Acinic cell carcinoma of the lacrimal gland
Adult presenting with right sided proptosis.
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A large extraconal low-density mass in the lateral aspect of the right orbit distorts and displaces the globe anteriorly. There is peripheral enhancement and no adjacent bony destruction.
The location of the mass and implied firm consistency (it distorts the globe)suggest the most likely diagnosis is that of a tumor of the lacrimal gland (rather than lymphoma or pseudotumor which tend to be softer and not distort the globe).
The patient went on to have the mass resected.
The right lacrimal gland tumor demonstrates a neoplasm, which contains numerous papillae of neoplastic cells, and cystic spaces, which frequently contain degenerated blood and exfoliated cells. The cells are arranged in a variety of patterns, including solid, papillary and microfollicular. The cells have abundant eosinophilic cytoplasm, which is focally finely granular. Numerous cells containing small to large vacuoles are present.
Cells on the surface of the papillae frequently show a "tombstone" appearance. On PAS and Diastase stains occasional cells show faint granular staining and occasional small glands containing PAS-positive material are present.
The tumor is surrounded by a fibrous capsule of varying thickness. Tumor focally infiltrates into the capsule and just up to beneath the painted resection margin. The tumor stains strongly for keratin, which highlights the invasive features at the margin of the tumor.
Immunoperoxidase stains for smooth muscle actin, S100, P63 and GFAP are negative.
FINAL DIAGNOSIS: The histologic features of this tumor are characteristic of an acinic cell carcinoma which is extremely rare within the lacrimal gland with only a handful of cases having been reported in the literature.
- Jung WS, Ahn KJ, Park MR et-al. The radiological spectrum of orbital pathologies that involve the lacrimal gland and the lacrimal fossa. Korean J Radiol. 2007;8 (4): 336-42. Free text at pubmed - Pubmed citation
- Qin W, Chong R, Huang X et-al. Adenoid cystic carcinoma of the lacrimal gland: CT and MRI findings. Eur J Ophthalmol. 2012;22 (3): 316-9. doi:10.5301/ejo.5000015 - Pubmed citation