.023 (0.538-1.946), and diagnostic odds ratio was 0.421 (0.129-1.374). The molecular testing and/or immunohistochemistry performed on cell block increased the diagnostic accuracy.
CONCLUSIONS: Recognition of subtle cytomorphological patterns, i.e., papillary formation, clusters, and singly dispersed cells along with presence of fine intracytoplasmic vacuolations were the characteristic findings in majority of cases, confirmed with diagnostic molecular profiling. This may be helpful in identification of this rare entity with limited published literature and help in increasing diagnostic accuracy.

Secretory carcinoma (SC) is a rare salivary gland tumor and has been recently included in the fourth edition of the World Health Organization classification of head and neck tumors. To understand the histopathologic findings and clinical behavior of mammary analogue secretory carcinoma (MASC) of the submandibular gland in a 23 year old female. MASC is an intriguing and rare malignant salivary gland tumor first described in 2010. It shares histologic, immunohistochemical and genetic features with secretory carcinoma of the breast. The clinical behavior of MASC ranges from slowly growing tumors to aggressive tumors that can cause widespread metastasis. Many cases of MASC were discovered in archived cases previously classified as pleomorphic adenoma, acinic cell carcinoma, mucoepidermoid carcinoma, and adenocarcinoma. They are only a few reported in submandibular gland. MASC is a newly recognized variant of salivary gland malignancy. Further research is needed to better delineate its overall prevalence and to define an appropriate treatment algorithm for this new clinical entity.

Secretory carcinoma (SC) is a recently recognized type of salivary gland tumor characterized by t(12;15) (p13;q25) translocation resulting in an ETV6-NTRK3 gene fusion. Most SCs are located in a main salivary gland, and primary sinonasal secretary carcinoma is rare. We describe three cases of primary SC in the sinonasal cavity with high-grade transformation (HGT) in one case, and the first case in the pharynx. All tumors comprised slightly atypical cells with solid, tubular, microcystic growth patterns. The case with HGT included two components with distinct sharp boundaries and comedo necrosis, high mitotic figures and obvious cellular atypia. Tumor cells were positive for vimentin, S100, and Gata-3 and negative for p63 and DOG-1. Three cases showed nuclear staining of pan-TRK and one showed cytoplasmic staining. All cases harbored ETV6 gene rearrangement, and ETV6-NTRK3 gene fusion was detected in three cases. Most patients were treated with radical resection and adjuvant therapy. After excision, all remained tumor-free for 65-164 months (medium 98.5 months). SC in the sinonasal cavity and pharynx is a low-grade malignant tumor with histologic features overlapping those of other salivary gland tumors. Immunohistochemical analysis and fluorescence in situ hybridization are useful techniques for its differential diagnosis.

Background: Mammary analogue secretory carcinoma (MASC) is characterized by similar histologic, immunohistochemical, and molecular features with breast secretory carcinoma. MASC usually occurs in adults. Case report: A 4-year-old boy presented with a right infra-auricular mass. Features of the tumor include solid, tubular, and papillary growth patterns, with homogenous eosinophilic secretions inside microcystic structures. Immunohistochemical stains showed strong, diffuse staining for CK7, S100, pan-TRK protein. P63 was positive in a peripheral pattern. Fluorescence in situ hybridization (FISH) analysis showed the characteristic ETV6-NTRK3 gene fusion. Conclusion: Typical histological, immunohistochemical, and molecular features are present in MASC occurring early in childhood.

Mammary Analogue Secretory Carcinoma (MASC) is a rare pathology of the salivary gland, most commonly involving the parotid gland. The objective of this study was to identify the characteristic features of MASC and its treatment outcomes. A retrospective review of 12 patients with histological diagnosis of MASC, who were managed between 2010 to 2019, was carried out at the Shaukat Khanum Memorial Cancer Hospital & Research Centre, Lahore. Their mean age was 34±16 years. There were 9 (75%) male and 3 (25%) female patients. Painless slow growing swelling was the most common presenting symptom. All the patients had undergone surgical excision with or without neck dissection, followed by adjuvant treatment. Of these, six patients had T2 tumours, while four had cervical lymph node metastasis. The mean follow-up period was 23±21 months. Local recurrence was seen in one patient. MASC is considered a low-grade tumour with good prognosis which can be treated with curative intent of surgery followed by radiotherapy effectively.

BACKGROUND: Mammary analogue secretory carcinoma is a rare malignant tumor of the salivary glands that typically involves the major glands. The aim of the current study is to report a rare case of mammary analogue secretory carcinoma that presented with left cervical lymphadenopathy.
METHODS: A 59-year-old lady presented with left cervical lymphadenopathy. Tissue biopsy and immunohistochemistry revealed metastatic carcinoma, favoring ovarian origin. Staging workup was performed and, ultimately, the patient was treated as having a carcinoma of unknown primary. After showing partial response to therapy, left side neck dissection was performed. Based on better assessment of the histologic picture and a broader panel of immunohistochemistry performed on the excision specimen, the final diagnosis was that of mammary analogue secretory carcinoma.
CONCLUSIONS: Mammary analogue secretory carcinoma is usually an indolent salivary gland carcinoma, with the majority of patients presenting with a slow-growing, painless mass measuring approximately 2 cm in size, and a reported duration ranging from 2 months to several years. In certain cases, pain and facial paralysis have been reported. It could also be found incidentally during radiologic assessment for thyroid illness or routine dental screening.
CONCLUSIONS: Diagnosing mammary analogue secretory carcinoma is challenging, and this should be in the differential diagnosis list of metastatic carcinomas to cervical lymph nodes.

Mammary analogue secretory carcinomas (MASCs) of the parotid gland are considered as low-grade malignancies with good clinical outcome but lacking data regarding prognostic factors. We performed meta-analysis assessing prognostic factors for disease-free survival (DFS) and overall survival (OS) in 256 patients with MASCs of the parotid gland. A total of 73 studies have met the inclusion criteria and 76.3% of patients were seen with T1 and T2 tumors and negative neck nodes. Lymph node metastasis (57.4%) and distant recurrences (46.2%) were particularly found in T4 tumors (p < 0.001). DFS at 5 and 10 years was 77.9% and 47.2% compared to 88.1% and 77.2% for OS at the same time points. Male sex, T3-T4 tumors, and recurrent disease represented independent worse prognosticators for survival outcome. Altogether, parotid gland MASCs show good long-term outcome, but T4 tumors behave significantly more aggressive and require extended treatment strategies along with close follow-ups.

OBJECTIVE: Secretory carcinoma (SC) of the salivary gland, formerly known as mammary analogue secretory carcinoma, is an uncommon and fairly newly described low grade malignant neoplasm of the salivary gland. Given the small number of cases reported in the literature to date, treatment guidelines are scarce. This study aimed to describe the clinical characteristics of SC, discuss prior management strategies, and provide recommendations for future treatment.
METHODS: We performed a systematic review of all the cases of SC reported in the literature since it was first recognized in 2010. Using Pubmed, Crossref, and Google Scholar, we identified all articles reporting cases of SC.
RESULTS: We identified 657 cases of SC in 109 articles. In addition, we provided 2 new cases, for a total of 659 cases in 110 articles. To our knowledge, this is the largest review of cases of SC in the literature to date. We summarized the clinical characteristics of SC, as well as the nodal status, clinical management, recurrence rate, and death rate.
CONCLUSIONS: SC occurs on average in middle age (with a large age range), presents most often initially as localized disease without metastasis, and has a low but not insignificant recurrence rate. Deaths have been reported. The generalized recommendations for treatment of SC are in line with those of other low-grade salivary gland malignancies.

OBJECTIVE: This retrospective study aimed to investigate the radiological features of head and neck mammary analogue secretory carcinoma (MASC) and systematically review previous publications and 11 new cases.
METHODS: We included patients with pathologically proven MASCs with preoperative CT or MRI images, including 11 patients from our hospital and 29 patients from 28 publications extracted after screening 645 abstracts. Two board-certified radiologists reviewed and evaluated all radiological images. The frequency of metastasis during the follow-up period in tumors with well- and ill-defined margins was compared using a Fisher\'s exact test.
RESULTS: The median age at diagnosis was 52.5 years (range, 7-78 years; 20 males). Of the 40 patients, those in their 50 s were the most common (10/40, 25.0%), and the main tumor site was the parotid gland (27/40, 42.5%). Characteristic radiological features included high intensity on T1WI in the cystic components and tumors frequently showed \"papillary and cystic,\" which showed a projection into the cystic components, or \"non-papillary and cystic\" morphology, which did not show projection of the solid components. Tumor metastasis was found in 10/35 patients (28.6%) during the follow-up period, with a significant difference in frequency between the tumors with well- and ill-defined margin (well-defined (4/26) vs. ill-defined (6/9); P = 0.0074).
CONCLUSIONS: MASCs are newly recognized malignant tumors. Characteristic T1WI high intensity on MRI and predominant cystic morphology may reflect its unique histological profile. Ill-defined tumor margin status was related to frequent metastasis. Awareness of these characteristic radiological features can assist radiologists in better detection of this novel entity.

BACKGROUND: Mammary analogue secretory carcinoma (MASC) is a rare low-grade malignant salivary gland tumor. The morphological and immunohistochemical features of MASC closely resemble those of breast secretory carcinoma. The key characteristics of the lesion are a lack of pain and slow growth. There is no obvious specificity in the clinical manifestations and imaging features. The diagnosis of the disease mainly depends on the detection of the MASC-specific ETV6-NTRK3 fusion gene.
METHODS: This report describes a rare case of a 32-year-old male patient who presented with a gradually growing lesion that was initially diagnosed as breast-like secretory carcinoma of the right parotid gland. Imaging and histological investigations were used to overcome the diagnostic difficulties. The lesion was managed with right parotidectomy, facial nerve preservation, biological patch implantation to restore the resulting defect, and postoperative radiotherapy. On postoperative follow-up, the patient reported a mild facial deformity with no complications, signs of facial paralysis, or Frey\'s syndrome.
CONCLUSIONS: The imaging and histological diagnostic challenges for MASC are discussed.