extraconal tumours of orbit

1. Extraconal orbital tumors
By- AAKRITI HASIJA

2. EXTRACONAL SPACE
• It is the space within the orbit , outside the
musculofascial cone.
• Base – anteriorly , by orbital septum
• External side- by bones of the orbit and their
periosteum
• Internal side- by extraocular muscles and their
fascia

4. CONTENTS
• NERVES
-Trochlear nerve
-Trigeminal nerve (ophthalmic division)
*facial , lacrimal
-Trigeminal nerve (maxillary branches)
*zygomatic , infraorbital
• VEINS
- Superior ophthalmic
- Inferior ophthalmic
- Infra-orbital vein

5. Cont..
• ARTERIES
-extraconal branches of ophthalmic artery
*lacrimal
*supraorbital
*posterior ethmoidal
*anterior ethmoidal
*internal palpebral
*supratrochlear
*dorsal nasal
-infra-orbital artery
• LACRIMAL GLAND
• FAT

6. EXTRACONAL ORBITAL LESIONS
• From structures within the space:
-Dermid cysts/tumors
-Lacrimal gland tumors
-Capillary hemangioma
-Lymphangioma
- Rhabdomyosarcoma
-Plexiform neurofibroma
-Orbital pseudotumor
-Cavernous hemangioma
-Langerhans histiocytosis (<1% )
-Lymphoma/leukemia
-Orbital Leiomyoma

7. Cont.
• Lesions extending from adjacent structures
into the space:
-metastasis
-sinonasal tumors
*squamous cell carcinoma
*olfactory neuroblastoma
*lymphoma
*adenocarcinoma
*adenoid cystic carcinoma

8. DERMOID TUMORS
• it originates from aberrant ectodermal tissue
• Most common site – temporal zygomatic
suture line on the lateral orbital wall
• Lined by keratinized squamous epithelium
with adnexal structure (hair follicles ,
sebaceous glands)
• Young children are more affected , M:F=1:1

9. • Clinical presentation –
-proptosis (displace orbital structures)
-motility abnormalities and diplopia (pressing
on extraocular muscles)
- vision problems (due to compression of
optic nerve)
• Management – surgical resection / avoid
rupture

11. Lacrimal tumors/neoplasia
• Benign epithelial tumors
-pleomorphic adenoma
-reactive lymphoid hyperplasia
-oncocytoma
• Malignant epithelial tumors
-adenoid cystic carcinoma
-adenocarcinoma of lacrimal glands
-acinic cell carcinoma of lacrimal glands
-malignant lymphoma
-squamous cell carcinoma
Mucoepidermoid carcinoma

12. Cont.
• Non – epithelial tumours
-lymphoma
-orbital granulocyic sarcoma(choloroma)
-haemangioma
-solitary fibrous tumors
-metastates /secondary tumours

13. Pleomorphic adenoma of lacrimal glands
• Benign epithelial neoplasia of the lacrimal gland
• Occurs mostly in 2nd – 5th decade , M:F=1:1
• Clinical presentation –
-palpable superotemporal mass
-proptosis
-down and inward displacement of globe
-S-shaped contour the lid
- restricted upgaze
• Pt. complains of diplopia , reduced visual acuity ,
redness , watering and pain.

14. Cont.
• Fundus examination shows globe indentation in
half of the patients
• Histopathology –
-epithelial components derived from ducts , with
spindle muscle layer.
-presence of microscopic nodular extensions into
the pseudocapsule.
-may undergo squamous metaplasia

15. Cont .
• Imaging-
1. best seen on CT scan , showing pressure indentation
with expansion of lacrimal fossa
-well circumscribed tumor with nodular configuration
and pseudocapsule , calcification may be present
2. on B-scan – highly reflective pseudocapsule , cystic
spaces and well demarcated mass
3. On MRI – smooth well demarcated mass with fossa
formation
- T2 – weighted images are heterogenous
and isointense

17. Management and prognosis
• Complete extripation via a modified lateral
orbitotomy without capsule rupture
• Removal of a margin or adjacent tissue , excision of
the periorbita , preservation of the uninvolved
palpebral lobe.
• Two factors to prevent recurrence –
1. Careful surgical excision without capsular rupture
2. Pre-op diagnosis without incisional biopsy
(incase biopsy is done then involved periorbita ,
adjacent tissue and tract are removed with the
tumor)

18. Adenoid cystic carcinoma of lacrimal gland
• Most common malignant epithelial tumor
• In any age group , F>M
• Bimodal - 2nd and 4th decade , peak incidence in 4th decade of
life
• Clinical presentation –
-frontotemporal mass
-proptosis
-globe displacement
-ptosis
-pain
-parasthesia
-decreased vision

19. Cont.
• Pathology –
-Grossly , grayish white firm , pseudoencapsulated
- Histopathologically – 5 types
*cribriform (glandular/swiss cheese)- most common
*solid (basaloid) – least common , most aggressive
*tubular (ductal)
*sclerosing
*comedocarcinomatous
-infilteration is mostly to bones

20. Cont.
• Imaging –
- Important feature – evidence of lytic (irregular) change
to adjacent bone
- On CT scan – intrinsic lesion is well defined , solid and
homogenous , almond shaped mass
- On MRI – involvent of cavernous sinus , perineural
tissue
- T1-weighted images show diffuse enhancement and
T2-weighted images isointense to brain and extraocular
muscles

22. Management
• Well defined tumors - local excision along with
adjacent bone followed by brachytherapy or
radical external beam therapy
• For tumor extending to bones and soft tissue of
orbit – radical en bloc orbitectomy
- excision of orbital roof , lateral wall and
anterior portion of temporalis muscle is done
- reconstruction involve a myocutaneous
flap for radical radiotherapy
• Distant metastasis is usually to lungs

23. Capillary Haemangioma
• Infantile haemangioma
• A true neoplasm with vascular channels lined by
proliferating endothelial cells
• Common in infancy
• Clinical features –
- Proptosis
- Subtle pulsations can be seen
- Enlarge on valsalva maneuver or crying
- Blue-violet discoloration of lids and conjunctiva
- Refractive errors like myopia and astigmatism
- Amblyopia and strabismus can develop.

24. Imaging
• MRI is the preferred choice –
-lobular contour borders
-bright T2 signal
-intense, homogenous enhancement , fat
deposition
-preservation of adjacent bone
• On B-scan – smooth/irregular contour with high
echogenicity
• Blood flow is demonstrated on doppler
echography

26. Management
• Mostly they regress
• Radiotherapy (radon seed implantation or external
beam therapy) , systemic and local steroids.
• Systemic dosage is 1.5mg/kg to 2.5 mg/kg prednisone
daily for a few weeks with titration downwards.
• Local injection of 40-80 mg triamcinolone with 25 mg
methylprednisolone is given directly into the lesion
• In very large platelet consuming lesions , systemic
antifibrinolytic agents are used.
• In non responsive cases ,controlled resection can be
performed with constant haemostasis

27. Lymphangioma
• Benign vascular tumor with venous lymphatic malformations
• Slow growing
• Can enlarge suddenly due to intralesional bleeding or URTI
• Clinical features-
- Proptosis
- Blepharoptosis
- Cellulitis
- Intraorbital hemorrhage, subconjunctival hemorrhage , ecchymosis
- Astigmatism , hyperopia
- Corneal exposure
- Strabismus
- Glaucoma
- Compressive optic neuropathy

28. Imaging
• On CT – bony abnormalities , enlargement of
orbit
- Venous or solid components of tumor are super
dense
• On MRI – venous component typically enhance ,
lymphatic component only shows fine
enhancement of septations and
macrolobulations

30. Management
• Orbital surgery either on urgent basis if the
hemorrhage is causing optic nerve
compression or on elective basis for chronic
compression
• Circumferential panorbitotomy may be done
to excise much of the offending lesions

31. Rhabdomysarcoma
• Most common childhood soft tissue sarcoma ,
10% in orbit
• 70% occur in 1st decade , orbital
rhabdomysarcoma mean age is 7-8 years
• Bimodal peak with embryonal and alveolar
types. Anaplastic variety is rare.
• Mostly familial – positive family history of
malignancy , Li-Fraumeni syndrome , mutation
of TSG p53.
- congenital malformations and hereditary
retinoblastoma have been seen.

32. Clinical presentation
• Primary can occur from conjunctiva , iris , ciliary
body or extension of primary orbital
rhabdomyosarcoma.
• Secondary occurs fro direct extension to orbits from
surrounding structures
• Orbital metastasis can occur from head and neck
rhabdomyosarcoma , poor prognosis
• Orbital locations –
-extraconal (37%)
-intraconal (17%)
-both (47%)

33. • Presenting complaints
-unilateral proptosis
-globe displacement
-ptosis
-conjunctival and eyelid swelling
-palpable mass
-pain in few cases

34. Pathology
• 3 broad morphological categories:
-embryonal (80%) – classical pattern
-alveloar
-anaplastic/undifferentitated
• Rhabdomyoblasts have cross striations with abundant
eosinophilic cytoplasm having spindle, tadpole or
racquet shaped cells
• On histochemistry – stain with Masson trichome ,
periodic acid schiff (PAS) and phosphotungstic acid-
hematoxylin(PTAH) for acidophilia
- Immunoperoxidase for desmin in tumor
• Alveolar and anaplastic subtype have poor prognosis

35. Imaging
• On CT scan – homogenous , well defined soft
tissue masses without bone destruction
- focal areas of necrosis or hemorrhage can be
seen
• On MRI – T-1 weighted images are isointense
or hypointense to brain
-T-2 weighted images are hyperintense

37. D/D
• Rapidly developing childhood masses or inflammatory
conditions –
-neuroblastoma
-chloroma
-lympangioma
-infantile hemangioma
-cellulitis
• Other tumors –
-neuroepithelioma
-ewing’s
-malignant melanoma
-malignant lymphoma

38. Group number Criteria
I Localised disease, completely resected
A. confined to the organ or muscle of origin
B. infiltration outside organ or muscle of origin;
regional lymph nodes not involved
II Compromised or regional resection of three types including:
A .Grossly resected tumors with microscopic residual
B .Regional disease, completely resected, in which
lymph nodes may be involved and/or extension of
tumor into an adjacent organ may be present
C. Regional disease with involved lymph nodes,
macroscopically resected but with
evidence of microscopic residual
III Incomplete resection or biopsy with macroscopic residual
disease
IV Distant metastases present at onset

39. Management
• In complete resection of tumour – chemotherapy
alone
• In groups II , III , IV – radiation of 4500-5000 cGy
over 4-5 weeks
• Intracranial spread – whole cranial irradiation and
intrathecal chemotherapy
• Good prognosis for group I , II , III
• Poor prognosis for group IV
• Recurrences occur within 3 years and are treated by
chemotherapy with local or radical excision of
tumor

40. Complications of treatment
• 90% develop cataract after radiotherapy
• Other sequelae –
-keratoconjuctivitis
-dry eye
-radiation retinopathy
-lacrimal duct stenosis
-facial asymmetry
-growth retardation due to pitutary hypoplasia

41. Orbital leiomyoma
• Benign tumour arising from the smooth muscle cells
• Rare tumour of the orbit
• Presents in first two decades with no sex predisposition
• Clinically presents as painless proptosis or
displacement of the globe progressing slowly over
several months or years
• Histological features :
-spindle shaped cells arranged in whorls
-"cigar shaped" nuclei
-cytoplasmic eosinophilia
-myogenic filaments stain with Masson’s trichome

42. • Vascular smooth muscle cells in the orbit are currently
believed to be responsible for the histogenesis of this tumor
• The tumor is best diagnosed on CT scan which demonstrates
a well-defined, round to oval circumscribed mass with
moderate contrast enhancement
• Treatment – complete tumor excision as the tumor is not
radiosensitive
- a ring of surrounding tissue should be removed due to multiple
lobulations of the tumor
- Incase subtotal tumor excision is made then the pt is called
for serial neuroimaging studies for signs of recurrence
• D/D - Neurofibroma, fibrous histiocytoma, schwannoma and
amelanotic melanoma

44. Lymphomas
1. B-Cell lymphoma –
-lesions composed of ‘small’ B-cells .
-low grade lymphoma of mucosal associated
lymphoid tissue(MALT) is most common
-On histology diffuse, nodular and germinal
centres
-On Immunology - CD 5 - , CD 10 - , CD 20+ , CD 23-
/+ ,
CD 43 -/+
-Mantle cell lymphomas are more aggressive and
require therapeutic intervention

45. Clinical presentation –
• Seen in 6th- 7th decade of life , in the anterior orbit
• Subconjuctival tumefaction of typical salmon flesh
appearance , which tries to mould the globe
• In lacrimal glands , lymphoepithelial lesions of the
ducts
• Globe displacement
• Mild proptosis
• Secondary orbital and adnexal involvement is seen

46. • On CT scan – well defined , homogenous and
extraconal , lobulated / nodular
- lacrimal gland invovlement is common
-mainly involve soft tissue and seldom
extraocular muscles
• On MRI – TI weighted images – isodense to
hyperintense to extraocular muscles , hypodense
to orbital fat
- T2 weighted images – hyperintense to both fat
and muscles

47. Treatment
• Overall management by a multidisciplinary
team
• Localised orbital lesions – local radiotherapy
(3000 to 3500 cGy)
• Widespread disease - chemotherapeutic
intervention
• Prognosis depends on the age of the pt. ,
tumour systemic spread and histological grade
• Prognosis is generally excellent

48. Other B cell lymphomas
2. Diffuse large B – Cell lymphoma –
-aggressive , intermediate or high-risk orbital lymphoma
-orbital involvement arising in the paranasal sinuses is common
-consists of diffuse sheets of large neoplastic lymphoid cells
3. Burkitt’s Lymphoma – outgrowth of B-cells from germinal
centres , intermediate sized lymphocytes with basophilic
cytoplasm and multiple small nuclei.
-”starry- sky pattern” of phagcytic histiocytes
-13-16% presents with exophthalmos
-chemotherapy with cyclophosphamide , methotrexate etc
gives good prognosis.

49. • Very rarely orbit may develop secondary
lesions from the extraorbital sites of T cell
lymphoma

50. Leukemia
• Soft tissue involvement of the orbit is more
frequent and sudden in acute (esp. lymphoblastic)
rather than in chronic leukemia.
• In childhood malignancies of the orbit , acute
leukemia and granulocytic sarcoma are a frequent
cause of unilateral proptosis (11%)
• Bilaterality is seen in 2% of pts with orbital
leukemia.
• Local irradiation and both intrathecal and systemic
chemotherapy may significantly prolong survival.

51. Orbital metastases
• Average survival is approximately 9 months from
the time of orbital presentation , where the
primary cancer has occurred 31 months before
• Breast cancer has of a delay 3 yrs , thyroid – 5 yrs
between the primary and orbital presentation
• Lung cancer , melanoma and GI tumors have an
early orbital presentation(3.6 average) n poor
prognosis
• RCC and metastatic carcinoid – slow growing ,
solitary orbital metastasis

52. Clinical presentation
• Mass – axial /non axial displacement of globe
• Infiltrative- diplopia , enophthalmos , limitations of eye
movement (frozen globe) , firm orbit
• Functional – decrease in 2nd , 3rd , 4th , 5th , 6th nerve function ,
out of proportional to mass or infilteration
• Inflammatory – pain , chemosis , injection , erythema , lid
swelling
• Silent - no s/s , discovered accidentally on CT/MRI
orbit/Enucealtion of eye
-Motility disturbance out of proportion to the degree of
proptosis can occur and is characteristic of orbital metastasis

53. Syndromes of presentation
• Syndrome of mass (66%) – most common
• 2nd most common is infiltrative presentation ,
eg in metastatic breast cancer (scrrihous) , GI ,
prostate , lung and other primary tumours
• Least common presentation – inflammatory
and functional . Seen in small tight places-
orbital canal / apex

54. Diagnosis and treatment
• Complete history and clinical examination should be
done
• Specific and non specific laboratory tests eg. CEA
• Radioimaging should be done
• Needle biopsy is the best application if suspecting
metastatic tumors
• Proper immunohistochemistry of the sample taken
-although life expectancy is less in orbital metastases
-various treatment modalities can be undertaken to
increase life expectancy in the form of radiotherapy ,
chemotherapy hormonal therapy and surgery

55. Sinonasal carcinoma
• Rare , highly aggressive , cliniopathologically
distinct .
• It is locally invasive often invading to skull base.
• Arises from the mucosal lining of the nasal cavity
and paranasal sinuses.
• It is composed of pleomorphic tumor cells with
necrosis.
• Due to its invasive nature to orbit, it results in
proptosis , cranial nerve palsies, visual
disturbances and pain.

57. Management
• Earlier “orbital exenteration” was done to
complete removal of the contents of the orbit
including the eyelids
• Now a days , “orbital clearance “ is done in
which the globe , muscles , fat and the
periorbita are removed
- Lids , palpebral conjuctiva are preserved for
reconstruction

58. TUMOUR CLINICAL FEATURES AGE OF ONSET TREATMENT
DERMOID CYST Proptosis,Diplopia,
Defective Vision
Childhood
M:F=1:1
Surgical
Resection,Avoid
Rupture
LACRIMAL GLAND
Benign-
PLEOMORPHIC
ADENOMA
Malignant-
ADENOID CYSTIC
CARCINOMA(ACC)
PLEOMORPHIC-
Superotemporal
Mass,S shaped
contour of
lid,Diplopia,Eccente
ric Proptosis,Def Vn
ACC-Pain,Proptosis,
Ptosis,Frontotempo
ral mass,Diplopia
Benign-2ND -5TH
Decade,M:F=1:1
Malignant-Bimodal
2nd and 4th decade,
Peak incidence-4th
decade,F>M
PLEOMORPHIC-
Modified Lateral
Orbitotomy without
capsular rupture
ACC-Well defined-
local excision with
brachytherapy
Radical en block
orbitectomy for
spreading tumours
CAPILLARY
HAEMANGIOMA
Port Wine
stain(Blue violet
discolouration of
lids and
conjunctiva),Propto
sis,squint and
Amblyopia
Infantile Mostly Regress,If
not,then
Radiotherapy,Syste
mic Antifibrinolytic
agents ,Controlled
Resection in worst
cases

59. TUMOUR CLINICAL FEATURES AGE OF ONSET TREATMENT
RHABDOMYOSARCOMA U/L Proptosis,Globe
Displacement,Palpable
mass,Ptosis,Conjunctival
and lid swelling,Diplopia
Most commom
childhood soft
tissue
sarcoma,7-8
yrs mean age
Chemotherapy in
complete resection
of tumour,
Groups 2,3,4-
Radiation of 4500-
5000 cGy in 4-5 wks
Intracranial spread-
whole cranial
irradiation and
intrathecal
chemotherapy
ORBITAL PSEUDOTUMOUR Diagnosis of Exclusion,U/L
Painful Proptosis and
Diplopia,Extra ocular
muscles most commomly
involved
Any age –
Differentiated
from Thyroid
eye disease as
latter spares
the tendinous
insertions of
EOM and not
painful
Steroids-mainstay

60. TUMOUR CLINICAL FEATURES AGE OF ONSET TREATMENT
LYMPHANGIOMA
(Benign)
Proptosis,Blepharoptosis,cell
ulitis,Intraorbital and
Subconj
haemorrhage,squint,glauco
ma,corneal
exposure,hyperopia
Childhood
(Congenital)
Urgent Orbital
surgery if
haemorrhage is
causing optic nerve
compression or on
elective basis for
chronic compression
LYMPHOMAS
B-Cell,Diffuse Large B
cell and Burkitt’s
Lymphoma
B-Cell-Globe
displacement,Proptosis,lymp
hoepithelial lesions of
lacrimal ducts, orbital and
adnexal involvement
6th -7th decade Localised lesions-
local radiotherapy
3000-3500 cGy
Widespread disease-
Chemotherapy
LEUKAEMIA Soft tissue involvement of
the orbit is sudden in acute
leukaemia rather than
chronic,U/L Proptosis
Childhood Local Irradiation and
intrathecal and
systemic
chemotherapy
prolong survival

61. Thank you