Adenoid cystic carcinoma (ACC) is an uncommon and aggressive head and neck cancer mainly affecting minor salivary glands. It affects more women than men in their 60s and 70s. The tumor is typically locally aggressive and has a high rate of distant metastatic disease. This report unveils a potential avenue for targeted therapy for the management of metastatic disease: a patient with ACC who harbored a specific fibroblast growth factor receptor 2 (FGFR-2) mutation and responded significantly to a novel FGFR-2 inhibitor. This finding could pave the way for personalized treatment options for ACC patients with similar genetic alterations. Nevertheless, the use of futibatinib requires further investigation to optimize treatment protocols, including exploring combination therapies, identifying predictive biomarkers for treatment response, and developing strategies to overcome potential resistance.

BACKGROUND: Acinic cell carcinoma (AciCC) is a rare clinical entity and a salivary gland malignancy. It is associated with wide histological variations in the cytomorphological patterns.
METHODS: Sixty cases diagnosed as AciCC from 2002 to 2023 were assessed for diverse cytomorphological patterns.
RESULTS: The mean age of patients at the time of diagnosis was 44.35±16.8 years ranging from 15 to 81 years. Females comprised 58.3% for a F: M ratio of 1.4:1. Fifty three cases (88.3%) occurred in the parotid gland, two cases in the nasal region (3.3%), and one case each in the soft plate and upper lip (1.7%). The location of the remaining three cases was not specified. The most common presenting complaint was a well-defined facial swelling associated with pain. The average tumor size was 3.8±1.9 cm. The most predominant architectural pattern was solid (83.3%) followed by microcystic (60%), then follicular (41.7%), papillary cystic (14.3%), and tubulocystic (28.6%), and AciCC with de-differentiation/high-grade transformation was reported in three cases (5%). In 83.3% of the cases (50 out of 60), we noticed a mixture of two or more growth patterns. Other degenerative changes included prominent lymphoid stroma, hemorrhage, and cystic change.
CONCLUSIONS: Awareness and recognition of diverse cytomorphological patterns of AciCC, especially in institutions of a developing country where there is limited availability of highly specific and sensitive immunohistochemical stains or molecular diagnostics, are crucial and essential.

Pleomorphic adenoma is a benign tumor that can occur in the salivary glands, most commonly in the parotid gland. While it primarily occurs in the major salivary glands, it can sometimes be found in the minor salivary glands. Within the minor salivary glands, it most often originates in the hard palate and soft palate, and less frequently in the upper lips. Due to its location in the minor salivary glands, most pleomorphic adenoma involve and protrude on the mucosa. A 61-year-old man presented with 1.5 cm exophytic mass on the skin of his upper lip. This mass was exophytic on the skin and did not involve or protrude into the inner lip mucosa. The mass was entirely excised, and a subsequent permanent biopsy diagnosed it as a pleomorphic adenoma. In such situations, it can be challenging to suspect pleomorphic adenoma during a physical examination, leading to potential diagnostic confusion. It might also be mistaken for an inclusion cyst or another type of mass, making it tempting to treat without verifying the pathological results.

Mammary analogue secretory carcinoma (MASC) is a rare salivary gland tumor which shares its histologic, immunohistochemical, and genetic features with the secretory carcinoma (SC) of breast. In this case report, we describe a case of MASC in a young adolescent male with swelling in the right angle of mandible which is a relatively rare site to present along with its correlation of cytological, histological, and immunohistochemical features. A 16-year-old male came with the complaint of swelling in the right angle of mandible since 2 years. Contrast-enhanced computed tomography (CECT) neck revealed differential diagnosis of nerve sheath tumor, pleomorphic adenoma, and adenoid cystic neoplasm was kept, and subsequently fine-needle aspiration cytology (FNAC) was done. FNAC was done in which differential diagnosis of myoepithelial neoplasm, acinic cell carcinoma, and SC was given. Surgical excision was done followed by histopathological examination. Immunohistochemistry panel was also applied, and final diagnosis of SC was rendered. SC has distinct cytological, histological, and immunohistochemical features which should be recognized by the pathologists for the appropriate management of the patient.

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Diagnosing a soft tissue tumor in the head and neck region can be challenging due to its complex anatomy and diverse histological spectrum. This case report highlights the case of a woman who presented with a painless neck lump in the posterior triangle of the neck. Various pathological and imaging studies were suggestive of pleomorphic adenoma, which arises from the left prevertebral space. The patient underwent complete surgical excision via the transcervical approach. Pleomorphic adenoma in the posterior triangle of the neck is extremely rare and causes a diagnostic dilemma in managing soft tissue tumors of the neck.

The differential diagnosis of salivary gland lesions with epithelial components and lymphoid stroma is often challenging. Salivary gland carcinoma with tumor-associated lymphoid proliferation, tumors composed of both epithelial and lymphoid components, lymphoid neoplasms in the salivary gland, and inflammatory lesions are all included in this category. It encompasses inflammatory lesions and neoplastic lesions. With the exception of Warthin tumors, these lesions are rare, making them more difficult to diagnose. Carcinoma showing thymus-like elements has recently been reported in the salivary gland. Similar to thymic carcinoma, tumor cells are positive for CD5 and are accompanied by T lymphocytes.

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.

Salivary gland tumors (SGT) display morphological diversity and pose diagnostic challenges. Preoperative fine needle aspiration cytology (FNAC) is a minimally invasive and efficient diagnostic test. However, due to the limited sample size, the final diagnosis may not be established based on FNAC alone. Although cytomorphology and architecture are usually preserved on FNAC, morphologic changes specific to FNAC can complicate the diagnosis. The Milan System for Reporting Salivary Gland Cytopathology categorizes complex FNAC interpretations. Because the cytological diagnosis is closely linked to the histological diagnosis, a multidimensional approach considering the possibility of several differential diagnoses is necessary. From the standpoint of treatment, distinguishing high-grade malignancy from low-grade malignancy is more important than distinguishing malignancy from benign tumors.

Of all the head and neck tumors, salivary gland tumors account to 3%. Pleomorphic adenomas are one of the most common benign tumors arising from major salivary glands, although it could also develop from minor salivary glands situated at accessory sites like nasal cavity, pharynx, parapharyngeal space, lacrimal glands etc. Tumors of infratemporal fossa are quite unusual, mainly because of its hidden location in retromaxillary region. We report an unusual case of 65 years old male presenting with complaint of progressive left cheek swelling for 4 years. FNAC revealed pleomorphic adenoma of minor salivary gland tumor. Intraoperatively a giant lobulated tumor was seen occupying almost whole space of infratemporal fossa, which was removed in-toto via open approach. Patient was kept on regular follow up with no evidence of recurrence reported till date.