Salivary Gland-type Tumors Research Articles

A Case Series of a Rare Parotid Gland Oncocytic Mucoepidermoid Carcinoma

Abstract Introduction/Objective We analyzed two cases of oncocytic mucoepidermoid carcinoma (OMEC) of the parotid gland which highlight the diagnostic challenges of oncocytic salivary gland neoplasms. OMEC’s are a rare type of salivary gland tumor commonly arising from the parotid gland that can easily be mistaken as benign. Proper pathologic diagnosis of these masses is critical, as it guides therapeutic intervention and has prognostic significance. Methods/Case Report In this case series, two patients were analyzed retrospectively for unilateral parotid masses. Results (if a Case Study enter NA) In a 67-year-old female, an MRI and biopsy were performed. Only a differential diagnosis could be provided, which favored oncocytoma or oncocytic carcinoma. Due to this uncertainty, a complete excision of the mass was performed. Considering the cytomorphology, immunohistochemistry (CK5/6 and P63 in oncocytes), and mucicarmine special stain highlighting scattered mucocytes, the patient was diagnosed with a low- grade OMEC. In our second case, an 80-year-old female, FNA of a posterior left ear mass 1 year prior revealed a low- grade oncocytic proliferation. With the presence of a left parotid mass one year later, additional imaging was performed and showed increased T2 hyperintensity on MRI, which caused concern for the clinicians. A superficial limited parotidectomy was performed and a differential of oncocytoma versus oncocytic variant of mucoepidermoid carcinoma was rendered. Further tumor characterization with fluorescence in situ hybridization (FISH) demonstrated a MAML2 translocation which guided diagnosis of OMEC. Conclusion With approximately 30% of all malignant salivary gland tumors being constituted by mucoepidermoid carcinoma, the ability to accurately diagnose even this rare subtype, oncocytic mucoepidermoid carcinoma, is of significant importance and relevance to pathologists in many practice settings. Herein we demonstrate the utility of H&E morphology, immunohistochemistry, and MAML2 FISH in diagnosing OMEC in the setting of oncocytic salivary gland lesions. Future avenues, including tumor molecular profiling, will aid in elucidating clinicopathologic characteristics of this entity.

Primary salivary gland-type tumor of the lung and bronchoscopic therapy. A valid option for lung parenchyma preservation? Case report

Primary salivary gland-type tumor of the lung and bronchoscopic therapy. A valid option for lung parenchyma preservation? Case report

Diagnostic utility and sensitivities of matrix Gla protein (MGP), TRPS1 and GATA3 in breast cancer: focusing on metastatic breast cancer, invasive breast carcinoma with special features, and salivary gland-type tumours

Matrix Gla protein (MGP) and trichorhinophalangeal syndrome type 1 (TRPS1) have recently emerged as novel breast-specific immunohistochemical (IHC) markers, particularly for triple-negative breast cancer (TNBC) and metaplastic carcinoma. The present study aimed to validate and compare the expression of MGP, TRPS1 and GATA binding protein 3 (GATA3) in metastatic breast carcinoma (MBC), invasive breast carcinoma (IBC) with special features, including special types of invasive breast carcinoma (IBC-STs) and invasive breast carcinoma of no special type with unique features, and mammary and non-mammary salivary gland-type tumours (SGTs). Among all enrolled cases, MGP, TRPS1 and GATA3 had comparable high positivity for ER/PR-positive (p=0.148) and HER2-positive (p=0.310) breast carcinoma (BC), while GATA3 positivity was significantly lower in TNBC (p<0.001). Similarly, the positive rates of MGP and TRPS1 in MBCs (99.4%), were higher than in GATA3 (90.9%, p<0.001). Among the IBC-STs, 98.4% of invasive lobular carcinomas (ILCs) were positive for all three markers. Among neuroendocrine tumours (NTs), all cases were positive for TRPS1 and GATA3, while MGP positivity was relatively low (81.8%, p=0.313). In the neuroendocrine carcinoma (NC) subgroup, all cases were positive for GATA3 and MGP, while one case was negative for TRPS1. All carcinomas with apocrine differentiation (APOs) were positive for GATA3 and MGP, while only 60% of the cases demonstrated moderate staining for TRPS1. Among mammary SGTs, MGP demonstrated the highest positivity (100%), followed by TRPS1 (96.0%) and GATA3 (72.0%). Positive staining for these markers was also frequently observed in non-mammary SGTs. Our findings further validate thehigh sensitivity of MGP and TRPS1 in MBCs, IBC-STs, andbreast SGTs. However, none of these markers arecapable of distinguishing between mammary and non-mammary SGTs.

SALTT study: A retrospective analysis of 111 SAlivary gland tumors of Lung and Tracheobronchial Tree

IntroductionPrimary pulmonary salivary gland-type tumours (PPSGT) are rare lung neoplasms arising from submucosal seromucinous glands in the central airway. Methods and resultsWe retrospectively analysed the clinicopathological features of 111 PPSGTs diagnosed at our institute between 2003 and 2021. The mean age at diagnosis was 43.8 years(range 6–78 years) and a male-to-female ratio of 2:1. On imaging, 92 % of cases had centrally located tumours and 37.3 % were early stage. The histopathological types included 70 cases (63 %) of mucoepidermoid carcinoma (MEC), 31 cases (27.7 %) of adenoid cystic carcinoma (ADCC), two cases of myoepithelial carcinoma, one case each of acinic cell carcinoma (ACC), clear cell carcinoma (CCC), epithelial myoepithelial carcinoma (EMC) and 5 others [including adenocarcinoma of minor salivary gland origin(n = 3), carcinoma with sebaceous differentiation(n = 1) and poorly differentiated carcinoma of salivary gland type(n = 1)]. The size of the tumours found in the resection specimens ranged from 1 cm to 13 cm, with an average size of 4.9 cm. High-risk attributes such as lymphovascular invasion (LVI), perineural invasion (PNI), pleural involvement, positive resection margins, and nodal metastasis were identified in 15.3 %, 15.3 %, 13.6 %,15.2 % and 6.7 % of cases, respectively. These attributes were found to be more frequent in ADCC than in MEC. Surgery was the main treatment modality [68/84 (80 %) cases]. ADCC cases had more recurrence and distant metastasis than MEC cases. The 3- year overall-survival (OS) and recurrence-free survival(RFS) were better in patients with age lesser than 60 years(p-value <0.0001), low pT stage (p-value 0.00038) and lower grade of MEC(p-value-0.0067). ConclusionIt is crucial to have an acquaintance with the morphologic spectrum and immunophenotypic characteristics of PPSGT to recognize them in this unusual location. In tandem, it is crucial to differentiate them from conventional primary non-small cell lung carcinoma, as the management protocols and prognostic implications differ significantly.

Pulmonary puzzles: salivary gland-type tumors of the lung and their metastatic equivalents

SummaryIn the last decade, the understanding of lung neoplasms, particularly rare salivary gland-type tumors (SGT), has deepened significantly. This review intends to spotlight the latest findings, particularly emphasizing the differentiation between primary and metastatic SGTs in the lung.

Bronchial oncocytic carcinoma in an adult: a case report and literature review

BackgroundLung salivary-type tumors originating from bronchial submucosal glands are rare, only four types of salivary gland-type tumors are listed in 2015 WHO classification of lung tumors. Here, we report a rare case of oncocytic carcinoma (OC) in the right main bronchus.Case presentationA 34-year-old man presented to our hospital with a two-month history of recurrent hemoptysis and with one month of inspiratory dyspnea. Pulmonary function tests showed mild restrictive ventilatory dysfunction and severe diffusion dysfunction. Furthermore, the flow volume loop showed a variable extra-thoracic obstruction. Computed tomography (CT) of the chest revealed that a polypiform nodule of 13 mm in diameter was at the proximal right main bronchus. Testing for purified protein derivative was positive (category 2). The nodule was resected under bronchoscopy. The bronchial aspirate was negative for mycobacterium tuberculosis and tumor cells. The biopsy sample showed a solid and acinar predominant pattern with abundant eosinophilic cytoplasm. The bronchial mucosa was destroyed and replaced by tumor cells. The loose edematous stromal reaction could be seen in a local area. Immunohistochemically, tumor cells were positive for CK, EMA, Vimentin, CD117, CK7, S100, Mammaglobin and SOX10. Only scattered tumor cells were stained by basal cell markers, including CK5/6, P40 and P63. Electron microscopy revealed numerous swelling mitochondria with lacking mitochondrial cristae in tumor cells. Fluorescence in situ hybridization (FISH) testing for MAML2 and ETV6 rearrangement were negative. Next-generation sequencing analysis of 520 genes in the tissue biopsy specimen showed no somatic mutation. The diagnosis of OC was made. Subsequently, the patient underwent a right upper lobectomy with sleeve resection of the main bronchus and lymph dissection. No recurrent evidence was seen during two years of chest CT follow-up.ConclusionsTo our knowledge, this is the first case of primary OC in the bronchus. This patient has no recurrence during two years of follow-up, indicating that primary OC in the bronchus has the same favorable prognosis as in salivary glands. Moreover, complete excision and thorough sampling to know the invasive growth pattern is important to reach the correct diagnosis.

Recent developments in the pathology of primary pulmonary salivary gland-type tumours.

Primary pulmonary salivary gland-type tumours are rare neoplasms that are thought to arise from seromucinous glands that are located in the submucosa of large airways. These neoplasms have clinical and pathologic features that are distinct from other pulmonary neoplasms. The majority of primary pulmonary salivary gland-type tumours are malignant, with the most common entities being mucoepidermoid carcinoma, adenoid cystic carcinoma, and epithelial-myoepithelial carcinoma. Less commonly seen are myoepithelial carcinoma, hyalinizing clear cell carcinoma, acinic cell carcinoma, secretory carcinoma, salivary duct carcinoma, intraductal carcinoma, and polymorphous adenocarcinoma. Benign salivary gland-type tumours of the lung include pleomorphic adenoma and sialadenoma papilliferum. Morphologic, immunophenotypic, and molecular features of these neoplasms are largely similar to salivary gland tumours elsewhere, and therefore the exclusion of metastatic disease requires clinical and radiologic correlation. However, the differential diagnostic considerations are different in the lung. The distinction of salivary gland-type tumours from their histologic mimics is important for both prognostication and treatment decisions. Overall, salivary gland type-tumours tend to have a more favourable outcome than other pulmonary carcinomas, although high-grade variants exist for many of these tumour types. Recent advances in our understanding of the spectrum of salivary gland-type tumours reported in the lung and their diversity of molecular and immunohistochemical features have helped to refine the classification of these tumours and have highlighted a few differences between salivary gland-type tumours of the lung and those primary to other sites.

Primary High-Grade Mucoepidermoid Carcinoma Lung Mimicking Endobronchial Papilloma: A Case Report and Review of Literature

Primary mucoepidermoid carcinomas (MECs) of the lung are rare and present a diagnostic challenge. MEC lung comes under the broad classification of primary salivary gland type tumours of the lung. It usually involves large airways and presents clinically and radiologically with non-specific features. We report the case of a 26-year-old female patient wherein an initial clinicoradiological consideration of tuberculosis and biopsy report of endobronchial papilloma led to misdiagnosis and treatment delay. Flexible bronchoscopic biopsy confirmed the diagnosis of MEC following which patient underwent curative pulmonary sleeve resection with good post-operative outcome. Final histopathology revealed high-grade MEC, stage as pT2N0, and thus, patient is receiving cisplatin-based adjuvant chemotherapy. More studies and reporting of such cases are needed to formulate guidelines and avoid delay in management.

Role of Histone Deacetylases in the Pathogenesis of Salivary Gland Tumors and Therapeutic Targeting Options.

Salivary gland tumors (SGTs) comprise a rare and heterogenous category of benign/malignant neoplasms with progressively increasing knowledge of the molecular mechanisms underpinning their pathogenesis, poor prognosis, and therapeutic treatment efficacy. Emerging data are pointing toward an interplay of genetic and epigenetic factors contributing to their heterogeneity and diverse clinical phenotypes. Post-translational histone modifications such as histone acetylation/deacetylation have been shown to actively participate in the pathobiology of SGTs, further suggesting that histone deacetylating factors (HDACs), selective or pan-HDAC inhibitors (HDACis), might present effective treatment options for these neoplasms. Herein, we describe the molecular and epigenetic mechanisms underlying the pathology of the different types of SGTs, focusing on histone acetylation/deacetylation effects on gene expression as well as the progress of HDACis in SGT therapy and the current status of relevant clinical trials.

The molecular landscape of breast mucoepidermoid carcinoma.

Mucoepidermoid carcinoma (MEC) of the breast is an extremely rare salivary gland-type tumor characterized by epidermoid, basaloid, intermediate, and/or mucinous cells arranged in solid and cystic patterns. Despite their triple-negative phenotype, breast MECs are generally considered low-risk malignancies but their biology is largely unexplored; therefore, guidelines for clinical management are lacking. Here, we sought to characterize the molecular landscape of breast MECs. Thirteen cases were histologically reviewed, characterized for tumor-infiltrating lymphocytes (TILs), and were subjected to immunohistochemistry for programmed death-ligand 1 (PD-L1, clone 22C3), EGFR, and amphiregulin (AREG). Rearrangements in MAML2 and EWSR1 were investigated by fluorescent in situ hybridization. Targeted next-generation sequencing of 161 genes was performed on eight cases. Most MECs had low histological grade (n= 10, 77%), with the presence of TILs (n= 9/12; 75%) and PD-L1 combined positive score ranging from 10 to 20 (n= 4/6; 67%). All cases showed EGFR and AREG overexpression and were fusion negative. Enrichment of genetic alterations was observed in PI3K/AKT/mTOR and cell cycle regulation pathways, while only one case harbored TP53 mutations. This is the first study providing extensive molecular data on breast MECs and the largest collection of cases available to date in the literature. Breast MECs lack TP53 mutations found in high-grade forms of triple-negative breast cancers and MAML2 or EWSR1 rearrangements pathognomonic of salivary MECs. Triple-negativity and PD-L1 positivity suggest a window of opportunity for immunotherapy in these patients. The EGFR/AREG axis activation, coupled with the mutational patterns in PI3K/AKT/mTOR and cell cycle pathways warrants caution in considering MECs as low-risk neoplasms.

Incidence and distribution of different histopathological types of salivary gland tumors in Southeastern Serbia: A three-year unicentre retrospective study

Incidence and distribution of different histopathological types of salivary gland tumors in Southeastern Serbia: A three-year unicentre retrospective study

Acinic Cell Carcinoma of the Breast: A Rare and Diagnostically Challenging Entity

Acinic Cell Carcinoma (AcCC) of the breast is a rare subtype of invasive breast carcinoma that falls under the category of salivary gland-type tumours of the breast. Histologically, it closely resembles AcCC of the salivary gland. Here, the authors report a case of AcCC of the breast in a 51-year-old woman who presented with a palpable lump in her right breast. Radiological investigation revealed a Breast Imaging Reporting and Data System (BIRADS) score V lesion. A core biopsy was performed, and the histopathological examination revealed relatively bland cells arranged in an acinar pattern within a myxoid stroma, closely resembling adenosis rather than a neoplasm. The diagnosis posed a challenge in the core biopsy due to its close resemblance to adenosis. However, the clinical and radiological findings strongly suggested malignancy. Immunostaining with p63 was conducted, which helped demonstrate the absence of myoepithelial cells around the tumour cell nests and supported the neoplastic nature of the lesion. Despite the bland and low-grade morphology, the tumour was found to be triple negative for Oestrogen Receptor (ER), Progesterone Receptor (PR), and Human Epidermal Growth Factor Receptor 2 (HER2), and it exhibited characteristic positivity for SRY-Box Transcription Factor 10 (SOX 10), S100, and CK7, which aided in making the diagnosis. The authors presented the case report to highlight the rarity of this subtype of breast carcinoma and to emphasise the importance of histomorphology and immunoprofile in reaching a diagnosis. To the best of the authors’ knowledge, the present case was the second reported case of AcCC of the breast from India, adding to the novelty of this case.

Primary Pulmonary Hyalinizing Clear Cell Carcinoma: Case Series With Review of Literature.

Background: Hyalinizing clear cell carcinomas of tracheobronchial origin are very rare salivary gland type tumors accounting for less than 1% of lung tumors with only 13 cases reported to date. Their radiological features, morphological spectrum, and molecular features are not well described. Aim: To perform a clinicopathological analysis of primary pulmonary hyalinizing clear cell carcinomas. Method: A retrospective search of primary pulmonary hyalinizing clear cell carcinomas was conducted from authors' institutions and the clinicopathological features including details of molecular testing were analyzed. Results: Five primary pulmonary hyalinizing clear cell carcinomas were identified. The mean patient age at diagnosis was 48.2 years (range: 33-64 years). Three patients were women. All patients were nonsmokers and 3 were symptomatic; 2 were detected incidentally during health screening. The tumors were located in the main lobar bronchi ranging from 1.3 to 4.9 cm in maximum dimension. Microscopy showed cords and nests of at least, focally clear tumor cells. Mucin cysts lacking goblet cells were seen. All tumors were uniformly positive for p40, p63, AE1/AE3, keratin 7, and epithelial membrane antigen but negative for TTF1, KIT, neuroendocrine markers, and other myoepithelial markers. All cases showed Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement. Perineural invasion and lymph node metastases were detected in patient 5. Two patients with available follow-up data were recurrence-free until 4 years (patient 1) and 9 months (patient 5) after resection. Conclusion: The present series adds to the scant available literature on primary pulmonary hyalinizing clear cell carcinomas highlighting the characteristic histomorphology, immunoprofiles, and benign outcomes of these rare tumors.

A case of myoepithelioma with cyst‐like structure in the maxilla

AbstractBackgroundMyoepithelioma is a rare type of salivary gland tumor that mainly occurs in the parotid glands and palate.Case presentationA 43‐year‐old woman presented with swelling and pain in the right maxillary molar. We performed enucleation and curettage under general anesthesia. The tumor was cyst‐like. Histopathological examination revealed nests of tumor cells diffusely interspersed within fibrotic connective tissues. A few cells were positive for cytokeratin AE1/AE3, α‐SMA, GFAP, and vimentin; S‐100 protein and calponin were also positive. Final diagnosis was myoepithelioma.ConclusionOver 7 years after the surgery, the postoperative course was good, with no recurrence of the tumor.

Lacrimal Gland involvement in systemic Sarcoidosis

Lacrimal gland lesions generallypresent as palpable masses in thesuperolateral aspects of the orbits. Approximately 50% of lacrimalgland masses are inflammatory lesions, 25% are lymphoid lesions or lymphoma, and the other 25% are salivary gland type tumors. Although there are overlaps and exceptions, features such as laterality, portion of gland involvement, presence or absence of bony findings, enhancement pattern, andclinical presentation are valuable in differentiating among lacrimal gland lesions. The 3D ultrasound has been used to define lacrimal gland shape, size, density, structural features the pattern of blood supply, as well as the anatomic and topographic position in the orbit. The study was conducted in the B- and 3D-modes of ultrasonography with color and energy Doppler mapping on both sides.

Bronchial salivary gland-type intraductal carcinoma with KIAA1217::RET gene fusion composed of intercalated and oncocytic components.

Salivary gland-type intraductal carcinoma (IC) is a rare malignant salivary gland neoplasm. Primary salivary gland-type IC has never been described in the lung. Herein, we present a primary pulmonary IC in a 63-year-old woman. The tumor originated in the bronchus wall of the right middle lobe. The tumor consisted of two histological types, intercalated component and oncocytic component. The intercalated component showed tubular/cystic pattern composed of column to cube-shaped cells and scattered mucous cells. The oncocytic component showed solid nests composed of large cells with abundant eosinophilic granular cytoplasm. Immunohistochemically, both histological components were positive for cytokeratin 7 (CK7), S-100 protein, SOX10, and mammaglobin. The rimming myoepithelial cells were highlighted by p63 and smooth muscle actin (SMA). The tumor cells were negative for androgen receptor (AR), HER-2, Dog-1, TTF-1, napsin A, GCDFP-15, and GATA3. In the present case, we detected KIAA1217::RET fusion via DNA-based next-generation sequencing (NGS) and RT-PCR, which established the diagnosis of IC at a molecular level. The present case expands the categories of bronchopulmonary salivary gland-type tumors.

Secretory carcinoma of the sinonasal cavity and pharynx: A retrospective analysis of four cases and literature review

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.

A RIDDLE WRAPPED IN A MYSTERY: MUCOEPIDERMOID CARCINOMA (MEC) FOUND DURING EVALUATION OF PERSISTENT LUNG COLLAPSE

A RIDDLE WRAPPED IN A MYSTERY: MUCOEPIDERMOID CARCINOMA (MEC) FOUND DURING EVALUATION OF PERSISTENT LUNG COLLAPSE

Mitotic Index and Immunohistochemical Expression of Ki-67 in Pleomorphic Adenoma of Salivary Glands

.&#x0D; Background: Pleomorphic adenomas (PAs) are the most common type of salivary gland tumors (SGTs) which may undergo malignant transformation to Carcinoma ex PA (CaExPA). The purpose of this study was to determine mitotic index (MI) and expression of Ki-67 in Pleomorphic adenomas of salivary gland tumors.&#x0D; Methodology: This study was carried out on sixty patients of pleomorphic adenoma. Slides with specimens were stained with Hematoxylin &amp; Eosin to count Mitotic index. Labeling Index (LI) of Ki-67 was determined immunohistochemically. Mitotic figures (MF) were counted in 10 HPF selected in the proliferative area. MI was scored as 1 (0 to 4 MF per 10 HPF); 2 (5-9 MF per 10 HPF) and 3 (&gt; 9 MF per 10 HPF). Immunoexpression of Ki-67 was scored as ‘0-negative’ when &lt;5% of neoplastic cells stained, ‘1-weak positive’ when 5-19% of neoplastic cells were stained, ‘2-moderate positive’ when 20-49% of neoplastic cells were stained, and ‘3-strong positive’ when &gt;50% of neoplastic cells were stained with Ki-67. Data was, entered in SPSS 21.&#x0D; Results: Mean age was 38.7 ± 12.86 years. Out of 60 cases, 26 (43.33%) were of males while 34 (56.67%) were of females. Expression of Ki-67 was negative in 50% while remaining weak positive. Only score 01 of MI observed.&#x0D; Conclusion: Ki-67 is more sensitive marker than mitotic index in pleomorphic adenomas even in small sized tumors and it can help in detection of malignant transformation of PAs.&#x0D; Keywords: Adenomas, Immunohistochemistry, Ki-67 Antigen, Parotid Neoplasms, Pleomorphic

Pathological diagnosis of rare and salivary gland-type tumors of breast

第5版WHO乳腺肿瘤分类中新增乳腺少见和涎腺型肿瘤，通常是雌激素受体、孕激素受体和HER2均为阴性、具有惰性生物学行为的“三阴性”肿瘤。因其总体预后良好，故临床治疗需区别于“高度恶性”的三阴性乳腺癌。本文就此类肿瘤的形态学、分子特征、预后及临床治疗进行介绍，以期提高对其临床病理认识水平，并为临床精准治疗提供参考。.