UNASSIGNED: Polymorphous low-grade adenocarcinoma (PLGA) is a rare neoplasm arising from minor salivary glands, representing approximately 3 % of head and neck tumors. The clinical presentation of PLGA is defined as a painless, slow-growing tumor, mostly occurring in the palate. We report a case of PLGA with a rare presentation.
METHODS: A 76-year-old male, known case of hepatitis B, diabetes, and hypertension, presented to the emergency department complaining of spitting blood and dysphagia. Imaging showed a heterogeneous enlarged left tonsil with hyperemia of the mucosa, and air foci. Biopsy with excisional biopsy confirmed the diagnosis of PLGA. The patient underwent completion tonsillectomy and selective neck dissection which yielded tonsillar tissue with underlying PLGA, and reactive lymph nodes with no malignant tissue respectively, margins were negative for malignancy.
UNASSIGNED: Polymorphous low-grade adenocarcinoma is a rare lesion with clinical behavior resembling that of a benign neoplasm. Predominantly occurring in the oral cavity, especially on the hard palate, buccal mucosa, and retromolar region, with fewer cases in the upper lip. Occurrence in the nasopharynx and oropharynx is rare. PLGA presents as painless slow-growing masses, typically in females aged 50-60. Local excision with careful margin evaluation is the preferred treatment, with good prognosis compared to other carcinomas.
CONCLUSIONS: PLGA is rare, with limited reported case from around the world. It is mostly seen in adults between their fifth and sixth decades with female predominance. PLGA is diagnosed using imaging, immunohistochemistry. Owing to the limited cases there is no standard approach to treating PLGA. However, most cases are managed with local excision and showed an excellent response in terms of tumor nonrecurrence.

Basal cell adenoma (BCA) is a rare, benign tumor originating from the epithelial cells of the salivary glands. It was earlier categorized as a subtype of monomorphic adenoma with distinctive histopathological features. BCA usually manifests as asymptomatic, slow-growing masses that exhibit a site and age predilection, commonly affecting the major salivary glands of elderly female patients. Histologically, solid, trabecular, tubular, and membranous patterns are recognized. It is imperative to establish a precise distinction between BCA, pleomorphic adenoma, and malignant salivary gland tumors before initiating treatment to ensure effective management. The standard treatment approach is surgical resection of the tumor. Recurrence and malignant transformation rarely occur, except for the membranous subtype. This article aims to report an unusual case of BCA arising from a minor salivary gland in the upper lip. The post-operative course was unremarkable, with complete healing of the surgical site. No recurrence was observed during a one-year follow-up. BCA arising from a minor salivary gland in the upper lip is an extremely uncommon entity. A comprehensive review of BCA in the upper lip, reported from 1991 to December 2023, revealed only 14 cases.

Dysgenetic polycystic disease, also known just as polycystic disease, is a very rare developmental abnormality affecting the salivary gland duct system. This entity has been reported in only 21 patients previously, although a careful review suggests only 16 patients have histological evidence of the disease. In previously reported cases, this lesion most commonly presents as either an incidental finding or as a swelling affecting the parotid glands bilaterally, or rarely the submandibular glands bilaterally. This case report details the first time dysgenetic polycystic disease is found affecting the minor salivary glands of the tongue in a 55-year-old male. Histochemical and immunohistochemical stains are presented and include positivity for AE1/AE3 and p63, and negativity for progesterone receptor, androgen receptor, mammaglobin, S100 and BRAF V600E. PAS-D and Congo Red highlight special microamyloid spheroliths structures intraluminally.

OBJECTIVE: Secretory Carcinoma is a malignant salivary gland tumor, initially described in 2010. This rare tumor is associated with the translocation t(12;15) (p13;q25), resulting in the fusion gene ETV6-NTRK3. Since this tumor is quite rare, most publications describe only small cohorts of patients. We aimed to investigate the clinical, pathological, and prognostic features of this newly defined malignant entity.
METHODS: Pubmed, Google Scholar, and Web of Science databases.
METHODS: All published articles between 2010 and 2023 were reviewed. Search terms included the terms \"Mammary Analogue Secretory Carcinoma\" and \"Secretory Carcinoma\". All articles published in English reporting on Secretory Carcinoma of salivary glands were retrieved.
RESULTS: One-hundred and 12 retrospective articles reporting a total of 674 patients were included, with 52% males and a mean age of 44.9 ± 18.9. The event rate for patients with advanced-stage disease (Stage 3/4) at presentation was 24.1% (95% CI 17.6%-31.9%, I2  = 9.2%), 14.6% for regional metastases (95% CI 10.5%-20%, I2  = 12%), and the event rate of distant metastasis was 8.4% (95% CI 5.5%-12.7%, I2  = 4.2%). Adjuvant radiotherapy was administered for 30.3% of patients (95% CI 24.1%-37.2%, I2  = 21.5%). The recurrence rate was 19% (95% CI 15.1%-23.8%, I2  = 5%). Survival outcomes showed a 17.2% death of disease rate for Secretory Carcinoma patients (95% CI 13.5%-21.8%, I2  = 7.3%).
CONCLUSIONS: Secretory Carcinoma is a rare and relatively newly defined entity arising in the parotid gland most commonly. Characterized as a low-grade tumor, the majority of patients are diagnosed at an early stage, without regional or distant disease, and the prognosis is relatively good.
METHODS: NA Laryngoscope, 134:1716-1724, 2024.

In the treatment of various patients, the presence of lymphovascular invasion is a prognostic determinant, often taken into account by surgeons and oncologists. The exact frequency and prognostic impacts of this microscopic event in adenoid cystic carcinoma (ACC) patients are, however, not clear. This systematic review and meta-analysis aimed to investigate the lymph node involvement and the clinical stage of cancer as predictors of ACC prognosis. A systematic search was conducted covering a number of databases, including PubMed, Science Direct, Google Scholar, Web of Science, and EBSCO. A total of three studies were included in this analysis, with 591 participants, 247 of whom were males. Lymph node involvement and clinical stage were demonstrated as significant bad prognosis factors among ACC patients (HR = 1.48, 95% CI, 1.00, 1.96; P<0.0001). We found that lymph node involvement and clinical stage of the cancer are both significant predictors of bad prognosis of ACC.

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.

Polymorphous adenocarcinoma (PAC) is a rare variant of minor salivary gland tumors. Because of its architectural diversity, histological diagnosis of PAC can be difficult especially for small biopsies, and immunohistochemistry is of great help in differentiating it from its histologic mimics. The aim of this study is to conduct a systematic literature review to identify reliable immunohistochemical markers for PAC. We conducted an electronic literature search of the MEDLINE, ScienceDirect, SpringerLink, and Wiley Online Library databases, covering the literature published in the period between 1988 and 2021. The eligibility criteria included case reports and retrospective studies of PAC cases with details of immunohistochemical markers. Following the search and selection process, 32 studies with 409 cases were included in this systematic review. Overall, > 90% positivity was observed for pan-cytokeratin (CK) (97.3%), CK7 (96.8%), CK7/8 (97.4%), E-cadherin (90.0%), Vimentin (92.5%), S100 (97.0%), p63 (91.7%), and SOX10 (100%), while little to no positivity was observed for CK20 (0.0%), p40 (0.0%), and GFAP (5.0%). The average MIB-1 labeling index was 3.78%. The results of this systematic review indicate that CK7+/CK20-, p63+/p40-, S100+, Vimentin+, and GFAP- immunophenotype have diagnostic value for PAC. In addition, the use of S100, MSA, p40, and c-Kit provide additional layers of information helpful to differentiate PAC from adenoid cystic carcinoma, one of challenging differential diagnoses.

UNASSIGNED: Acinic cell carcinoma (ACC) is a rare low-grade salivary gland malignancy that accounts for approximately 17% of all salivary gland malignancies. The most common site affected by ACC is the parotid gland followed by the submandibular glands, minor salivary glands, and sublingual glands. Also, it could hardly be observed in unusual sites such as the jaw bones.
METHODS: This case is an example of a central acinic cell carcinoma in a 73-year-old man who came up with a painless gradual swelling for 15 months. Based on clinico-radio-pathologic findings, the diagnosis of a solid variant Intraosseous Acinic Cell Carcinoma was established. Subsequently, the patient underwent hemimandibulectomy and modified radical neck dissection, followed by postoperative radiotherapy. Within a six-month follow-up period, no evidence of residual tumor was found.
UNASSIGNED: Central salivary gland carcinoma is a rare entity and intraosseous ACC is more scarcely observed. Based on our findings, a total of 17 cases of primary intraosseous ACC have been reported so far. Etiology and clinical presentations of this tumor are still vague due to its rarity.
CONCLUSIONS: Dentists and oral surgeons must be aware of such a rare malignant lesion when encountering a radiolucent lesion within the jaws. The early diagnosis and a complete surgical excision to achieve tumor-free surgical margins and a long-term follow-up could result in significantly improved survival rates.

Epithelial-myoepithelial carcinoma (EMC) is a low grade tumor that comprises 1% of all salivary tumors. Local recurrence is not uncommon, but rarely does this tumor demonstrate distant metastasis. We describe a case of a 53-year old female presenting with an asymptomatic, slowly enlarging left submandibular neck mass. Excision of the left submandibular gland (SMG) revealed epithelial-myoepithelial carcinoma with extensive perineural invasion and microscopically positive margins. A subsequent left supraomohyoid neck dissection demonstrated no residual tumor. The patient was stable for one year until a magnetic resonance imaging (MRI) workup for low back pain revealed multiple sclerotic lesions in the iliac crest and lumbar spine, with an iliac crest biopsy demonstrating metastasis. 2.5 year post-operative positron emission tomography-computed tomography (PET-CT) revealed increased [18F]-fluorodeoxyglucose (FDG) avidity in the right iliac crest, pubic symphysis, thoracic and lumbar spine, 9th rib, and sternum concerning for local recurrence and further osseous metastasis. We report the first known case of a submandibular gland EMC with osseous metastasis and highlight the need for prolonged tumor surveillance.

Clear cell carcinoma (CCC) is a rare low-grade malignant salivary gland carcinoma. EWSR1-ATF1 fusion has been characterized as a consistent finding in CCC, with breakpoints described between EWSR1 exon 11 and ATF1 exon 3. So far, over 100 cases of CCC harboring EWSR1 rearrangement arising from salivary gland of the oral cavity have been reported. Although EWSR1 involvement in these cases was confirmed by EWSR1 break-apart FISH indicating the translocation, sequence analysis for EWSR1-ATF1 fusion type has been reported only in three cases of CCC so far. Herein, we report a CCC case with novel EWSR1-ATF1 fusion (EWSR1 exon 15 and ATF1 exon 5) arising in minor salivary gland and review the role of the chimeric variants in some malignancies with EWSR1-ATF1 rearrangement. Current tumor was composed of the small nests of clear tumor cells and hyalized fibrous stroma. Immunohistochemically, the tumor was positive for AE1/AE3, CK5/6 and p63, negative for S100, Melan-A, SMA and CD10. After 8 months of follow-up, there are no evidence of recurrence.