Orbital Tumors - Lacrimal Gland Tumors

General

• 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 very good for differentiating inflammation from tumor: inflammation and lymphoid with in gland cause diffuse enlargement, elongated shape, contour around globe; neoplasms are isolated, globular, displace & indent globe

 

 

• Pathology
• Epithelial Tumors
  • 50% benign mixed
    • Benign Mixed Tumor (Pleomorphic Adenoma)
      • most common epithelial tumor
      • 30-50 year old, M sl>F
      • palpable, painless, slow (history often reveals symptoms > 1 year) growing with globe dispalced 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, 1^o adeno carcinoma, mucoepidermoid carcinoma, squamous carcinoma)
    • Malignant Mixed Tumor
      • often arise from 1^o benign mixed or from recurrent benign mixed if incomplete excision
      • path
        • 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
  • African or Scandinavian descent, systemic disease, non-caseating granuloma with monocytes, typical bilateral lacrimal gland involvement, conj. biopsy or lac gland biopsy or gallium scan could establish diagnosis, ACE, lysozyme, chest x-ray for hilar adenopathy, anergy on skin test
• benign lymphoproliferative lesions of lacrimal gland middle aged F, dry eye, if with rheumatoid arthritis, then classic Sjogren&/260-Lacrimal Gland Tumors/#146;s