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A 60-year-old woman with an asymptomatic left lacrimal gland mass found incidentally
Digital Journal of Ophthalmology 2017
Volume 23, Number 4
October 18, 2017
DOI: 10.5693/djo.03.2017.07.001
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Sudip D. Thakar, BS | Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston
Oded Sagiv, MD | aOrbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston
Michael Tetzlaff, MD, PhD | Department of Pathology and Department of Translational and Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston
Adel El-Naggar, MD, PhD | Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston
Matthew Debnam, MD | Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston
Thomas J. Kandl, MD | Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston
Bita Esmaeli, MD, FACS | Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston
Diagnosis and Discussion
We describe a rare case of an asymptomatic lacrimal gland schwannoma detected incidentally on routine imaging. The annual incidence of lacrimal gland tumors is approximately 1 in 1 million, with pleomorphic adenoma (benign mixed tumor) being the most common epithelial tumor of the lacrimal gland.(1,2) Patients with lacrimal gland tumors can present with findings of diplopia, restricted ocular motility, and globe dystopia with an inferomedial shift.(3) A Schwannoma of the lacrimal gland is an extremely rare tumor; only 8 previous cases have been described in the literature.(4-9)

Schwannomas are benign, peripheral nerve sheath tumors, composed of neural crest–derived Schwann cells; they comprise 0.7%-2.3% of orbital tumors.(10,11) It commonly manifests along the supraorbital branch of the frontal nerve.(12) We postulate that our patient had an extension of her schwannoma from the lacrimal branch of the ophthalmic division of the trigeminal nerve.

Schwannomas classically consist of spindle cells arranged as hypercellular and alternating hypocellular regions known as Antoni A and Antoni B, respectively.(13) This biphasic phenomenon underlies the classic signal heterogeneity on T2-weighted imaging, as demonstrated in Figure 1B.(14,15) There can also be palisading arrangements of nuclei known as Verocay bodies.

The clinical and radiologic diagnosis of the lacrimal gland tumor in our patient was further hindered by her complex history of an aggressive primary adenocarcinoma of the maxillary sinus and local radiation. Our presumptive diagnosis was a benign epithelial tumor or metastatic disease. The gross specimen after surgical resection had a yellow appearance with a potatolike consistency characteristic of a pleomorphic adenoma. However, it was not until histopathologic assessment that a diagnosis of Schwannoma of the lacrimal gland was made. In addition to nonspecific S100 protein and glial fibrillary acid protein expression in schwannomas, markers such as calretinin, podoplanin, and SOX10 may also be useful in elucidating the diagnosis.(16-18) In a recent review of the literature, Yamanaka and Hayano reported 28 cases of radiation-induced schwannomas.(19) Because the primary tumor was on the left maxillary sinus and nasal cavity, the radiation field may have included the left orbit. Since the schwannoma was not present on imaging prior to radiation, it is possible that adjacent surrounding structures may have been affected thereby promoting tumorogenesis of this rare, neoplastic variety.

Our case demonstrates that, although rare, schwannoma of the lacrimal gland should be considered on the differential diagnosis of a well-circumscribed lacrimal gland mass. Gross total surgical resection is the appropriate treatment particularly if there are signs of growth or concerns for other more malignant diseases, as was the case in our patient with a history of significant malignant carcinoma of the maxillary sinus.
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