Polymorphous low-grade adenocarcinomas are rare tumors that can arise from many areas of the head and neck. We reviewed the pertinent literature pertaining to the incidence, diagnostic evaluation, and treatment strategies for this malignancy. Histopathologic diagnosis always involves careful analysis of tissue and especially characteristic immunohistochemical staining patterns. Common differential diagnoses include adenoid cystic carcinoma, pleomorphic adenoma, and other benign or malignant salivary gland neoplasms. Although the ideal treatment is debated, surgical resection at times combined with adjuvant radiation therapy is preferred by many physicians. Further research will be needed to delineate optimal management.
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A rare case of an aggressive polymorphous adenocarcinoma of minor salivary gland in the retro-maxilla.
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Department of Oral and Maxillofacial Surgery, Manipal College of Dental Sciences, Manipal Academy of Higher Education, Manipal, India.
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Division of Neurology, McMaster University, Hamilton, ON, Canada.
Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a rare central nervous system (CNS) pathology predominantly observed in the pediatric population. Ependymomas also exhibit a peak incidence in early childhood, with rare presentations after early adulthood. In this report, we describe a rare case of a 41-year-old man diagnosed sequentially with a polymorphous low-grade neuroepithelial tumor of the young, followed by a supratentorial ependymoma within a year.
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Article Synopsis
- - Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a new type of brain tumor mainly affecting children, characterized by specific imaging and histological features, often presenting with seizures.
- - In a study of 15 diagnosed cases, imaging revealed well-defined solid-cystic tumors with calcifications, and histopathology showed a mix of tumor cell types that were immunopositive for CD34.
- - Molecular analysis detected mutations consistent with PLNTY, including BRAF and FGFR2/3 gene alterations, indicating it should be considered as a potential diagnosis for similar tumors, particularly in younger patients with seizure histories.
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