An Atypical Presentation of Adenoid Cystic Carcinoma of the Lacrimal Gland

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. Interventional case report. An orbital magnetic resonance imaging study of a 58-year-old man who complained of progre...

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Bibliographic Details
Published in:American journal of ophthalmology 2006, Vol.141 (1), p.187-189
Main Authors: Tse, David T., Benedetto, Pasquale, Morcos, Jacques J., Johnson, Thomas E., Weed, Donald, Dubovy, Sander
Format: Article
Language:English
Subjects:
Biological and medical sciences
Carcinoma, Adenoid Cystic - pathology
Carcinoma, Adenoid Cystic - therapy
Combined Modality Therapy
Eye Neoplasms - pathology
Eye Neoplasms - therapy
Humans
Lacrimal Apparatus Diseases - pathology
Lacrimal Apparatus Diseases - therapy
Magnetic Resonance Imaging
Male
Medical research
Medical sciences
Middle Aged
Miscellaneous
Neoplasm Invasiveness
Ophthalmology
Orbital Neoplasms - pathology
Orbital Neoplasms - therapy
Paranasal Sinus Neoplasms - pathology
Paranasal Sinus Neoplasms - therapy
Sinuses
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Summary: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. Interventional case report. 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. 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. 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.
ISSN:0002-9394
1879-1891
DOI:10.1016/j.ajo.2005.08.070