Orbital And Peri-Orbital

Tumours

Orbital And Peri-Orbital

Tumours
Acute proptosis of the orbit is usually a harbinger of serious
ophthalmologic disease. The varied anatomic structures within
the orbit allow it to host numerous disorders, including
infectious, inflammatory, immune mediated, vascular, and
neoplastic disease. While some of the diseases producing
proptosis (e.g., inflammatory pseudotumor) are labeled benign,
they almost always produce significant morbidity if diagnosis
and treatment are delayed. Failure to properly diagnose
malignant lesions producing acute proptosis (e.g., lacrimal gland
carcinoma) can lead to both morbidity and mortality. We have
outlined the most common diseases that ophthalmologists
encounter and provided a framework for diagnosis and
treatment.

LYMPHOID TUMORS
Orbital involvement with lymphoid tumors is characterized by
subacute development of painless proptosis, lid swelling, and diplopia.
A more fulminant course with mild pain, motility disturbance, and
decreased vision has been observed in the poorly differentiated
lymphomas. Lymphoid tumors may occur anywhere in the orbit but
have a predilection for superior and anterior locations, often resulting
in downward displacement of the globe. A firm mass, not fixed to
bone, is frequently palpable. Subconjunctival extension, presenting as
a fleshy, salmon-colored mass of the conjunctival fornix, may occur.
On CT scan, lymphoid tumors typically appear as an enhancing soft
tissue mass, usually oblong in shape, with well-defined margins and
puttylike molding to the contours of adjacent orbital structures. Orbital
bone erosion is rare in lymphoid tumors

Lymphoma

Lymphoma
LYMPHOPROLIFERATIVE DISORDERS
HISTOLOGICAL CLASSIFICATION
BENIGN REACTIVE LYMPHOID HYPERPLASIA 15% systemic
disease
ATYPICAL LYMPHOID HYPERPLASIA
40% systemic disease
NON-HODGKINS LYMPHOMA
60% systemic disease

Lymphoma

Unifying feature is dense cellular infiltrate of lymphocytes, usually B cells. (Germinal centre
formation, polyclonality and lack of cellular atypia indicate benign lesions).
Some polyclonal tumours may become malignant and some monoclonal tumours may remain benign.
NHL can be classified histologically, immunocytochemically and genetically.

CLINICAL

>60yrs
Insidious painless mass / proptosis
Predilection for anterosuperior orbit and lacrimal gland
Look for smooth salmon patch in superior fornix (BRLH often nodular)

Lymphoma

DIFFERENTIAL DIAGNOSIS
Chronic pseudotumour
Dermoid
Mixed adenoma of lacrimal gland

INVESTIGATION
CT scan:- well defined homogenous mass moulded to globe and other orbital structures,
Bony destruction unusual
INCISIONAL BIOPSY :- send fresh specimen for immunocytochemistry and formalin specimen

SYSTEMIC INV: FBC, LFT, EUC, EPG/IEPG, CT abdomen, pelvis and chest, bone marrow biopsy
Physician review

TREATMENT
1. observation: BRLH
2. systemic steroids: BRLH
3. radiotherapy: localised lesion, 2000rads
4. chemotherapy: if systemic disease, use DXT to orbital lesion if there is a poor response to chemotherapy
5. surgical excision / debulking

PROGNOSIS
Visual prognosis good unless radiation retinopathy
All patients with localised lesions need continual review for development of systemic disease

CAVERNOUS
HAEMANGIOMA

commonest benign orbital neoplasm in adults

PATHOLOGY
Dilated vascular lakes lined with endothelial cells
Usually intraconal causing progressive, painless, axial proptosis
May enlarge due to bleeding, pregnancy
INVESTIGATION
B scan - high internal reflectivity
CT scan - round intraconal mass
DIFFERENTIAL DIAGNOSIS
Haemangiopericytoma, neurolemmoma, meningioma, glioma.

TREATMENT
Indications: pain, loss of vision, diplopia, proptosis, histological diagnosis

Excision via lateral orbitotomy.

LYMPHANGIOMA

children and young adults

Involve lids, conjunctiva and orbit (3% of orbital tumours).NOTE: Normally there are no lymphatic vessels in the orbit.
May present within the first weeks of life up to the age of 10 years.
Often extend deep into normal tissues of the orbit with ill-defined margins making excision difficult.

They appear as soft masses, which do not expand in contrast to orbital varices which do enlarge during a valsalva manoeuvre.
Nevertheless mixed lesions can co-exist, so within a lymphangioma there may be some orbital varices.

Lymphangiomas may bleed internally causing rapid enlargement leading to chocolate cyst formation and dramatic proptosis.

INVESTGATION
They may enlarge rapidly and in which case the differential diagnosis includes rhadomyosarcoma and neuroblastoma.
CT scan to show bony landmarks bone destruction does not occur but pressure can cause smooth remodelling.
MRI the appearance varies with the blood if any present: hypointense or hyperintense.

TREATMENT

Deep lymphangiomas are best managed by observation: surgical excision is difficult and liable to result in further haemorrhage.
Superficial lesions (which appear bluish or transilluminate) may be easily removed.
In cases of proptosis where the optic nerve is threatened aspiration of a chocolate cyst may be useful.

CAPILLARY HAEMANGIOMA
Lobules of proliferating endothelial cells present within the first few weeks of life.
Behaves as a tumour with rapid growth (may be mistaken for rhabdomyosarcoma and in
these cases a biopsy is required).
Predilection for the superonasal orbit.
Astigmatism is the very common with the steep axis in the direction of the mass.
Superficial lesions have a lobulated surface and are aptly named strawberry haemangiomas.

Rarely systemic involvement: visceral, respiratory, spinal cord

If large capillary haemangiomas can result in the Kasabach-Meritt syndrome:Platelet trapping causing
thrombocytopenia and fibrinogen depletion,and CCF due to AV shunting.

PROGNOSIS
No treatment will give the best cosmetic results.
Tendency to grow for the first 12 months and then spontaneously involute.
But amblyopia is common and should be prevented or treated !

CAPILLARY HAEMANGIOMA

TREATMENT
Indicated only if amblyogenic or causing a systemic problem
Steroids: short courses of systemic steroids (up to 2mg/kg for 3-6 weeks and repeated if
successful).

May need to taper over some months (otherwise a growth rebound may occur.Patients will need
paediatric monitoring.
Intralesional injection (use a mixture of a short and long acting steroid such as betamethasone and
triamcinolone). Avoid injecting into the normal skin peripheral to the lesion as it may lead to skin
atrophy. Injections may be repeated.
Remember to refract.
Surgical excision, cryotherapy
Interferon a 2a.

Laser (yellow wavelength).

Radiotherapy- 150 rads/month x 6 months

VENOUS MALFORMATIONS

The orbital venous anomalies include varix, varicocele, and the venous
angioma. These malformations may be confined to the orbit (primary),
or may be associated with an intracranial arteriovenous malformation
(secondary). The classic history is one of intermittent proptosis
associated with positional changes or Valsalva maneuvers. Hemorrhage
or thrombosis within the lesion may be associated with sudden
proptosis, pain, nausea, and vomiting, especially if it is situated deep in
the orbit. Some patients have ecchymosis and swelling of the eyelids,
chemosis, motility disturbance, and visual deficit. CT scan with contrast
enhancement reveals the extent of the malformation. Orbital varices
often have phleboliths with calcification, which are easily diagnosed on
CT scanning. Management of venous malformations, including
lymphangioma, consists primarily of observation.

ORBITAL VARICES
Intermittent proptosis: demonstrated by the valsalva manoeuvre.

May be divided into high flow and low flow varices:

Low Flow Varices

Really indistinguishable from a lymphangioma.
Congenital but may present acutely due to internal haemorrhage

High Flow Varices

Expand with jugular vein pressure.
May have an intracranial communication.
These ones rarely bleed!
TREATMENT
Observe,
Interventional radiology may have a role to play.

Excise anterior lesions?

Cauterise? orbital decompression?

Surgery is indicated for compromise of the orbital structures or, rarely, for cosmetic disfigurement.

HAEMANGIOPERICYTOMA

Proliferating pericytes which may undergo malignant transformation.

Usually extraconal in the superior orbit.
The tumour spreads by locally invasion.

TREATMENT
Surgical excision.
Try to preserve the pseudocapsule.
Recurrences may require exenteration.

CAROTICO-CAVERNOUS FISTULA

TRAUMATIC

Usually high flow, young adults

SPONTANEOUS

Often low flow, elderly hypertensive females

CLINICAL
Arteriolisation of conjunctival vessels marked engorged chemosis, proptosis
Ophthalmoplegia,
Glaucoma (episcleral pressure increased; also ocular hypoxia due to venous stasis causing rubeosis; ciliary body swelling due to vortex vein pressure which can result in ACG)

INVESTIGATION
CT scan- enlarged muscles / superior ophthalmic vein / proptosis.
Angiography.

TREATMENT
Spontaneous recovery, embolisation
Electrocoagulation sinus
Intracavernous repair
Ligation ICA

CAROTICO-CAVERNOUS FISTULA

CAROTICO-CAVERNOUS FISTULA

RHABDOMYOSARCOMA
Rhabdomyosarcoma is the most common soft-tissue sarcoma in
patients under 15 years of age and the most common primary
orbital malignancy in childhood. These facts should not imply its
frequent occurrence. Including all body sites, the annual incidence
of rhabdomyosarcoma is between one case per 4 million and one
case per 1 million children under 15 years of age.2 The orbit is the
site of origin in 5% to 25% of cases.2,3 Although relatively rare,
the tumor has a devastating natural history and demands a high
index of suspicion in all cases of pediatric proptosis.
Orbital rhabdomyosarcomas are more common in males than
females by a ratio of approximately 5:3,4 The average age of
presentation is 7.8 years.

RHABDOMYOSARCOMA

The commonest primary malignant orbital tumour in

M>F, average age 8yrs, may be familial

PATHOLOGY
Composed of primitive malignant mesenchymal cells in varying stages of
embryogenesis
EMBRYONAL: commonest, paucity of cross striations
ALVEOLAR:
tumour cell cytoplasm forms alveolar type structures, most
malignant
PLEOMORPHIC: adults, more differentiated, cross striations prominent, best
prognosis

children

RHABDOMYOSARCOMA

CLINICAL
Sudden, rapidly progressive proptosis
Redness and oedema, pain is late feature

INVESTIGATION
CT scan

Biopsy - formalin for LM and glutaraldehyde for electron microscopy

CXR, bone marrow and LP

TREATMENT
Local irradiation (4500- 6000 rads) and chemotherapy

PROGNOSIS
90% survival if confined to orbit, poor prognosis if bony involvement
Overall survival = 65%

Biopsy
The clinical diagnosis must be confirmed by biopsy.
Because of the risk of seeding the biopsy tract, a
transcranial approach is contraindicated. If possible, the
periosteum should not be violated because it presents a
relative barrier to tumor invasion. The lesion should be
approached trans-conjunctivally or through a transseptal
eyelid incision. The surgeon must balance the advantages
of tumor debulking with the risk of dissemination that may
accompany excessive manipulation. Tissue samples should
be fixed in both formalin and glutaraldehyde for light and
electron microscopic study.

BENIGN ORBITAL INFLAMMATION

LYMPHOPROLIFERATIVE
TUMORS
Benign orbital inflammation (inflammatory pseudotumor) is a
general term that may be used to encompass all
nongranulomatous inflammatory lesions of the orbit that lack
a specific etiology. Histopathologically, these benign, reactive
lesions are composed of varying degrees of polymorphic
infiltration by inflammatory cells, including lymphocytes,
plasma cells, eosinophils, and macro-phages. These disorders
often involve more than one orbital structure, but may be
classified according to the predominant anatomic site of
involvement, such as anterior, posterior, myositis, lacrimal,
optic nerve, or diffuse.

PSEUDOTUMOUR

Focal or diffuse, non-granulomatous orbital inflammation of unknown aetiology affecting any tissue within the
orbit.

PATHOLOGY
Polymorphous infiltrate with lymphocytes, plasma cells, fibroblasts.
Perivascular lymphocytic cuffing.
Fibrosis occurs in late stage

CLINICAL
30-50yr
Unilateral (bilateral rarely)
M=F
Painful proptosis progressing over a few months
Conjunctival injection and chemosis over the recti muscles is common.
Myositis commonly affects IR (cf. thyroid)

+/- CNV involvement, meningeal irritation

Prednisone 1mg/kg/day for 2-4 weeks, then taper
Radiotherapy 1500rads

PSEUDOTUMOUR

DIFFERENTIAL DIAGNOSIS

Thyroid eye disease, tumour, orbital cellulitis, systemic vasculitis (SLE, PAN),
lymphoma, lacrimal gland carcinoma
INVESTIGATION
1. B scan:- echo free zone posterior to sclera due to oedema in Tenons capsule, EOM
enlargement, discrete mass
2. CT scan:- EOM- muscle and tendon thickened (cf. TED- only muscle belly
thickened), inflammatory infiltrate of retrobulbar fat pad,scleral enhancement with
contrast due to tenonitis

3. Incisional biopsy:- cover pre and post op with steroids as will exacerbate pre-existing
inflammation

PSEUDOTUMOUR

BASAL CELL CARCINOMA

( BCC )

The commonest form of lid neoplasia.

Most commonly occur on the lower lid.
Metastases do not occur but local spread occurs.
Medial canthal BCCs are more likely to go deep thereby
involving orbital structures.
Basal Cell Naevus syndrome causes multifocal BCCs
Xeroderma pigmentosa (autosomal recessive) predisposes
BCC SCC and melanoma.

BASAL CELL CARCINOMA

( BCC )
CLINICAL
Nodular and nodular-ulcerative type:
Typical nodules and
telangiectasia ulceration.
Cystic type: May resemble a benign epithelial inclusion cyst.
Sclerosing / Fibrosing / Morpheic type: Easily missed. May present
as loss of lashes, ectropion, lid notching etc. Requires wider excision
and follow-up.
MANAGEMENT
Excision: is the ideal treatment which can be backed up by histology.
Radiotherapy: useful in selected cases but it can result in long term
complications such as skin atrophy and canalicular stenosis.

BASAL CELL CARCINOMA

( BCC )

SQUAMOUS CELL CARCINOMA OF

LIDS ( SCC )

A malignant, invasive tumour arising from the epidermis with evidence of keratinisation.

Only occur 1: 40 as often as BCC lid tumours.

The margin of the lower lid is the commonest site of origin.
Increased incidence in folk with fair complexions and sun exposureradiotherapy can predisposedefective
DNA repair (Xeroderma pigmentosa)
Actinic keratosis predisposes to SCC possibly with a more benign course than cases arising from carcinoma
in situ.
Squamous cell carcinomata may metastasise. Nevertheless fatalities due to SCC secondaries from the lid are
extremely rare.

HISTOLOLOGY
Squamous carcinoma cells have eosinophilic cytoplasm. Focal keratinisation causes keratin pearls.
Disordered cell keratinisation is known as malignant dyskeratosis.
Most squamous cell carcinomas of the lids are well differentiated.
TREATMENT
Aim for complete surgical excision.

SEBACEOUS CELL CARCINOMA OF

LIDS

Highly malignant and prone to misdiagnosis or late diagnosis.

Fortunately less than 1% of lid tumours are due to sebaceous cell carcinoma.
Radiotherapy eg. for retinoblastoma appears to predispose.
Typically they originate from the meibomian glands of the upper or lower lids.They may also arise from sebaceous glands of the
eyebrow, from the sebaceous glands of Zeiss close to the lashes, or of the caruncle.
Sebaceous cell carcinomas enlarge by nodular infiltrative growth or by Pagetoid intraepithelial growth, which makes the tumour
extent difficult to define.

The upper lid is more often involved than the lower lid.
They may resemble chalazia or papilloma or lacrimal gland tumours.
Many of the tumours have a yellowish colouration.
Madarosis is usual for tumours near the lid margin.

HISTOLOLOGY
Large anaplastic cells with open vesicular nuclei with prominent nucleoli and frothy cytoplasm.
The cytoplasm contains lipid vacuoles which are normally dissolved by alcohol in routine processing.
Frozen sections stained with oil red O (for lipid content) can be useful if there is diagnostic difficulty.
Usually there is a high mitotic rate.
MANAGEMENT
Complete excision which may require exenteration where there is orbital spread.

CARCINOMA OF THE LACRIMAL

GLANDS

Primary malignant epithelial carcinoma of the lacrimal gland produces

progressive unilateral proptosis and downward and medial
displacement of the globe. These findings usually develop over a
relatively short period of time. Adenoid cystic carcinoma and
pleomorphic adenocarcinoma (malignant mixed tumor) constitute the
majority of the malignant lacrimal gland tumors.
Typically, the patient is young or middle-aged and presents with
progressive proptosis, pain, blepharoptosis, and diplopia. A firm,
palpable mass may be present in the superotemporal quadrant.
Occasionally, a benign mixed lacrimal gland tumor undergoes
malignant degeneration, which manifests as acute proptosis.
These patients may have a history of prior excision of a benign mixed
lacrimal gland tumor.

Metastatic Ocular Carcinoma

The eye may not infrequently be the site of tumour metastases, the
most frequent primary site is the breast in females and the bronchus in
males, often these secondaries metastasize to the choroid. Other less
common sites include kidney, testis, gastrointestinal tract. The prostate
is a rare primary site. Weiss and Kanski note that the uveal tract is a
highly favoured site for metastases. The incidence of metastases to the
uvea is compared with that in eight other (extraocular) target sites, in
patients with metastatic primary carcinomas of the breast, colorectum,
and lungs. When the incidence of intraocular metastases was viewed
in relation to the calculated numbers of cancer cells delivered via the
arterial route, the uveal tract is the most highly favoured target site for
the development of metastases per unit of delivered cancer cells.

SUPPORT GROUPS
Ocular cancer is an extremely disturbing diagnosis, best practice
involves prompt referral to an ocular oncologist without delay. Support
groups for patients with ocular tumours inlude:
Cancer of the Eye Link Line ( CELL)
PO BOX 2586, Radstock, Bath BA3 2YP
HELPLINE: 01761-411 055