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Purpose 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.

Introduction Mammary analogue secretory carcinoma (MASC) is a recent discovered entity of salivary glands tumors, reported for first time in 2010. The presence of a translocation encodes the ETS variant transcription factor 6-neurotrophic tyrosine receptor kinase (ETV6-NTRK3) gene fusion differences MASC from other tumors. Case presentation A 68-year-old male showed a non-painful right parotid enlargement, came from dermatology service, and followed by some facial squamous cell carcinomas. A computed tomography (CT) scan showed a 1.7×1.6 cm right parotid enlargement in superficial lobe. The patient underwent a right superficial parotidectomy. The final pathology confirmed the presence of ETV6-NTRK3-positive MASC. Complete right deep parotidectomy and functional cervical emptying were performed. Discussions and conclusions It is necessary to establish an appropriated differential diagnosis between salivary gland tumors. MASC is a low-grade malignancy cancer that sometimes can evolve to a high-grade tumor that might produce local and distance dissemination. Most times, these tumors are only treated by surgical resection and evaluating by a multidisciplinary team the need of more treatments. In our case, the patient showed a primary parotid tumor, removed surgically with free edges, and being identified as MASC. We decided to underwent neck dissection and discovered a second MASC focus on cervical salivary gland; however, there was no nodal dissemination. The patient remains disease-free after 14 months from last surgery. It is important to keep studying genetic therapy targets to ETV6-NTRK3 to obtain a new therapy line to treat those cases that require.

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. 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. 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). 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.

Secretory carcinoma (SC) of salivary gland, previously known as mammary analogue secretory carcinoma, is a rare low-grade malignancy harboring a diagnostic ETV6-NTRK3 gene fusion. SC of salivary gland shares histopathological, immunohistochemical and genetic characteristics with SC of the breast. There are several previous cytomorphological characterizations of SC of salivary gland reported in the literature. The most commonly reported patterns are of epithelial clusters with papillary architectural features, or of single dispersed epithelial cells on a background of abundant histiocytes. Tumor cells exhibit vacuolated eosinophilic cytoplasm and round to oval nuclei with regular nuclear contours and inconspicuous or small nucleoli. The cytomorphology of SC may closely mimic that of acinic cell carcinoma or low-grade mucoepidermoid carcinoma. Moreover, when cohesive epithelial clusters do not appear on the smears, it may be very difficult to distinguish dispersed tumor cells from histiocytes. In this article, we review the literature pertaining to SC cytomorphology and we report a fine needle aspiration biopsy case of SC in salivary gland showing well-defined intracytoplasmic hyaline globules, a feature that has not been previously reported. This novel cytomorphological feature may be helpful in distinguishing the tumor cells of SC from histiocytes and from other low-grade salivary gland tumors.

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. Case summary 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. Conclusion The imaging and histological diagnostic challenges for MASC are discussed.

Mammary analogue secretory carcinoma is a rare salivary gland tumor that has a wide range of clinical presentation ranging from indolent, local and slowly growing tumors to a very aggressive, infiltrative and widely metastasizing forms. We report a case of an unfortunate patient whose tumor had a very quick ominous disease course with aggressive clinical and histological presentation. This case is the first case, to our knowledge, to be reported in the literature from Saudi Arabia of MASC with features of high grade transformation

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. Keywords: Mammary analogue secretory carcinoma, Parotid gland, Salivary tumour.

Aims Secretory carcinoma (SC) (synonym: mammary analogue secretory carcinoma) is a low-grade salivary gland tumour that occurs in both major and minor salivary glands. SC is known for its wide morphological, architectural and immunohistochemical spectrum, which overlaps with those of several salivary gland neoplasms, including acinic cell carcinoma (AciCC) and intercalated duct-type intraductal carcinoma (IDC) in major salivary glands, and polymorphous adenocarcinoma (PAC) in minor salivary glands. These tumours share with SC some morphological features and SOX10 immunoreactivity; also, with the exception of AciCC, they all coexpress S100 and mammaglobin. Methods and results We compared MUC4 and mammaglobin expression in 125 salivary gland carcinomas (54 genetically confirmed SCs, 20 AciCCs, 21 PACs, and 30 IDCs) to evaluate the potential of these two markers to differentiate these entities. Moderate to strong diffuse MUC4 positivity was detected in 49 SCs (90.7%), as compared with none of the IDCs and PACs. In contrast, mammaglobin was frequently expressed in SCs (30 of 36 cases; 83.3%), IDCs (24/28; 85.7%), and PACs (7/19; 36.8%). Two of three high-grade SCs lost MUC4 expression in the high-grade tumour component. No significant correlation was found between MUC4 expression and the fusion variant in SC (ETV6-NTRK versus non-ETV6-NTRK). Conclusion The results of our study identify MUC4 as a sensitive (90.7%) and specific (100%) marker for SC, with high positive (100%) and negative (93.4%) predictive values. Thus, MUC4 may be used as a surrogate for SC in limited biopsy material and in cases with equivocal morphology.

Mammary analogue secretory carcinoma (MASC) of the salivary gland is a rare tumor that was first described by Skalova et al in 2010, and since then, only a few hundred cases have been reported in the literature. Prior to Skalova’s report, MASC was histologically misclassified as acinic cell carcinoma (ACC), pleomorphic adenoma, mucoepidermoid carcinoma, or adenocarcinoma, not otherwise specified. Mammary analogue secretory carcinoma has a low incidence rate overall, accounting for less than 0.3% of all salivary gland tumors. Histopathologic and cytogenic analysis of MASC is identical to secretory carcinoma of the breast, leading to the proposed name by Skalova. The purpose of this case presentation is to describe an atypical presentation of MASC, to compare this case with the classic description of MASC, and to contrast the various features of MASC to ACC in order to improve the accuracy of future diagnoses and help guide treatment.

We present magnetic resonance imaging findings of an 11-year-old girl with a mammary analogue secretory carcinoma (MASC) of the parotid gland. MASC is a recently described tumor of the salivary glands that is genetically and histologically similar to secretory breast carcinoma. To date, a few cases have been reported in the pediatric population, with limited information of its imaging characteristics. We suggest that decreased T2 signal of the solid component of the MASC representing cellular components with associated complex cystic parts may be a helpful imaging finding and can make a substantial contribution in differentiating this new entity from other rare pediatric parotid masses. Although there are no characteristic imaging findings at this time, MASC should be considered in the differential of salivary gland tumors in the pediatric population as well.

Mammary analogue secretory carcinoma (MASC) of the salivary gland is a rare salivary cancer, histologically resembling to secretory carcinoma of the breast. In 2017 World Health Organization reported MASC is a new salivary cancer subtype. The aim of this article is to collect and analyze data about MASC, particularly clinical, histological and molecular profile, to evaluate targeted therapy effects. We discuss a case report of dramatic and durable response with entrectinib and the development of acquired resistance in an NTRK3-fusion positive salivary cancer, detected by next-generation sequencing. Next-generation sequencing as a comprehensive molecular profiling, that helps to investigate molecular profile of rare tumors and gives an opportunity to use an effective therapeutic options. Identifying ETV6-NTRK3 positive MASC provides a better prognosis for metastatic disease by using a novel effective targeted therapy with tyrosine kinase inhibitors (entrectinib, larotrectinib). Despite a durable and dramatic response, we showed an interesting case of the development of acquired resistance to tyrosine kinase inhibitors mediated by the appearance of a novel NTRK3 G623R mutation. Finally, we believe in great perspectives of comprehensive molecular profiling and targeted therapy for rare malignancies with NTRK gene fusions, including second-generation tyrosine kinase inhibitors.

This is a case of a 55-year-old female with past medical history of tobacco smoking, hypertension, and lupus who presented with a left lung nodule, found on computed tomographic scan of the chest during workup for a recently diagnosed squamous cell carcinoma in a cervical lymph node. Resection of the lung nodule showed a 2.3-cm well-defined pale-tan mass, and histologic examination showed a well-circumscribed lesion with papillary, solid, and gland-like architectural patterns, with eosinophilic secretions, areas of comedonecrosis, and increased mitotic activity. Immunohistochemical stains showed the lesion to be positive for cytokeratin 7, S100 protein, and focally positive for mammaglobin. Fluorescence in situ hybridization studies confirmed rearrangement of the ETV6 gene at 12p13.2. To date, our case is the second reported case of a primary mammary-analogue secretory carcinoma arising in the lung, and the first to show evidence of tumor necrosis: an unusual feature in an unusual location.

Mammary analogue secretory carcinoma (MASC) of the salivary gland is a newly-described, rare, malignant tumour. Few patients present with MASC of the parotid gland, so the exact characteristics, outcomes of treatment, and prognosis are unknown. The aim of this study was to analyse the clinical features and outcomes of treatment of MASC of the parotid gland in patients being treated at a single hospital. Five patients with histopathologically-confirmed MASC of the parotid gland between January 2015 and August 2018 were retrospectively enrolled. In all cases preoperative fine-needle aspiration cytology had failed to provide an accurate diagnosis. All patients underwent a macroscopically complete oncological resection. Two patients had postoperative radiotherapy (RT). On immunohistochemical examination all tumours stained for S-100 and mammaglobin but not for DOG1. There were no regional recurrences or distant metastases in any of the patients at their last follow-up. We obtained good results for patients with MASC of the parotid gland with surgical treatment and postoperative RT.

Mammary analogue secretory carcinoma is a rare neoplasm usually confused with other neoplasms in the salivary glands region. It has great similarity with the breast carcinoma. We report a case of a patient who presented with gingival submucosal bleeding and lesion, with the initial histopathological examination revealing salivary gland neoplasm of low crane. Computed tomography revealed the lesion near the tooth 27, with extension to the floor of the left maxillary sinus and to the palate mucosa. Resection of the infra-structure was performed, with a diagnosis of breast cancer secretory carcinoma in the minor salivary gland.

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 the good long-term outcomes, but T4 tumors behave significantly more aggressive and require extended treatment strategies along with close follow-ups.

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.

Mammary analog secretory carcinoma (MASC) of salivary gland is a recently described entity. Due to its rarity and cytomorphological overlap with other salivary gland tumors, it is often difficult to recognize on cytology. Here we describe three such cases with their histopathological correlation. All the three tumors arose in the parotid gland. They were misdiagnosed as mucoepidermoid carcinoma, acinic cell carcinoma and salivary duct adenocarcinoma, respectively. Final diagnosis of MASC was established on their follow-up histopathology and immunochemistry evaluation. Cytosmears of these tumors showed high cellularity with papillary architecture lying within fluid background rich in foamy macrophages. Nuclear atypia varied from minimal to marked with frequent mitosis and presence of necrosis. Cytoplasmic vacuolation was a consistent finding. Although the cytomorphological features of MASC are not specific, a diagnosis of MASC should be strongly considered in the presence of papillary architecture, prominent cytoplasmic vacuolations of the tumor cells and a background of cyst fluid. Immunohistochemistry on cell block may be done to confirm the diagnosis.

Objective The present study aimed to characterize and differentiate the ultrasonography (US) and computed tomography (CT) features of mammary analogue secretory carcinoma (MASC) and acinic cell carcinoma (AciCC). Methods and patients A total of 83 patients with clinically proven MASC and AciCC were analyzed. The following characteristics were assessed on US, CT, and magnetic resonance imaging: lesion size, shape, margin, echogenicity, echotexture, cystic components, posterior echo, vascularity, density, degree of enhancement, enhancement pattern, signal intensity (SI) on T1 and T2 weighted images (WI), hemorrhages, and lymph node enlargement. Results Similarities were observed between the imaging performance of MASC and AciCC. Differences between the two characteristics of shape on US and cystic components on CT were statistically significant. The proportion of MASC to regular shape on US (P = 0.006) and cystic components on CT (P = 0.027) was significantly higher than that of AciCC. Regular shape on US had the highest sensitivity in the identification of MASC and AciCC, while regular shape on US + cystic component on CT had the highest specificity. Conclusions The shape on US and cystic components on CT are key characteristics for distinguishing MASC and AciCC.

Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagnosed at five years of age. In children the gender distribution has been reported as female to male ratio of 1:1.2. SC is generally considered a low-grade malignancy with characteristic morphological features and immunological profile. SC also harbors ETV6-NTRK3 fusion (t(12;15)(p13:q25)). Surgical resection with or without lymph node dissection is the standard treatment, with generally favorable clinical outcomes. Here we present a single institution case series of six patients (ages 9-21) with SC and a review of the previously described pediatric cases. Our small series showed male predominance in pediatric patients with predominantly low-grade and stage tumors. All cases underwent complete surgical resections and when follow up is available there was no evidence of recurrences or metastases. To the best of our knowledge, this is the only SC case series comprised exclusively of pediatric and youth patients.

Mammary analogue secretory carcinoma (MASC) is a rare malignant tumour of the salivary glands, with only few cases reported in the literature to date. Initial preoperative staging is crucial for all patients with an oral malignancy to visualize the tumour, detect lymph node or distant metastases and plan therapeutic interventions. In the case presented herein, radiological imaging revealed a tumour of the right hard palate with suspected positive contralateral lymph nodes. Therefore, local tumour resection comprising hemimaxillectomy and bilateral neck dissection was performed. The diagnosis of MASC was finally based on characteristic histopathological and immunohistochemical findings, such as S100 protein and mammaglobin positivity. The diagnosis of MASC may be challenging, as such findings lack specificity. To confirm the diagnosis, molecular genetic examinations may be performed to detect a highly specific ETV6-NTRK3 fusion gene. Depending on the results of these examinations, surgery, alone or combined with adjuvant radiation or chemoradiation, is the recommended approach. In summary, MASC should be treated similarly to other low-grade salivary gland tumours, such as acinic cell carcinoma, as they exhibit biological and histopathological similarities.

Salivary gland secretory carcinoma, also termed mammary analogue secretory carcinoma (MASC), is a recently described salivary gland neoplasm with characteristic histomorphologic findings similar to those of secretory carcinoma of the breast and harboring recurrent ETV6–NTRK3 fusions. Recent findings have expanded the molecular profile of salivary gland secretory carcinoma to include multiple novel ETV6 fusion partners, including RET, MET, and MAML3. Here, we report a case of cystic MASC with cribriform and papillary histology harboring two gene fusions, ETV6–RET and EGFR–SEPT14, identified by targeted RNA sequencing. The presence of the rearrangements was confirmed by FISH, RT-PCR, and Sanger sequencing. This is the first EGFR–SEPT14 fusion reported in secretory carcinoma as a single event or in association with an ETV6 rearrangement. This finding adds to the expanding molecular profile of this tumor entity, and may translate into novel treatment strategies.

We present a 72 years old male with a left nasal cavity (mammary analogue) secretory carcinoma (SC) which exhibited classical morphological features on light microscopical examination, diffuse strong S100 and mammoglobin positivity on immunohistochemistry, and ETV6-NTRK3 gene fusion on next generation sequencing (NGS) analysis. Unusual features of this tumor are expression of p63 and DOG1 on immunohistochemistry and the atypical junction between Exon 4 of the ETV6 gene and Exon 14 of the NTRK3 gene.

We present a case of a 63-year-old male with an 8-year history of a left-sided cystic facial mass which recurred despite multiple drainage procedures. Imaging findings showed a cystic mass in the left parotid gland and it was surgically resected. Pathology confirmed it to represent a mammary analogue secretory carcinoma, a relatively newly described entity. This case illustrates that mammary analogue secretory carcinoma can masquerade as a cyst within the parotid gland. Keywords: Parotid gland, Mammary analogue secretory carcinoma, Acinic cell carcinoma, ETV6-NTRK3 translocation, Magnetic resonance imaging, Cystic mass

Mammary analogue secretory carcinoma (MASC) has recently been recognized as a salivary gland tumour that is characterized by the ETV6–NTRK3 fusion gene. A case of locally advanced MASC of the parotid gland in a 67-year-old man is presented here. The patient visited the hospital due to a large right infra-auricular mass, which had been enlarging gradually over a period of 2 years. Contrast-enhanced computed tomography (CT) demonstrated a multilocular mass, 75 × 63 mm in size, containing a fluid component with non-uniform contrast effects in the interior portion. The mass had invaded the orbit, skull base, and parapharyngeal space. The patient had neither lymph node nor distant metastasis. The tumour showed tubular and ductal proliferation lined by a single layer of neoplastic cuboidal cells with clear foamy cytoplasm. Characteristic hobnail cells were observed. Expression of ETV6–NTRK3 fusion transcript in the tumour tissues was confirmed by RT-PCR. The final diagnosis was MASC (T4bN0M0, stage IVB). The patient received cetuximab together with radiotherapy at a total dose of 66 Gy. After treatment, CT showed a slightly reduced tumour volume, indicating stable disease. More than 56 months after treatment, the patient remains alive with no remarkable change in the tumour.

Mammary analog secretory carcinoma is a primary salivary gland neoplasm with histologic, immunophenotypic, and molecular features identical to those of secretory carcinoma of the breast. Similar neoplasms have now been reported to occur in various nonmammary sites including the parotid gland, submandibular gland, sinuses, lip, skin, thyroid gland, and lung. We report, to our knowledge, the first example of a primary vulvar neoplasm with pathologic features identical to secretory carcinoma of the breast and an ETV6-NTRK3 fusion.