A Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a+) Mimicking Glioblastoma Multiforme in a Young Adult.

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A Very Rare Case of Right Insular Lobe Langerhans Cell Histiocytosis (CD1a+) Mimicking Glioblastoma Multiforme in a Young Adult.

World Neurosurg. 2019 Jan;121:4-11

Authors: Bärtschi P, Luna E, González-López P, Abarca J, Herrero J, Costa E, Paya A, Sales J, Moreno P

Abstract
BACKGROUND: Langerhans cell histiocytosis (LCH) is a multisystemic dendritic cell proliferation that is relatively uncommon in adults. Central nervous system LCH outside the pituitary gland is even more uncommon.
CASE DESCRIPTION: We report the case of a 42-year-old man who had complained of right-side hemicranial pain and left arm minor paresis. The symptoms were due to a right insular lobe heterogeneous-enhancing lesion associated with extensive vasogenic edema. The first diagnostic impression suggested glioblastoma multiforme or localized metastasis. The thoracic, abdominal, pelvic computed tomography scan only detected small upper lung inactive nodules suggesting silent focal LCH. A very hard lesion was almost completely removed through a pterional craniotomy approach, with no fluorescence after aminolevulinic acid infusion. The intraoperative biopsy findings ruled out glioma but could not confirm lymphoma. The definitive cerebral biopsy findings showed lymphocytes and histiocytes (CD1a+, S-1001+), with a diagnosis of intracerebral parenchymal LCH. Fractioned radiotherapy resulted in clinical and radiological remission.
CONCLUSIONS: The present case is so rare it should not be used as a guide. We probably will never see a single intraparenchymal supratentorial central nervous system LCH lesion. However, we hope our report will help colleagues in the future with the thought process.

PMID: 30261373 [PubMed - indexed for MEDLINE]

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