Orbital Tumors

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

     

    Orbital Tumors - Lacrimal Gland Tumors

     

    Orbital Tumors - Lacrimal Gland Tumors

     

    Orbital Tumors - Lacrimal Gland Tumors

     

    Orbital Tumors - Lacrimal Gland Tumors

     

  • 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’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
      • 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
  • 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 may 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’s

References

 

   

 

 

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