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