A Case of Erdheim-Chester Disease that was Difficult to Differentiate from Meningioma Tomomi YOSHIYAMA 1 , Wataru MUNAKATA 2 , Akiko MAESHIMA 3 , Arisa UMESAKI 1 , Hiroo YAMAGA 1 , Akira NISHIYAMA 1 , Takato NAKAJO 1 , Yuko TANAKA 1 , Hiroaki MATSUMOTO 1 , Tomoaki TERADA 1 1Department of Neurosurgery, Showa University Fujigaoka Hospital 2Department of Hematology, Rare Cancer Center, National Cancer Center Hospital 3Department of Pathology, National Cancer Center Hospital Keyword: Erdheim-Chester disease , non-Langerhans histiocytosis , meningioma , central nervous system , surgery pp.809-818
Published Date 2020/9/10
DOI https://doi.org/10.11477/mf.1436204277
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 OBJECTIVE:Erdheim-Chester disease(ECD)is a rare type of non-Langerhans histiocytosis. We report a surgical case of ECD with multiple lesions at the falx cerebri, tentorium cerebelli, and in the suprasellar region, with a literature review.

 CASE REPORT:A 70-year-old woman presented with frequent falling and difficulty in standing. Her past medical history revealed ovarian cystectomy, transient thrombocytopenia, hypertension, left lower leg pain, and overactive bladder. Her head CT and MRI findings revealed well-defined mass lesions, suspected of meningioma, at the falx cerebri and tentorium cerebelli. Craniotomy and near total resection of the tumor at the falx cerebri was performed, leaving a hard portion of the tumor on the right falx. Intraoperative findings showed a solid and hard tumor, which was extremely difficult to decompress. Although the histopathological diagnosis was originally a metaplastic meningioma, considering her complaints of lower leg pain, we suspected ECD and performed a right tibial biopsy. The right tibial biopsy revealed ECD. Twenty-two months after the operation, the patient exhibited a marked enlargement of the tentorium lesion and a new lesion in the suprasellar region. Resection of the tentorial lesion was performed. The second intraoperative findings were similar to those of the first. The histopathological diagnosis of the tentorial lesion was ECD. After the surgeries, steroid therapy and radiation therapy were performed, but only with temporary improvement.

 CONCLUSION:ECD is a rare disease;therefore, accumulation of clinical data to establish its treatment is necessary.

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