A Case Report of Surgical Resection of a Supraseller Granuloma in a Patient with Erdheim-Chester Disease Shinji NODA 1 , Hideomi KITAJIMA 1 , Masanori TSUJIMOTO 1 , Nozomi SASAKI 1 , Yohei ITOH 1 , Jun SHINODA 2 1Department of Neurosurgery, Toki General Hospital 2Department of Neurosurgery, Chubu Medical Center for Prolonged Traumatic Brain Dysfunction, Kizawa Memorial Hospital Keyword: Erdheim-Chester disease , craniotomy , tumor resection , granuloma pp.231-236
Published Date 2020/3/10
DOI https://doi.org/10.11477/mf.1436204168
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 Erdheim-Chester disease(E-CD)is a rare pathology characterized by systematic granulomatosis that occasionally involves the central nervous system. We report about a 68-year-old woman with E-CD who presented with right-side visual disturbance. Magnetic resonance imaging showed a suprasellar tumor that elevated the right optic nerve and involved the right internal carotid and right anterior choroidal arteries. The tumor was partially resected via a trans-Sylvian approach and was histologically diagnosed as a granuloma. Considering the abnormal findings of postoperative X-ray and 99 mTc bone scintigraphy of the long bones, the pathology was diagnosed as E-CD. After surgery, her right-side visual disturbances disappeared. However, 1 year later, she died of systemic infection and heart failure. Histological autopsy findings indicated numerous yellowish nodules in the heart, lung, and kidney with pericardial and pleural effusions and whole-body granulomatosis, including the brain.

 E-CD is a rare but critical disease. This pathological entity should be considered when encountering cases of intracranial granuloma to ensure its early diagnosis and appropriate treatment. Surgical resection of intracranial granulomas in patients with E-CD may promptly improve neurological dysfunction.

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