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Orbit tumours (including lacrimal gland)

Orbit tumours (including lacrimal gland). CASE 1. Mass in supero-temporal orbit Painless proptosis. Lacrimal gland. Well defined, lesion. Neoplastic glands embedded in a fibro (pink) -myxoid (blue) matrix.

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Orbit tumours (including lacrimal gland)

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  1. Orbit tumours(including lacrimal gland)

  2. CASE 1 Mass in supero-temporal orbit Painless proptosis

  3. Lacrimal gland Well defined, lesion

  4. Neoplastic glands embedded in a fibro (pink) -myxoid (blue) matrix

  5. Ducts are composed of bilayer of benign epithelium (inner epithelium and outer myoepithelial)

  6. Chondromxoid matrix

  7. DIAGNOSIS ?

  8. Benign pleomorphic adenoma Commonest benign lacrimal gland tumour. Orbital lobe usually-painless Frank bone invasion not present. Can become malignant Shouldn’t really biopsy-can seed and recur. Psuedo-encapsulated. Can transform into carcinoma.

  9. CASE 2 Painful superotemporal orbital mass.

  10. Swiss cheese appearance. Smoothly rounded sheets of deceptively Bland basaloid cells, containing round pools of mucin that mimic glands

  11. Tumour on both sides of pink lacrimal gland capsule = infiltration=malignant

  12. Adenoid cystic carcinoma 2nd most common epithelial lacrimal gland tumour Malignant Perineural invasion-therefore painful Various histological patterns Adenoid-’gland like’ bacause not true glands. The mucin pools develop after stroma is pinched off from outside. Poor prognosis

  13. For Lymphoma (Maltoma) See conjunctival session-exactly the same appearance

  14. CASE 5

  15. HISTORY • CHILD WITH PROPTOSIS

  16. Spindle cells in loose matrix, with scattered deep pink larger cells.

  17. Strap-like larger pink cells

  18. Cells positive for Muscle marker Desmin.

  19. Tumour nuclei positive for Muscle transcription factor MyoD1

  20. Rhabdomyosarcoma(embryonal type) Sarcoma Arise from pluripotential mesenchymal cells Commonest malignant orbital tumour of childhood Muscle marker positive Electron microscopy shows striations (can see on light microscopy sometimes) and Z bands. Immunohistochemistry detects muscle proteins-Desmin and MyOD1 transcription factor. After biopsy-chemo and radiotherapy usually 95 % 5 y survival for embryonal subtype.

  21. Other one…..Alveolar rhabdomyosarcoma • Nests of smaller round blue cells, with central discohesion. Peppered with some larger cells with pink cytoplasm. • Has 1: 13 or 2:13 specific and diagnostic cytogenetic translocation • Worse prognosis than embryonal

  22. DISCOHESION

  23. A note on orbital tumours • Adult-commonest primary-non-Hodgkin’s lymphoma. • Kids-commonest primary-rhabdomyosarcoma. • Other tumours in orbit: Adults-Primary orbital tumours: liposarcoma, malignant peripheral nerve sheath tumour, solitary fibrous tumour. Remember metastatic carcinoma. • Children-primary alveolar soft part sarcoma. Metastatic tumours commoner: neuroblastoma, leukaemia, Ewing’s sarcoma, Wilm’s tumour are commoner ones (Jerry and Carol Shields series).

  24. CASE 6

  25. HISTORY • 8 YEAR-OLD CHILD • REDUCED UNILATERAL VISION • FUSIFORM SWELLING OF OPTIC NERVE

  26. Normal Optic nerve

  27. ABNORMAL OPTIC NERVE expansion

  28. Expansion by tumour showing process bearing cells

  29. Process bearing cells alternating with fibrous areas containing intense pink bodies called Rosenthal fibres

  30. DIAGNOSIS ?

  31. Optic nerve pilocytic astrocytoma Unilateral visual loss and proptosis First 2 decades of life. NF-1 association Fusiform swelling of ON Confined by dura Extends through optic foraman to chaism. Cells, with many long delicate fibres, with more fibrous areas containing Rosenthal fibres. Cystic areas. Tumour often involves subarachnoid space-cardinal features. Rosenthal fibre = collections of alpha-beta crystalline. Slowly growing tumour-hardly ever fatal.

  32. CASE 7

  33. HISTORY • 40 YEAR OLD WOMAN • UNILATERAL VISUAL LOSS • PROPTOSIS • IMAGING SHOWS MASS AT SPHENOID RIDGE

  34. Tumour composed of bland spindly cells With occasional foci of dystrophic calcification

  35. Cells assume whorls

  36. Bland Interlacing spindle cells Bland=do not look cytologically malignant

  37. DIAGNOSIS?

  38. Meningioma Mostly benign Optic nerve sheath OR Sphenoid wing origins Many patterns-but classicial is whorls of meningothelial cells, +/-psammoma bodies (calcification arranged as concentric circles-like rings of a tree) Arise from arachnoid cells Female bias Express progesterone receptors Associated with NF-2

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