Endolymphatic sac tumour (ELST) is a primary low-grade, locally invasive adenocarcinoma of the endolymphatic sac, characterized by the proliferation of cuboidal cells forming a papillotubular pattern and colloid-filled cysts. Rare in the general population, it coincides significantly with the presence of von Hippel-Lindau disease. The natural history, mechanisms underlying the early symptoms, anatomical origin of ELST and optimal timing of their treatment are unknown. In this study, we report a Polish male patient with sporadic ELST (without a family history of VHL disease) along with a review of literature. The light microscopic and immunohistochemical features as well as clinical presentation were typical of ELST.
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Endolymphatic sac tumors (ELSTs) are rare, slow-growing, and locally aggressive neoplasms that originate from the epithelial lining of the endolymphatic duct and sac. These are characterized by their infiltrative growth pattern and the potential for local destruction of surrounding structures, including the inner ear and temporal bone. We report a case of an incidentally diagnosed sporadic ELST.
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View Article and Find Full Text PDFIs the TriNetX Database a Good Tool for Investigation of Real-World Management of Von Hippel-Lindau?
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Article Synopsis
- The study explores the suitability of the TriNetX database for researching the real-world management of Von Hippel-Lindau (vHL), a rare hereditary disease characterized by various tumors.
- The researchers analyzed data from 1,232 patients diagnosed with vHL within the TriNetX database to identify the prevalence of several vHL-related conditions, finding that certain conditions like renal cell carcinoma and pheochromocytomas were more common than others.
- Despite the robust patient data available, the findings suggest that vHL and its associated conditions may be underdiagnosed within the TriNetX database, casting doubt on its effectiveness for comprehensive vHL studies compared to existing literature.
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