We thank to Drs. Derakhshan and Roshanfarzad for their interest in our paper “Pandora’s box: eosinophilic granuloma at the cerebellopontine angle—should we open it?” [1]. They have stated their concerns about our paper in their letter to the editor. We gave our answers and made our comments based upon their questions.
Eosinophilic granuloma (EG) is a focal disease form of Langerhans cell histiocytosis (LCH) presenting in the skull, femur, vertebrae, pelvis, mandible, and ribs; yet, multiple sites of occurrence have been described in the literature with confirmation of the pathology [2,3,4,5].
The second query of the authors was about approach to a patient with intracranial EG. In our previous paper, we stated that steroid therapy could be initiated if EG diagnosis was made via clinical, radiological, and laboratory findings with close monitoring of the patient. We agree that surgical option is in the first line in case of life-threatening scenarios. However, we think it is too early to make a guideline for follow-up of patients with intracranial EGs in the absence of a large-scaled clinical study or a meta-analysis of the current literature.
In their case of EG, the authors did not mention if they had made a histopathological confirmation of the diagnosis; yet they insisted on this fact in their letter. They did not give details about clinical presentation and laboratory work-up of their patient. So, it is hard to make a further comment on their case at this point.
We, once again, insist on conducting a multi-center collaboration and set up an international database to collect such cases to perform a large-scaled clinical study and get more steps further ahead in clinical and therapeutic approach to those patients with central nervous system EGs.
References
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Öğrenci, A., Ekşi, M.Ş. A reply to the letter to the editor by Nima Derakhshan and Mahsa Roshanfarzad with regard to the paper “Pandora’s box: eosinophilic granuloma at the cerebellopontine angle—should we open it?”. Childs Nerv Syst 33, 1427 (2017). https://doi.org/10.1007/s00381-017-3533-8
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DOI: https://doi.org/10.1007/s00381-017-3533-8