This document provides information on lacrimal gland tumors, including: 1) Lacrimal gland tumors typically present with upper eyelid fullness, alteration of the eyelid contour, and downward displacement of the globe. They can be either epithelial or non-epithelial tumors. 2) Epithelial tumors include pleomorphic adenoma (the most common), adenoid cystic carcinoma, and adenocarcinoma. Pleomorphic adenomas are usually benign but can become malignant. Adenoid cystic carcinoma often invades surrounding bone. 3) Treatment involves complete surgical removal with postoperative radiation for malignant or invasive tumors. Prognosis depends on tumor type, with adenocarcinomas having

1. OPRC CLUBPanit Cherdchu M.D.
Ophthalmology department
Phramongkutklao hospital

2. THE MAGIC “SEVEN P”
3 P from history
• Pain
• Progression
• Past medical history
4 P from examination
• Proptosis
• Palpation
• Pulsation
• Periorbital change

3. • Infection, inflammation, hemorrhage, bone and neural invasionPain
• Hour : hemorrhage, Days : infection, Week : inflammation, Month :
malignant,Year : benignProgression
• Thyroid, trauma, cancerPast medical history
• Next slide pleaseproptosis
• Retrobulbar resistant, mass (consistency, periorbital)palpation
• AV fistula, touch durapulsation
• Next slide pleasePeriorbital change

4. proptosis
• Bird eye’s view
• Worm eye’s view
• Exophthalmometry :Thai male 14-16 ,Thai female 14-15 (+/- 2 mm)
• Direction : axial vs non-axial
• Inferomedial : lacrimal gland tumors or lymphoma
• Inferotemporal : frontal sinus mucocele
• Lateral : ethmoid sinus mucocele, tumor, subperiosteal abscess
• Superior : lymphoma, maxillary sinus tumor
• Medial : rare
• Axial :TED, cavernous hemangioma,ON glioma,ON sheath meningioma

5. Periorbital change
Sign Etiology
Salmon-colored mass in the cul-de-sac Lymphoma
Eyelid retraction, vascular congestion over
rectus muscle
TED
Corkscrew conjunctival vessels AVF
S-shaped eyelid Plexiform neurofibroma or lacrimal gland mass
Ecchymosis of eyelid skin Metastatic neuroblastoma, leukemia, amyloidosis
Prominent temple Sphenoid wing meningioma, metastatic
neuroblastoma
Edematous swelling of lower lid Meningioma, inflammatory tumor, metastases
Frozen globe Metastases or zygomycosis
Black-crusted lesions in nasopharynx Phycomycoses
Facial asymmetry Fibrous dysplasia or neurofibromatosis

6. LACRIMAL GLANDTUMOR
definition – classification – etiology – investigation - management

7. presentation
Lacrimal gland tumors typically present
with upper eyelid fullness, alteration of
the upper eyelid contour, and downward
and nasal displacement of the globe.
Lacrimal fossa masses consist of
inflammatory, neoplastic, and structural
disorders

8. introduction
• Lacrimal gland tumors are rare
• 5-18% of orbital space-occupying lesions
• Incidence of 1:1,000,000 per year
• More prevalent in elderly

9. LACRIMAL
GLANDS

10. Lacrimal
gland tumors
50% epithelial
50% pleomorphic
adenoma
50% carcinoma
50% adenoid cystic
carcinoma
50% other
carcinoma
50% non-epithelial
Lymphoproliferative &
inflammatory

11. NON-EPITHELIALTYPE

12. Inflammatory
• Acute onset of swelling associated with periorbital
pain, chemosis and indurated lid indicate an
inflammatory process of either idiopathic
(pseudotumor) or of infective etiology
(dacryoadenitis).
• A computed tomography scan usually reveals a
diffuse lacrimal enlargement with irregular margins,
frequently demonstrating contrast enhancement and
no bony change.
• Idiopathic orbital inflammatory disease
(pseudotumor) is treated with a short course of
corticosteroids.
• In case of failure to resolve over a few weeks, an
incisional biopsy should be taken should, since acute
inflammatory episodes may be related to an
underlying carcinoma.

13. Lymphoproliferative lesions
• These are characterized by insidious onset, painless proptosis in a slightly older
population and is often bilateral.
• CT scans show that all lymphoid tumors mold themselves around the existing
orbital structures, such as the globe and the bony orbit, without eroding bone or
enlarging the orbit

15. EPITHELIALTYPE

16. Benign malignant
Pleomorphic adenoma Malignant mixed tumor
Myoepithelioma Adenoid cystic carcinoma
Oncocytoma Adenocarcinoma
Cystadenoma Mucoepidermoid carcinoma

17. Pleomorphic
adenoma
• Benign mixed tumor
• Tumors derive from a
mixture of epithelial and
mesodermal elements
• Most in 40-50th year of age ,
but case report in children
of 6 yearsold
• Men = women

18. • Mostly involve orbital lobe of lacrimal gland
• 10% involve palpebral lobe
• Palpebral lobe tumors are freely movable, non-tender, shorter
duration

19. MANIFESTATION
unilateral
progressive
proptosis Downward
and inward
displacement
of the globe
Ocular
motility
disturbance
Course of
progression
12 months
with no
inflammatory
sign
Diplopia
Painless
Orbital
discomfort
Choroidal
fold
Ptosis
RF error
change

20. Imaging
• CT images : round to oval , well-define
lesions that are smooth in outline,
displacing and deforming the globe
• MRI images : low signal intensity on theT1-
weighted image and of high signal intensity
on theT2-weighted image, frequently of
heterogenous distribution, pseudocapsule
may appear

25. Management and prognosis
• Best to complete excision of the tumor within its pseudocapsule via a lateral
orbitotomy
• Adequate margin of the surrounding lacrimal gland and the adjacent periorbita
should be removed in the extirpation
• Make sure no residual tumor remain
• High recurrence rate following incomplete excision or incisional biopsy
• 5-year recurrence rate was 3% in complete removal
• 5-year recurrence rate was 32% in incomplete removal
• Malignant change in 10% by 20 years after treatment

26. Malignant mixed
tumor
• 4-15% of epithelial
tumors of the lacrimal
gland
• Patients with malignant
mixed tumors tend to be
older than those with
pleomorphic adenoma

27. 3 ways of presentation
1. Patient whose benign mixed tumor was not removed totally may develop a
sudden recurrence several years later
2. Indolent long-standing lacrimal tumor history presents with sudden expansion
of the mass, pain, swelling of upper eyelid
3. Rapidly developing symptoms of pain and bone destruction and first diagnosed
as malignant at the first presentation

29. Imaging
• CT imaging : enlarged lacrimal
fossa surrounded by bone
destruction means malignant
tumors
• The bone window shows the
osseous changes best
• Contrast enhancement helps to
reveal involvement of the dura and
intracranial extension

30. Management and prognosis
Transeptal biopsy followed by complete removal of the tumor
one-stage procedure for the surgical removal of the tumor and adjacent adnexa: an
en bloc resection of the neoplasm, its periorbital base, and surrounding bone.
A radical orbitectomy with regional and cervical lymph node dissection has been
advised because an adenocarcinoma arising in a benign mixed tumor may
disseminate early via lymphatics.
Postoperative radiotherapy may also be recommended
If metastases have occurred, treatment is often limited to surgical debulking
followed by postoperative radiotherapy
High mortality rate 30% in 5 years, 45% at 11 years, 50% by 12 years

31. Adenoid cystic
carcinoma
• Second most common epithelial tumor of
the lacrimal gland and the most common
malignant epithelial tumor of the lacrimal
gland
• 1.6% of all orbital tumors
• 3.8% of all primary orbital tumors
• 40 years of age at presentation, range 6.5-79
years

32. Clinical features
• Mass effect with a more rapid temporal sequence usually under 1 year
•Pain
• Globe displacement
• Mass or swelling
• Numbness
• Diplopia
• Visual change
• Lacrimation
• Ptosis
• Invades perineurally and into adjacent bone  pain
+numbness

33. Imaging
• CT imaging : elongated mass
extending along the lateral orbital
wall, with expansion of the lacrimal
fossa with bone invasion
• Globular or round, irregular and
serrated borders than those seen in
pleoemorphic adenoma
• Bone erosion 75%
• Bone destruction 34%
• Soft tissue calcification 22%
• Contrast enhancement helps to
reveal involvement of the dura and
intracranial extension

34. • MRI + enhancement : best for
assessing the invasion of the
tumor into the cavernous sinus,
brain, and bone marrow
• The tumor is hypointense onT1-
weighted image and
hyperintense on theT2-
weighted image with contrast
enhancement

38. pathology
• The gross appearance of adenoid cystic
carcinoma is grayish-white, firm, nodular,
and deceivingly circumscribed
• Microscopically, five histologic patterns
have been described: cribriform (“Swiss
cheese”), solid (basaloid), sclerosing,
comedocarcinomatous, and tubular
(ductal), in order of frequency.32 All or
several of these patterns may be present in
one tumor, but one pattern usually
predominates

39. Management and prognosis
• The best is yet to come
• Tumor removal and postoperative radiotherapy
• Surgical technique : local resection, en bloc removal, exenteration, and radical
exenteration(radical orbitectomy).25,33–37 Radical exenteration with removal of the
orbital roof, the lateral wall, and the anterior portion of the temporalis muscle where the
zygomaticofrontal and zygomaticotemporal nerves extend has been recommended
• Radiation therapy in the dose of 50 to 60 Gy after local resection of adenoid cystic
carcinoma significantly delays the onset of tumor recurrence and prolongs survival.
• Implanted sources of radiation (brachytherapy)
• Intra-arterial chemotherapy

40. Adenocarcinoma
• More common in males
• Older population ranging from 18 to 80 years
• 7% of epithelial neoplasms of the lacrimal gland
• Metastasizes earlier and is associated with a shorter patient survival time
• Rapidly growing mass, exceeding the limits of adequate surgical excision at the time of presentation
• Proptosis
• Pain
• Globe displacement
• Visual loss
• Diplopia
• Ptosis

42. Management and prognosis
• Short survival time 1.5 years from initial presentation
• Exenteration followed by radiation therapy
• Monobloc craniofacial orbitectomy combined with regional LN dissection

43. Mucoepidermoid carcinoma
• Most common primary carcinoma of the salivary glands
• Rare in lacrimal gland
• Age 12-81 years old, average 49
• Male > female
• Present as painless, slowly enlarging mass in lacrimal gland fossa
• Might be mistaken for benign mixed tumor

44. Management and prognosis
• Clinical behavior and prognosis parallel the histologic grading
• 7/8 patients of low grade tumor survived
• 1/8 patients in high grade tumor remain tumor free
• Perform exenteration, radiation, and resection of involved orbital bone for patient
with high-grade tumors
• Perform extirpation with or without adjuvant radiation for patient with low-grade
tumor

45. Other tumors
• Acinic cell carcinoma
• Oncocytoma (oxyphil cell adenoma)
• Spindle cell myoepithelioma
• Sebaceous carcinoma
• Solitary fibrous tumor
• Malignant rhabdoid tumor
• hemangioma

46. TAKE HOME MESSAGE

48. THANKYOU FORYOUR COOPERATION