Brief report
An Atypical Presentation of Adenoid Cystic Carcinoma of the Lacrimal Gland

https://doi.org/10.1016/j.ajo.2005.08.070Get rights and content

Purpose

To report a case of lacrimal gland adenoid cystic carcinoma (ACC) with an atypical initial presentation and to postulate an anatomical explanation for this unusual biologic behavior.

Design

Interventional case report.

Methods

An orbital magnetic resonance imaging study of a 58-year-old man who complained of progressive diplopia and orbital discomfort disclosed a soft tissue mass in the left cavernous sinus and orbital apex. The left lacrimal gland and the contiguous bone appeared normal.

Results

Biopsy of the intracranial mass revealed ACC of unknown origin which was judged to be unresectable. He underwent two cycles of intraarterial cytoreductive chemotherapy followed by a left medial maxillectomy with sphenoethmoidectomy, orbital exenteration, and chemoradiation. ACC tumor cells were identified in nerve fiber bundles surrounding and within the lacrimal gland.

Conclusions

In patients presenting with an infiltrative mass in the cavernous sinus or orbital apex, metastatic disease from an occult lacrimal gland primary should be considered, even with a normal-appearing lacrimal gland.

References (5)

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Cited by (27)

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    2022, International Journal of Surgery Case Reports
    Citation Excerpt :

    The diagnosis of ACC in these cases with the lack of the exact origin of the tumor in relation to the LG is challenging since these cases might have atypical clinical presentation. Tse and his coauthors reported ACC that presented initially as an orbital apex mass with cavernous sinus involvement and normal appearing ipsilateral LG [13]. Tumor origin in deep orbital ACC is uncertain.

  • Neoadjuvant Intra-Arterial Cytoreductive Chemotherapy for Lacrimal Gland Adenoid Cystic Carcinoma: A Long-Term Follow-up Study of a Trimodal Strategy

    2022, American Journal of Ophthalmology
    Citation Excerpt :

    To illustrate the importance of assessing outcomes in the context of the patient group under treatment, the current series was compared with 2 contemporary globe-sparing surgery reports with sufficient raw data to abstract for K-M curve construction: PCSWo39 and PCSH.40 PCSWo reports a series of patients treated with proton beam therapy after conservative surgery.42 The Kaplan-Meier survival curves derived from the 2 reports appear similar to the IACC per-protocol (group 1).

  • Atypical clinical presentation of an orbital cylindroma

    2018, Journal Francais d'Ophtalmologie
  • Long-term outcomes of neoadjuvant intra-arterial cytoreductive chemotherapy for lacrimal gland adenoid cystic carcinoma

    2013, Ophthalmology
    Citation Excerpt :

    The residual disease served as a nidus to spawn tumor cells to the sinus mucosa probably by retrograde tracking along the lacrimal nerve toward the trunk of the ophthalmic nerve (V1) in the trigeminal ganglion. The tumor cells then crossed to the nasociliary nerve, a branch of V1 in the cavernous sinus, and tracked in an antegrade manner toward the anterior and posterior ethmoidal branches to involve the sinus mucosa.36 Completing all elements of the IACC protocol in a timely manner was important to the success of this treatment.

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Supported in part by NIH center grant P30-EY014801; an unrestricted grant from Research to Prevent Blindness Inc, New York, New York; and a grant from the Plum Foundation, Los Angeles, California.

The authors, their families, their employers, and their business associates have no financial or proprietary interest in any product or company associated with any device, instrument, or drug mentioned in this article. The authors have not received any payment as consultants, reviewers, or evaluators of any of the devices, instruments, or drugs mentioned in this article.

This manuscript is based on a thesis that was prepared in partial fulfillment of the requirements for membership in the American Ophthalmological Society.

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