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Improved Turnaround Times | Median time to first decision: 12 days

Research ArticleRADIOLOGY-PATHOLOGY CORRELATION

Optic Nerve Choristoma Mimicking a Neurenteric Cyst

J.C. Benson, C. Giannini, S. Cohen Cohen, J. Van Gompel, D.K. Kim, J. Port, F. Diehn, L. Eckel and C. Carr
American Journal of Neuroradiology December 2020, DOI: https://doi.org/10.3174/ajnr.A6892
J.C. Benson
aFrom the Department of Radiology (J.C.B., D.K.K., J.P., F.D., L.E., C.C.)
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  • ORCID record for J.C. Benson
C. Giannini
bPathology (C.G.), Mayo Clinic, Rochester, Minnesota
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S. Cohen Cohen
cNeurosurgery (S.C.C., J.V.G.), Mayo Clinic, Rochester, Minnesota
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J. Van Gompel
cNeurosurgery (S.C.C., J.V.G.), Mayo Clinic, Rochester, Minnesota
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D.K. Kim
aFrom the Department of Radiology (J.C.B., D.K.K., J.P., F.D., L.E., C.C.)
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J. Port
aFrom the Department of Radiology (J.C.B., D.K.K., J.P., F.D., L.E., C.C.)
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F. Diehn
aFrom the Department of Radiology (J.C.B., D.K.K., J.P., F.D., L.E., C.C.)
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L. Eckel
aFrom the Department of Radiology (J.C.B., D.K.K., J.P., F.D., L.E., C.C.)
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C. Carr
aFrom the Department of Radiology (J.C.B., D.K.K., J.P., F.D., L.E., C.C.)
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Figures

  • FIG 1.
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    FIG 1.

    Preoperative CT and MR images of the lesion. Axial CT (A), axial (B) and sagittal (C) MPRAGE, and axial T2 Cube (GE Healthcare) (D), T2-FLAIR (E), and DWI (F) demonstrate an extra-axial cystic lesion in the anterior cranial fossa (between arrows, A). The cyst drapes around the anterior falx and causes substantial mass effect on the adjacent bifrontal gyri. The optic chiasm (curved arrow, C) and pituitary gland (dashed arrow, C) are deviated posteriorly. The intracystic fluid signal is slightly hyperintense to CSF on T2 Cube, does not suppress on FLAIR, and does not restrict on DWI.

  • FIG 2.
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    FIG 2.

    Intraoperative view of a left frontal craniotomy with a subfrontal approach for resection of an anterior cranial fossa cyst. A granular lesion adhering to the medial surface of the left optic nerve is shown, with adjacent structures labeled. L. indicates left.

  • FIG 3.
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    FIG 3.

    Re-review of preoperative coronal MPRAGE (A) and postcontrast (B) images demonstrates a T1-hyperintense focus along the left optic nerve (arrows), correlating with the surgical findings. Intrinsic T1 signal was presumably related to the intralesional fat tissue noted on subsequent histologic analysis. Although faint enhancement was seen in the adjacent tissue, it was not certain whether a component of this solid focus demonstrated definite enhancement (B).

  • FIG 4.
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    FIG 4.

    Optic nerve choristoma composed of adipose tissue, glandular lobules, and ducts (A), with predominant mucinous (B) and scant serous (C) glands consistent with salivary gland tissue. The duct (D) shows a typical basal cell layer (black arrow) and columnar lining.

  • FIG 5.
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    FIG 5.

    The collapsed cyst wall shows largely a flattened lining (A and B) bistratified positive for CAM5.2 (C). p63-positivity is limited to the basal cells of the bistratified epithelium lining the cyst wall as typically seen in duct structures (D). BRAF V600E, a marker of papillary craniopharyngioma, is negative (E); the stain is also typically positive in normal cilia, which were not present in the cyst lining. The cyst lining shows normal cytoplasmic beta-catenin expression (F) and also of epithelial membrane antigen (EMA) (G), while progesterone receptor (PR) stain is present in a subset of the arachnoidal cells observed in the fibrous stroma associated with the cyst wall (J).

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J.C. Benson, C. Giannini, S. Cohen Cohen, J. Van Gompel, D.K. Kim, J. Port, F. Diehn, L. Eckel, C. Carr
Optic Nerve Choristoma Mimicking a Neurenteric Cyst
American Journal of Neuroradiology Dec 2020, DOI: 10.3174/ajnr.A6892

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Optic Nerve Choristoma Mimicking a Neurenteric Cyst
J.C. Benson, C. Giannini, S. Cohen Cohen, J. Van Gompel, D.K. Kim, J. Port, F. Diehn, L. Eckel, C. Carr
American Journal of Neuroradiology Dec 2020, DOI: 10.3174/ajnr.A6892
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