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Common primary benign tumors of the orbit in children.
May present at birth or in first few weeks after birth, enlarges dramatically
over 6-12 months of life, and involuting after first year, 75% resolve during
first 4-5 years.
Risk : premature babies & mothers who had undergone chronic villous
Superficial appear as bright red, soft mass with dimpled texture or
Subcutaneous & bluish in color.
Always be aware of rapidly growing mass s/o malignancy –
MRI may be used to help distinguish CH & lie in superonasal quadrant of orbit
& medial upper eyelid.
Associated with hemangiomas on other parts of the body.
Lesions that involve neck can compromise the airway & lead to respiratory
obstruction, and multiple large visceral lesions can produce
thrombocytopenia. (Kasabach-Merritt syndrome).
Main ocular complications :
amblyopia, strabismus, and
Most lesions regress spontaneously ,
therefore, observation, refractive
correction, and amblyopia therapy
are the first line of management.
Treatment consists of steroids,
administered either topically, by
local injection, or orally.
Adverse effects : necrosis of the skin,
S/C fat atrophy, systemic growth
retardation, risk of orbital hmrg &
retinal embolic visual loss.
Recent studies, β-blockers (propranolol & topical timolol gel), careful with
combination with steroids.
Surgical excision with meticulous hemostasis.
Radiation therapy : cataract, bony hypoplasia & future malignancy.
Pulse-dye laser for superficial components of hemangioma
Most common benign neoplasm of the
orbit in adults.
W > M
Slowly progressive proptosis, growth
accelerates during pregnancy.
Other : retinal striae, hyperopia, ON
compression, Increased IOP,
Orbital imaging : homogenously
enhancing, well-encapsulated mass.
MRI : small intralesional vascular
channels containing slowly filling
Chronic lesions : Phleboliths.
Histologically, lesions are encapsulated & are composed of large cavernous
spaces containing RBCs, walls contain smooth muscle.
Surgical excision if the lesion compromises ocular function.
Approach for surgery is dictated by location of lesion.
Coronal imaging is important to find relation with ON.
Differentials : Hemangiopericytoma
Varices – Combined venous-lymphatic
Primary varices consist of a plexus
of thin walled distensible low-flow
vein like vessels that are commonly
intrinsic to the normal circulation.
Varices of lid & conjunctiva.
Present at anytime from early
childhood to late middle age.
Occurs secondarily to a local high
flow vascular lesion or trauma.
Varices - Diagnosis
Usually unilateral and the most
Intermittent non-pulsatile proptosis
without a bruit.
Elicitated by increasing venous
pressure through coughing or Valsalva
Imaging shows lobulated mass with
variable contrast enhancement , amy
demonstrate phleboliths &
sometimes orbital expansion or
associated orbital wall defect
Complication : acute orbital
hemorrhage, thrombosis, atrophy of
surrounding fat, giving enophthalmos
with a deepened
Varices - Treatment
Small lesions doesn’t require treatment.
Surgical excision is technically difficult as they are friable & bleed easily.
Embolisation & C02 laser surgery may be helpful.
Indication for surgery : pain , severe proptosis & ON compression.
Represents vascular dysgenesis.
Occurs as disruption of the initially
pluripotent vascular analage, which
leads to aberrant developmene &
Contain both venous & lymphatic
Enlarge during URTI., sudden
proptosis caused by spontaneous
Histologically, large, serum-filled channels lined by flat endothelial cells that
have immunostaining pattern consistent with lymphatic capillaries.
Some of them are localised, slowly progressive, others may diffusely infiltrate
orbital structures & enlarge.
MRI : Pathognomic : Multiple grapelike cystic lesions with fluid layering of the
serum & RBCs.
Lymphatic malformations - Management
Surgical intervention is deferred unless any affect in vision.
Subtotal resection to avoid sacrifice of important structures.
Intralesional sclerosing agents
Orbital hmrg should be allowed to resorb spontaneously, but should be
cautious in ON or CU : hollow bore needle, open surgical exploration.
Low flow vascular lesions from vascular dysgenesis.
May exhibit enophthalmos at rest.
Proptosis increases when head is dependent or after a Valsalva maneuver s/o
Diagnosis : contrast enhanced rapid spiral CT during valsalva shows
characteristic enlargement of the engorged veins.
Treatment : conservative, avoid biopsy.
Surgery : vision-threatening compressive ON.
High-flow developmental anomalies resulting from vascular dysgenesis.
Composed of abnormally formed anastomosing arteries & veins without an
intervening capillary bed.
Dilated corkscrew episcleral vessels may be prominent.
Selective occlusion of the feeding vessels, followed by surgical excision of
Acquired lesions caused by abnormal direct communication between an artery
& a vein .
Blood flows directly from artery to vein without passing through an
intervening capillary bed.
Cause : Trauma or degeneration.
2 forms : Carotid cavernous fistula – basal skull #.
Dural cavernous fistula – degenerative process in older patients.
Carotid cavernous fistula
High blood flow rate
Characteristic tortous epibulbar vessels & a bruit may be audible
Pulsatile proptosis present
Ischaemic ocular damage : diversion of arterialised blood into venous system,
which causes venous outflow obstruction.
Results in elevated IOP, choroidal effusions, blood in Schlemm canal,
Increased pressure in Cavernous sinus : compression of 3,4,6 CN associated
with EOM palsy.
Dural cavernous fistula
Occurs when small meningeal arterial branches communicates with venous
Dural fistulas produce less blood flow than carotid cavernous fistula.
Their onset can be insidious with only mild orbital congestion, proptosis,&
Arterialisation of the conjunctival veins chronic red eye.
Asymmetric IOP elevation increased episcleral venous pressure on ipsilateral
Dural cavernous fistula
CT shows diffuse enlargement of all EOM resulting from venous engorgement
& characteristically enlarged SOV.
Embolisation using coils to obstruct the fistula is generally accomplished
through an endovascular transarterial route.
Optic nerve glioma
Usually benign, tumors that occur predominantly in children in 1st decade of
Malignant ON glioma are very rare & occur in adult male.
Malignant ONG : severe retro-orbital pain, U/L or B/L visual loss & typically
massive swelling & hemorrhage of ON head .
Despite high dose radiotherapy & chemotherapy, these tumors usually result
in death within 6-12 months.
Optic nerve glioma
Upto half of ON gliomas are associated with neurofibromatosis.
C/F : gradual, painless, U/L axial proptosis associated with loss of vision & an
afferent pupillary defect.
Other : OA, OD swelling, nystagmus & strabismus.
Chiasm is involved in half the cases.
Optic nerve glioma – Pathological
Grossly : smooth, fusiform intradural lesion.
Microscopically benign tumors are considered juvenile pilocytic (hairlike)
astrocytomas, arachnoid hyperplasia, muco substance & Rosenthal fibers.
ONG in patient with NF often proliferate in the subarachnoid space.
Optic nerve glioma – Diagnosis
By means of orbital imaging.
CT & MRI shows fusiform enlargement of the ON, with stereotypical kinking of
MRI : cystic degeneration, more accurate in defining the lesion.
Optic nerve glioma - Management
Most cases remain stable, some behaves aggressively.
1. Observation only
2. Surgical excision
3. Radiation therapy
Optic nerve glioma – observation
Radiological evidence confines tumor within the orbit.
F/U with MRI
Optic nerve glioma – Surgical excision
Rapid Intraorbital tumor growth.
Effort to prevent chaismal invasion.
Use intracranial approach to obtain tumor free surgical margin.
Complete excision is possible if the tumor ends 2-3 mm anterior to the
Optic nerve glioma – Radiation therapy
Sole treatment if the tumor cant be resected & if the symptoms progress.
Postoperative radiation of the chiasma & optic tract , if the involvement is
Last resort : debilitating side effects : mental retardation, growth
retardation, & secondary tumors within the radiation field.
Optic nerve glioma-Chemotherapy
Combination chemotherapy : actinomycin D, vincristine, etoposide are
effective with progressive chiasmal/hypothalamic gliomas.
May delay the need for radiation therapy & thus enhance long-term
intellectual development & preservation of endocrinal function in children.
Chemotherapy carry risk of blood-borne cancer.
Careful individualisation, decision should be made on tumor growth
characteristics, extent of ON & chaismal involvement
Clinical, radiological evaluation , the VA of the involved & uninvolved
eye,presence or absence of concomitant neurological or systemic disease
Tumors composed chiefly of proliferating Schwann cells within the nerve
Axons, endoneural fibroblasts, and mucin are noted histologically.
Consist of diffuse proliferation of Schwann cells within nerve sheaths, usually
occur in NF-1.
Well vascularised & infiltrative lesions, making surgical excision difficult.
Less common than plexiform
Usually can be excised without recurrence.
Surgery is limited to tumors that compromise vision or produce disfigurement.
Aka von Reckinghausen disease
Characterised by presence of hamartomas involving the skin, eye, CNS, &
viscera classified as phakomatosis.
NF1 is most common phakomatous disorder.
Plexiform neurofibromas involving the lateral aspect of the upper eyelid & S-
shaped contour of the lid margin, pulsating proptosis secondary to sphenoid
bone dysplasia, & ON glioma.
Invasive tumor that arise from the arachnoid villi
Originate intracranially along the sphenoid wing with secondary extension
into the orbit through the bone, the superior orbital fissure, or the optic
canal, or may arise primarily in the optic nerve.
Meningioma – Ophthalmic manifestation
Arising near the sella & ON cause early visual field defects & papilloedema or
Arising near the pterion , often produce a temporal fossa mass& may be
associated with proptosis/non-axial, eyelid edema,& chemosis.
Sphenoid wing meningiomas : hyperostosis of the involved bone & hyperplasia
of associated soft tissues. Presence of dural tail , differentiates from fibrous
Meningioma –primary orbital
Originate in the arachnoid of the ON sheath.
Occurs commonly in women
3rd & 4th decade
Gradual, painless, unilateral loss of vision
Decreased VA & RAPD
ONH : Normal, atrophic,or swollen; and a optociliary shunt vessels may be
ON sheath meningiomas are associated with neurofibromatosis
Meningioma – Imaging
Both CT & MRI show diffuse tubular
enlargement of the ON with
CT may show calicification within
the meningioma, tram tracking.
MRI reveals a fine pattern of
enhancing striations from the
lesion in a longitudinal fashion,
which represents the infiltrative
MRI of sphenoid wing meningiomas
show dural extension into the
chiasm & the intrac
Sphenoid wing meningiomas are typically observed until they cause profound
proptosis, Compressive ON, motility impairment,or cerebral edema.
Subtotal resection of a tumour (intracranial & orbital approach).
Goal of surgery : reverse the volume-induced compressive effects of the
Post operative radiotherapy is advocated.
ON sheath meningiomas should be individualised & should be minimally
consideration for surgery.
Factors : extent of visual loss & the presence of intracranial extension.
Fractionated stereotactic Radiation therapy results in stabilisation or
improvement of visual function.
Surgical excision may lead to irreversible visual loss d/t compromisation of
the ON blood supply.
In cases, with profound proptosis & severe visual loss, ON is excised with the
tumor, from back of globe to the chiasma.
Proliferations of schwann cells that are encapsulated by perineurium.
Characteristic biphasic pattern of solid areas with nuclear palisading (Antoni
A) & myxoid areas (Antoni B).
Hypercellular schwannomas has potency to recur & seldom undergo malignant
Well encapsulated & can be excised with relative ease.
Most common primary orbital malignancy tumor of childhood.
Onset : 8-10 years
Classic clinical picture : sudden onset & rapid progression of U/L proptosis.
Early teens : gradual progressive proptosis lasting from weeks to more than a
May also be present with ptosis & strabismus.
Mass present in SN quadrant of orbit, rarely from conjunctiva.
Workup should be done on an urgent basis.
CT & MRI to define location & extent of the tumor.
Biopsy : anterior orbitotomy
Possible to remove rhabdomyosarcoma if it has a pseudocapsule.
In smaller volume , chemotherapy & radiotherapy is more effective.
Large biopsy specimen is taken in diffusely infiltrating tumors.
Permanent light-microscopy, electron microscopy, and immunohistochemistry
are important investigation .
Palapate : cervical & preauricular LN to rule out regional metastasis.
Chest radiograph, bone marrow aspirate & biopsy & LP is obtained for distant
Arise from undifferentiated pluripotential mesenchymal elements in the
orbital soft tissues & not from the EOM.
Grouped into 4 categories :
Rhabdomyosarcoma – Embryonal
Most common type
More than 80%
SN quadrant has predeliction
Tumor is composed of loose fascicles of undifferentiated spindle cells, only a
minority show cross-striations in immature rhabdomyosarcoma on trichrome
Rhabdomyosarcoma - Alveolar
Predilection for inferior orbit
Accounts for 9%
Regular compartments composed of fibrovascular strands in which rounded
rhabdomyoblasts either line up along the connective tissue strands or float
freely in alveolar space.
Most malignant form, 10 year survival rate is 10 %.
Rhabdomyosarcoma - Pleomorphic
Least common & most differentiated form.
Many of the cells are straplike or rounded, and cross-striations are easily
visualised with trichrome stain.
Rhabdomyosarcoma - Botyroid
Rare variant of embryonal rhabdomyosarcoma appears grapelike
Occurs as secondary invader from the PNS or from the conjunctiva.
Rhabdomyosarcoma - Management
Before 1965, the standard treatment was orbital exenteration, and the
survival rate was poor.
Intergroup Rhabdomyosarcoma studies 1-4
Since 1965, radiation & systemic chemotherapy has become the mainstay of
primary treatment . Chemotherapy is to eliminate molecular cellular
Exenteration is reserved for recurrent cases.
Dose of radiation : 4500 to 6000 cGy, given over 6 weeks period.
Survival rate : 90% if tumor has not invaded beyond the bony orbital walls.
Adv. Effects : cataract, radiation dermatitis , & bony hypoplasia in case of
incomplete bony development.
Miscellaneous Mesenchymal tumors
Tumors of fibrous connective tissue, cartilage, and bone are uncommon
lesions that may invade the orbit.
Firm & displaces normal structures
Both fibroblastic & histiocytic cells in storiform (matlike) pattern are found.
Less than 10% have metastatic potential.
Recently described solitary fibrous tumor is composed of spindle shaped cells,
with CD 34 positive.
Benign developmental disorder of the bone that may involve a single region or
CT : hyperostotic bone, MRI : lack of dural enhancemens
Association with cutaneous pigmentation & endocrine disorder : Albright
Treatment : resection or debulking if there’s disfigurement or visual loss d/t
stricture of Optic canal.
Benign tumor that can involve any of paraorbital sinuses.
CT : hyperostosis with well defined margins
Lesions produce proptosis, CON, & orbital cellulitis secondary to obstructive
Symptomatic lesion requires complete excision.
Malignant mesenchymal tumors
Rarely appear in the orbit.
When chondrosarcoma & osteosarcomas are present, they usually destroy
normal bone & have calcifications in radiographs & CTs.
B/l retinoblastomas have high risk of osteosarcoma, chondrosarcoma ,or a
Lymphoid hyperplasia & lymphoma : lymphoproliferative lesions of ocular
adnexa consist a heteregenous group of neoplasms that are defined by
clinical, histologic, immunologic, molecular, and genetic characteristics.
Most orbital lymphoproliferative lesions are non-Hodkin lymphomas.
Incidence of NHL : 4TH Most common malignancy.
Hisk risk : bioactive solvent exposure & reagents, old age , chronic
Identification & classification of
REAL (Revised European American Lymphoma)
1. Mucosa associated lymphoid tissue (MALT)
2. Chronic lymphocytic lymphoma : low-grade lesion of small, mature appearing
3. Follicular center lymphoma : low grade lesion with follicular centers
4. High grade lymphomas : large cell lymphoma, lymphoblastic lymphoma &
Mucosa associated lymphoid tissue
Accounts for 40-60% of orbital lymphomas.
Originally occurring in GI, have approximately 50% of MALT .
Evidence suggest that MALT might be Ag – H.pylori
Conjunctival MALT lymphomas : chronic chlamydial infxn
Low grade malignancy
Long term follow up to observe for systemic disease involvement.
May also have histological transformation to a higher grade lesion, usually of
Gradually progressive, painless mass
Located anteriorly in the orbit or beneath the conjunctiva ; show salmon-
Benign or malignant
Usually mold to surrounding structure rather than invade them
Reactive lymphoid hyperplasia & low-grade lymphomas : H/o slow expansion
over a period of months to years.
orbital Imaging : characteristic Puttylike moulding of the tumor to normal
structures, Bone erosion is seen with high-grade malignant lymphomas.
50% occur in lacrimal fossa, 17% are bilateral.
For all lymphoproliferative lesion, an open biopsy is preferred.
To establish a diagnosis & to characterise the lesions morphologic,
immunologic, cytogenic, and molecular properties under REAL classification.
Reactive hyperplasia & malignant lymphoma are hypercellular proliferations
wtih sparse or absent stromal components.
Malignant lymphomas represent clonal expansions of abnormal precursor cells
Immunogenic identification of cell marker on lymphocytes : B/T cells as being
Specific monoclonal Ab directed against surface light chain (κ or λ) are used
to study for determination of cells that represent monoclonal (malignant)
Newer techniques of molecular analysis : DNA hybridisation
Approx. 90% prove monoclonal & 10% polyclonal by molecular genetic studies.
Both types have prior, concurrent or systemic spread.
Risk of systemic involvement : conjunctival > orbital > eyelid
Lymphoid lesion in lacrimal fossa carry a greater risk of systemic disease than
B/L periocular involvement : increases risk of systemic disease but not
Examination by an oncologist.
General physical examination, CBC, BM biopsy, a liver & spleen scan, chest
radiograph, serum immunoprotein electrophoresis.
CT : Thorax & Abdomen : mediastinal/retroperitoneal LN involvement .
Steroid : useful in NSOI but not in lymphoproliferative lesions.
Radiotherapy : treatment of choice.
2000-3000 cGy is typically administered.
Achieves local control in virtually all cases, if lesion is isolated , may prevent
D/t invasive nature : surgical cure cant be achieved.
Aggressive lymphomas : radiation, aggressive chemotherapy or both .1/3rd of
lesion can be cured.
Composed of predominantly of mature plasma cells may be plasmacytomas or
localised plasma rich pseudotumors.
Rule out : MM if there is bone destruction or any mitotic activity among the
Composed of lymphocytes or lymphoplasmocytes.
Shows similar spectrum but are less common.
Langerhans cell histiocytosis / Histiocytosis X
Rare disorder of mononuclear phagocytic system
Results from abnormal immune regulation
Characterised by accumulation of dendritic histiocytes.
Children : 5-10 years
Varies from benign lesion to chronic dissemination resulting in dea
Histiocytic Disorders - Presentation
Superotemporal orbit, sphenoid wing is affected
Relapsing episode : orbital inflammation misinterpreted as orbital cellulitis
Mass may cause proptosis
Young children present with significant overlying soft tissue , have likeliness
of multifocal or systemic involvement.
Treatment of localised orbital disease : debulking followed by intralesional
steroid injectionor low dose radiotherapy
Systemic disease are treated aggressively with chemotherapy.
Often associated with systemic manifestations
classified as 4 syndromes :
1. Necrobiotic xanthogranuloma (NBX)
2. Adult-onset asthma with periocular xanthogranuloma (AAPOX)
3. Erdheim Chester Disease (ECD)
4. Adult onset xanthogranuloma (AOX)
Necrobiotic xanthogranuloma (NBX)
char. by presence of S/C lesions in the eyelids & Ant. Orbit ; the lesions may
occur through out the body
Lesion have propensity to ulcerate & fibrose
Systemic findings : paraproteinemia & MM.
Adult-onset asthma with periocular
Periocular xanthogranuloma, asthma, lymphadenopathy, and often increased
Erdheim Chester Disease (ECD)
Dense, progressive, recalcitrant fibrosclerosis of the orbit & internal organs,
including mediastinum , pericardium ; and the pleural, perinephric, and the
Xanthogranuloma of ECD is diffuse , may lead to visual loss.
Bone involvement & death is higher.
Adult onset xanthogranuloma (AOX)
Isolated xanthogranulomatous lesion without systemic involvement
Juvenile xanthogranuloma : separate non-Langerhans histiocytic disorder ,
self-limited, corticosteroid sensitive, usually focal S/C disease of childhood.
Most common epithelial tumor of LG.
Occurs during 4th – 5th decade.
Presents with progressive, painless downward & inward displacement of the
globe with axial proptosis.
A firm, lobular mass may be palpated –SL orbital rim
Imaging shows expansion of lacrimal fossa.
Lesions appears well circumscribed but may have nodular configuration.
Benign mixed tumors have a varied cellular structure consisting of a
proliferation of benign epithelial cells & stroma composed of spindle shaped
cells with occasional cartilaginous, mucinous, or even osteoid degeneration or
metaplasia. Lesion circumscribed by pseudocapsule.
Treatment : complete removal along with its pseudocapsule & a surrounding
margin of orbital tissue.
Surgery performed with preliminary biopsy.
Adenoid cystic carcinoma (cylindroma)
Most common malignant tumor of LG .
Causes pain : perineural invasion & bone destruction
Rapid course , less than 1 year
extends upto posterior orbit because of its capacity to infiltrate & lack of
Histologically, made of benign appearing cells that grow in tubules , solid
nests,, or cribriform Swiss –Cheese pattern.
Basaloid has worst prognosis than cribriform.
Management of malignant LG tumors
Suspicion warrants with biopsy with permanent histological confirmation.
Exenteration & radical orbitectomy with removal of the roof, lateral wall, &
floor along with overlyinf soft tissues & anterior portion of temporalis muscle
High dose radiation along with debulking , taken as alternative.
Despite this measure, there is perineural extension into the cavernous sinus .
Typical : multiple painful recurrences ultimately ending up in mortality from
Nonepithelial tumors of the LG
Most of them represent lymphoid proliferation or inflammations.
Up to 50% occur in LG.
May also occur in Sjögren or a localised lacrimal/salivary gland (Mikulicz
Usually B/L , female , c/o dry eye
Biopsy : spectrum of lymphocytic infiltration.
May have association with RA , may also have low-grade B-cell lymphoma.
Tumors from the nose or the PNS may secondarily invade the orbit.
Proptosis & globe displacement are common.
Diagnosis is made by imaging & must be carried upto the base of sinus.
Mucoceles & mucopyoceles
Silent sinus syndrome
SCC : most common pwithelial tumor invading orbit secondarily, arise within
the maxillary sinuses, followed by NP/OP.
Metastatic orbital neuroblastoma produces abrupt ecchymotic proptosis that
may be B/l .
May also have Horner syndrome
Bone destruction is apparent, particularly in the lateral orbital wall or
Occur late in the course of the disease , primary can be detected in the
abdomen, mediastinum, or neck.
Treatment : primarily chemotherapy, radiotherapy is reserved .
Congenital neuroblastoma of the cervival ganglia : ipsilateral Horner
syndrome with heterochromia.
May produce U/L or B/L proptosis
Acute lymphoblastic leukemia : most likely to metastasize to the orbit.
Primary leukemic mass/granulocytic sarcoma/chloroma : Rare variant of
Present with sudden visual loss & swelling of ON
Orbital lesions present in advance of bone or marrow signs
Leder stain (cytoplasmic stain) – indicate granulocytic precursor cells
Survival is improved, if chemotherapy is instituted before leukemic
involevement in BM or peripheral blood.
Virtually any carcinomas can metastize to orbit.
Breast & lung tumors account for the majority.
Presence of pain, proptosis, inflammation, bone destruction, and early
ophthalmoplegia s/o metastatic carcinoma.
75% have known primary tumor, 25% have orbital metastasis.
Eom frequently involved d/t abundant blood supply.
2nd most common : BM space of sphenoid bone : high volume of low flow
blood, lytic destruction of lateral wall : highly suggestive.
Elevated CEA levels
Fine needle biopsy.
Most commonest primary source of orbital metastases in women.
May occur many year after tumor removal.
May elicit a fibrous response that causes enophthalmos & restriction of ocular
Some responds well to hormonal therapy.
Fresh tissue for estrogen- receptor assay if metastatic breast cancer is found
Hormone therapy responds well for receptor positive.
Most frequent origin in men.
Primary lesion may be small, CT lung should be performed if in suspicion.
Management of orbital metastases.
Local radiation therapy.
Wide excision of orbital lesion : carcinoids & RCC : better survival.