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

LymphomaLYMPHOPROLIFERATIVE DISORDERS

HISTOLOGICAL CLASSIFICATION

BENIGN REACTIVE LYMPHOID HYPERPLASIA 15% systemic diseaseATYPICAL LYMPHOID HYPERPLASIA40% systemic disease

NON-HODGKINS LYMPHOMA60% systemic disease

LymphomaUnifying 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

>60yrsInsidious painless mass / proptosisPredilection for anterosuperior orbit and lacrimal glandLook for smooth salmon patch in superior fornix (BRLH often nodular)

LymphomaDIFFERENTIAL DIAGNOSISChronic pseudotumourDermoidMixed adenoma of lacrimal gland

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

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

TREATMENT1. observation: BRLH2. systemic steroids: BRLH3. radiotherapy: localised lesion, 2000rads4. chemotherapy: if systemic disease, use DXT to orbital lesion if there is a poor response to chemotherapy5. surgical excision / debulking

PROGNOSISVisual prognosis good unless radiation retinopathyAll patients with localised lesions need continual review for development of systemic disease

CAVERNOUS HAEMANGIOMA

commonest benign orbital neoplasm in adultsPATHOLOGYDilated vascular lakes lined with endothelial cellsUsually intraconal causing progressive, painless, axial proptosisMay enlarge due to bleeding, pregnancyINVESTIGATIONB scan - high internal reflectivityCT scan - round intraconal massDIFFERENTIAL DIAGNOSISHaemangiopericytoma, neurolemmoma, meningioma, glioma.

TREATMENTIndications: pain, loss of vision, diplopia, proptosis, histological diagnosis

Excision via lateral orbitotomy.

LYMPHANGIOMA

children and young adultsInvolve 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.

INVESTGATIONThey 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.

PROGNOSISNo 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 HAEMANGIOMATREATMENTIndicated only if amblyogenic or causing a systemic problemSteroids: 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, cryotherapyInterferon 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 VaricesReally indistinguishable from a lymphangioma. Congenital but may present acutely due to internal haemorrhage

High Flow VaricesExpand with jugular vein pressure.May have an intracranial communication. These ones rarely bleed!TREATMENTObserve, 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.

TREATMENTSurgical 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

CLINICALArteriolisation of conjunctival vessels marked engorged chemosis, proptosisOphthalmoplegia, 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)

INVESTIGATIONCT scan- enlarged muscles / superior ophthalmic vein / proptosis.Angiography.

TREATMENTSpontaneous recovery, embolisationElectrocoagulation sinusIntracavernous repairLigation 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 children

M>F, average age 8yrs, may be familialPATHOLOGYComposed of primitive malignant mesenchymal cells in varying stages of embryogenesisEMBRYONAL: commonest, paucity of cross striationsALVEOLAR: tumour cell cytoplasm forms alveolar type structures, most malignantPLEOMORPHIC: adults, more differentiated, cross striations prominent, best prognosis

RHABDOMYOSARCOMA

CLINICALSudden, rapidly progressive proptosisRedness and oedema, pain is late feature

INVESTIGATIONCT scan

Biopsy - formalin for LM and glutaraldehyde for electron microscopyCXR, bone marrow and LP

TREATMENTLocal irradiation (4500- 6000 rads) and chemotherapy

PROGNOSIS90% survival if confined to orbit, poor prognosis if bony involvementOverall survival = 65%

BiopsyThe 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 INFLAMMATIONLYMPHOPROLIFERATIVE 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.

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

CLINICAL30-50yr Unilateral (bilateral rarely)M=FPainful proptosis progressing over a few monthsConjunctival injection and chemosis over the recti muscles is common.Myositis commonly affects IR (cf. thyroid)

+/- CNV involvement, meningeal irritationPrednisone 1mg/kg/day for 2-4 weeks, then taperRadiotherapy 1500rads

PSEUDOTUMOURDIFFERENTIAL DIAGNOSIS

Thyroid eye disease, tumour, orbital cellulitis, systemic vasculitis (SLE, PAN), lymphoma, lacrimal gland carcinomaINVESTIGATION1. B scan:- echo free zone posterior to sclera due to oedema in Tenons capsule, EOM enlargement, discrete mass2. 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 BCCsXeroderma 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.

MANAGEMENTExcision: 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.

HISTOLOLOGYSquamous 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.TREATMENTAim 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.

HISTOLOLOGYLarge 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.MANAGEMENTComplete 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 GROUPSOcular 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 2YPHELPLINE: 01761-411 055