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Lacrimal Gland Tumors

General

Lacrimal glands secrete tears and are located above and to the side of the eye. When lacrimal gland cells change and grow uncontrollably, they form a cancerous or benign tumor. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread. The diagnostic process involves the following steps:

• look for fullness of upper lid, asymmetry of superior sulcus, abnormal lid contour
• majority lacrimal gland masses are idiopathic inflammatory dacryoadenitis
• especially S-shape, often palpable
• check for mobility, smooth, rubbery or nodular
• proptosis is evidence of posterior growth, otherwise globe is down and media

Imaging

CT scans are very good for differentiating inflammation from tumor. Inflammation and lymphoid within gland cause diffuse enlargement, elongated shape, contour around the globe while neoplasms are isolated and globular.

Pathology

Epithelial Tumors

Epithelial tumors represent 50% of the masses involving the lacrimal gland. The remaining lesions are due to lympho-inflammatory lesions. While pleomorphic adenomas are the most common benign epithelial tumors, adenoid cystic and mucoepidermoid carcinomas are the most common malignant neoplasms. More information is provided below:

• 50% benign mixed
  • Benign Mixed Tumor (Pleomorphic Adenoma)
    • most common epithelial tumor
    • 30-50 year old, M>F
    • palpable, painless, slow (history often reveals symptoms > 1 year) growing with globe displaced down, medial, axial proptosis
    • incites bony cortication, enlargement/expansion lacrimal gland fossa, firm lobular mass
    • CT: lacrimal gland is oblong if inflammatory, globular if malignant
    • Pathology: metaplasia of epithelial cells to form stroma, cartilage, benign epithelial cell nests with loose mesenchymal connective tissue, variability of above is mixed tumor, microscopic extension into pseudocapsule causes recurrence if margins not adequate at excision
    • Treatment: must excise it all with lateral orbitotomy with en bloc excision including pseudocapsule, don&/260-Lacrimal Gland Tumors/#146;t biopsy b/c of 1/3 chance of recurrence, significant risk of malignant degeneration
• 50% carcinomas (50% of these are adenoid cystic, remainder: malignant mixed, 1o adeno carcinoma, mucoepidermoid carcinoma, squamous carcinoma)
  • Malignant Mixed Tumor: often arise from 1o benign mixed or from recurrent benign mixed if incomplete excision
    • Pathology: similar to benign mixed but with malignant change, least common epithelial tumor
    • Treatment: frequent exenteration, bone removal necessary, fatality rate of 50%
  • Adenoid Cystic Carcinoma (Cylindroma) (25% if epithelial lacrimal gland tumors)
    • most common (highly) malignant tumor of lac gland
    • PAIN from bone destruction, perineural invasion, rapid course differentiates from benign mixed
    • Pathology: swiss cheese appearance, stain with mucicarmine, looks benign, infiltration of orbital tissue, incl. perineural invasion, basaloid pattern worst prognosis
    • Treatment: radical orbital exenteration (of roof, lateral wall, floor, orbital soft tissue, anterior temporalis muscle), with XRT, death from intracranial extension or systemic metastisis after multiple recurrences

Non-Epithelial Lacrimal Gland Tumors

Inflammatory

• 1/2 of lacrimal tumors

Orbital inflammatory syndrome

• pseudotumor

Sarcoidosis

Sarcoidosis is a systemic disease of African or Scandinavian descent. Orbital sarcoidosis is common among patients with systemic sarcoidosis and can involve the lacrimal gland, the orbit and its soft tissue along with the optic nerve. Uveitis is the most common manifestation. It is defined by non-caseating granuloma with monocytes and typical bilateral lacrimal gland involvement.