1. Orbit tumours (including lacrimal gland)

2. Mass in supero-temporal orbit Painless proptosis
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. Painful superotemporal orbital mass.
CASE 2
Painful superotemporal orbital mass.

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

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

13. 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

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

15. CASE 5

16. HISTORY
CHILD WITH PROPTOSIS

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

18. Strap-like larger pink cells

21. Cells positive for
Muscle marker Desmin.

22. Tumour nuclei positive for Muscle transcription factor
MyoD1

23. Rhabdomyosarcoma (embryonal type)
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.

24. 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

25. DISCOHESION

26. 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).

27. CASE 6

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

29. Normal Optic nerve

30. ABNORMAL
OPTIC NERVE
expansion

31. Expansion by tumour showing process
bearing cells

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

34. DIAGNOSIS ?

35. 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.

36. CASE 7

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

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

39. Cells assume whorls

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

42. DIAGNOSIS?

43. 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