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August 11, 2015; 85 (6) NeuroImages

Cerebellar meningeal melanocytoma associated with nevus of Ota

An extremely rare case

Hongxu Chen, Wenke Liu, Si Zhang, Jianguo Xu, Xuhui Hui
First published August 10, 2015, DOI: https://doi.org/10.1212/WNL.0000000000001837
Hongxu Chen
From West China Hospital, Sichuan University, China.
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Wenke Liu
From West China Hospital, Sichuan University, China.
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Si Zhang
From West China Hospital, Sichuan University, China.
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Jianguo Xu
From West China Hospital, Sichuan University, China.
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Xuhui Hui
From West China Hospital, Sichuan University, China.
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Citation
Cerebellar meningeal melanocytoma associated with nevus of Ota
An extremely rare case
Hongxu Chen, Wenke Liu, Si Zhang, Jianguo Xu, Xuhui Hui
Neurology Aug 2015, 85 (6) 555-556; DOI: 10.1212/WNL.0000000000001837

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A 20-year-old woman presented with a 2-day history of headache. She had a congenital grayish-blue pigmented nevus on her left side face, which involved ipsilateral sclera and oral mucosal membrane, diagnosed as nevus of Ota (figure 1, A and B). Neuroradiologic manifestation revealed a lesion in the left cerebellar hemisphere, the etiology of the presenting tumor apoplexy (figure 1, C–F). A left cerebellar hemisphere craniotomy was performed. A well-defined black lesion involved dura mater to the pia mater and was firmly attached to the cerebellar tentorium (figure 2, A and B). Histopathologic examination confirmed a meningeal melanocytoma. Postoperative CT demonstrated total tumor resection.1

Figure 1
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Figure 1 Photograph of the patient and preoperative brain MRI and CT

Physical examination found a congenital irregular grayish-blue macular pigmented nevus located in the territory of the left trigeminal nerve (A); bluish pigmentation of ipsilateral sclera and oral mucosal membrane were also observed (A, B, arrow). Axial CT of the head scan showed a heterogeneous hyperdense mass surrounded by a little slightly hyperdense shadow and flake edema (C). Axial MRI disclosed a slightly T1-hyperintense and T2-hypointense lesion with a well-demarcated border adjacent to the brainstem (D, E), and the posterior part of the tumor enhanced obviously after gadolinium administration (F).

Figure 2
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Figure 2 Intraoperative findings

The well-defined, soft, friable and black lesion involved dura mater to the arachnoid membrane and the pia mater (A, B), and it was firmly attached to the tentorium of cerebellum (B, arrow).

Footnotes

  • ↵* These authors contributed equally to this work and should be considered co-first authors.

  • Author contributions: All authors were involved in clinical care and investigative workup of the patient. Xuhui Hui provided pictures of the patient and drafted and revised the manuscript. Hongxu Chen and Wenke Liu performed the patient follow-up and drafted and revised the manuscript. Si Zhang and Jianguo Xu were responsible for the study concept and revised the manuscript for intellectual content.

  • Study funding: No targeted funding reported.

  • Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

  • © 2015 American Academy of Neurology

References

  1. 1.↵
    1. Munoz-Hidalgo L,
    2. Lopez-Gines C,
    3. Navarro L,
    4. et al
    . BRAF V600E mutation in two distinct meningeal melanocytomas associated with a nevus of Ota. J Clin Oncol 2014;32:e72–e75.
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