Endolymphatic sac tumours (ELST) are aggressive papillary tumours of the temporal bone. The name was finally determined after the endolymphatic sac was determined as the site of their origin. They should be considered in patients with tumours eroding the petrous part of the temporal bone, extending to the cerebellopontine angle or other adjacent structures. These very rare tumours in the general population have much higher prevalence in von Hippel-Lindau disease. Hence molecular analysis of the VHL gene should be performed in patients with ELST and their relatives. The purpose of this study is to present a case report, histopathological characterization of endolymphatic sac tumours, their association with von Hippel-Lindau disease and use of molecular analysis.
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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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