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Orbital neoplasms & malformations

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For those willing for knowing more about orbital neoplasms.
Classifications hasn't been mentioned much in this slide.

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Orbital neoplasms & malformations

  1. 1. ORBITAL NEOPLASMS & MALFORMATIONS BIPIN BISTA RESIDENT OPHTHALMOLOGY
  2. 2. Vascular Tumors, Malformations, & Fistula
  3. 3. Capillary Hemangiomas  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 sampling.  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 – Rhabdomyosarcoma.
  4. 4. Capillary Hemangiomas  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).
  5. 5. Capillary Hemangiomas  Main ocular complications : amblyopia, strabismus, and anisometropia.  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.  .
  6. 6. Capillary Hemangiomas  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
  7. 7. Cavernous 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, strabismus.  Orbital imaging : homogenously enhancing, well-encapsulated mass.  MRI : small intralesional vascular channels containing slowly filling blood.  Chronic lesions : Phleboliths.
  8. 8. Cavernous hemangioma  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
  9. 9. Varices – Combined venous-lymphatic malformations  Primary varices consist of a plexus of thin walled distensible low-flow vein like vessels that are commonly intrinsic to the normal circulation.  Probably hamartomatous  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.
  10. 10. Varices - Diagnosis  Usually unilateral and the most upper nasal.  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
  11. 11. 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.
  12. 12. Lymphatic malformations  K/a lymphangiomas.  Represents vascular dysgenesis.  Occurs as disruption of the initially pluripotent vascular analage, which leads to aberrant developmene & congenital malformations.  First decade  Contain both venous & lymphatic components.  Enlarge during URTI., sudden proptosis caused by spontaneous intralesional haemorrhage.
  13. 13. Lymphatic malformations  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.
  14. 14. 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.
  15. 15. Venous malformation  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 valsalva maneuver.  Diagnosis : contrast enhanced rapid spiral CT during valsalva shows characteristic enlargement of the engorged veins.  Treatment : conservative, avoid biopsy.  Surgery : vision-threatening compressive ON.
  16. 16. Arteriovenous Malformations  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 malformations.
  17. 17. AV fistula  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.
  18. 18. 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, nongranulomatous iritis  Increased pressure in Cavernous sinus : compression of 3,4,6 CN associated with EOM palsy.
  19. 19. Dural cavernous fistula  Occurs when small meningeal arterial branches communicates with venous drainage.  Dural fistulas produce less blood flow than carotid cavernous fistula.  Their onset can be insidious with only mild orbital congestion, proptosis,& pain.  Arterialisation of the conjunctival veins chronic red eye.  Asymmetric IOP elevation increased episcleral venous pressure on ipsilateral side.
  20. 20. Dural cavernous fistula  CT shows diffuse enlargement of all EOM resulting from venous engorgement & characteristically enlarged SOV.  Selective arteriography  Embolisation using coils to obstruct the fistula is generally accomplished through an endovascular transarterial route.
  21. 21. Neural tumors  ON gliomas  Neurofibromas  Meningiomas  Schwannomas
  22. 22. Optic nerve glioma  Uncommon  Usually benign, tumors that occur predominantly in children in 1st decade of life  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.
  23. 23. 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.
  24. 24. Optic nerve glioma – Pathological feature  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.
  25. 25. Optic nerve glioma – Diagnosis  By means of orbital imaging.  CT & MRI shows fusiform enlargement of the ON, with stereotypical kinking of the nerve.  MRI : cystic degeneration, more accurate in defining the lesion.
  26. 26. Optic nerve glioma - Management  Controversial  Most cases remain stable, some behaves aggressively. 1. Observation only 2. Surgical excision 3. Radiation therapy 4. Chemotherapy
  27. 27. Optic nerve glioma – observation  Good vision  Radiological evidence confines tumor within the orbit.  F/U with MRI
  28. 28. Optic nerve glioma – Surgical excision  Rapid Intraorbital tumor growth.  Corneal exposure.  Compromised cosmesis.  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 chaisma.
  29. 29. 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 extensive.  Last resort : debilitating side effects : mental retardation, growth retardation, & secondary tumors within the radiation field.
  30. 30. 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
  31. 31. Neurofibroma  Tumors composed chiefly of proliferating Schwann cells within the nerve sheaths .  Axons, endoneural fibroblasts, and mucin are noted histologically.  Plexiform neurofibromas  Discrete neurofibromas
  32. 32. Plexiform neurofibromas  Consist of diffuse proliferation of Schwann cells within nerve sheaths, usually occur in NF-1.  Well vascularised & infiltrative lesions, making surgical excision difficult.
  33. 33. Discrete neurofibromas  Less common than plexiform  Usually can be excised without recurrence.  Surgery is limited to tumors that compromise vision or produce disfigurement.
  34. 34. Neurofibromatosis 1  Aka von Reckinghausen disease  AD pattern  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.
  35. 35. Meningioma  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.
  36. 36. Meningioma – Ophthalmic manifestation  Arising near the sella & ON cause early visual field defects & papilloedema or OA.  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 dysplasia.
  37. 37. Meningioma –primary orbital meningiomas  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 present.  ON sheath meningiomas are associated with neurofibromatosis
  38. 38. Meningioma – Imaging  Both CT & MRI show diffuse tubular enlargement of the ON with contrast enhancement.  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 nature .  MRI of sphenoid wing meningiomas show dural extension into the chiasm & the intrac
  39. 39. Meningioma-Management  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 lesion.  Post operative radiotherapy is advocated.
  40. 40. Meningioma-Management  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.
  41. 41. Schwannoma  A/k/a neurilemomas  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 transformation.  Well encapsulated & can be excised with relative ease.
  42. 42. Mesenchymal tumors
  43. 43. Rhabdomyosarcoma  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 month.  May also be present with ptosis & strabismus.  Mass present in SN quadrant of orbit, rarely from conjunctiva.
  44. 44. Rhabdomyosarcoma  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.
  45. 45. Rhabdomyosarcoma  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 metastases.
  46. 46. Rhabdomyosarcoma  Arise from undifferentiated pluripotential mesenchymal elements in the orbital soft tissues & not from the EOM.  Grouped into 4 categories : 1. Embryonal 2. Alveolar 3. Pleomorphic 4. Botyroid
  47. 47. 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 straining.
  48. 48. 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 %.
  49. 49. Rhabdomyosarcoma - Pleomorphic  Least common & most differentiated form.  Many of the cells are straplike or rounded, and cross-striations are easily visualised with trichrome stain.  Best prognosis.
  50. 50. Rhabdomyosarcoma - Botyroid  Rare variant of embryonal rhabdomyosarcoma appears grapelike  Occurs as secondary invader from the PNS or from the conjunctiva.
  51. 51. 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 metastases.  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.
  52. 52. Miscellaneous Mesenchymal tumors  Tumors of fibrous connective tissue, cartilage, and bone are uncommon lesions that may invade the orbit.  Fibrous dysplasia  Fibrous histiocytoma  Osteoma  osteosarcoma  Liposarcoma  fibrosarcoma  chondrosarcoma
  53. 53. Fibrous histiocytoma  Most common  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.
  54. 54. Fibrous dysplasia  Benign developmental disorder of the bone that may involve a single region or polyostotic.  CT : hyperostotic bone, MRI : lack of dural enhancemens  Association with cutaneous pigmentation & endocrine disorder : Albright syndrome.  Treatment : resection or debulking if there’s disfigurement or visual loss d/t stricture of Optic canal.
  55. 55. Osteomas  Benign tumor that can involve any of paraorbital sinuses.  CT : hyperostosis with well defined margins  Lesions produce proptosis, CON, & orbital cellulitis secondary to obstructive sinusitis.  Usually asymptomatic  Symptomatic lesion requires complete excision.
  56. 56. 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 fibrosarcoma.
  57. 57. Lymphoproliferative Disorders  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 autoimmune disorders.
  58. 58. Identification & classification of lymphoproliferative disorders.  REAL (Revised European American Lymphoma) 1. Mucosa associated lymphoid tissue (MALT) 2. Chronic lymphocytic lymphoma : low-grade lesion of small, mature appearing lymphocytes. 3. Follicular center lymphoma : low grade lesion with follicular centers 4. High grade lymphomas : large cell lymphoma, lymphoblastic lymphoma & Burkitt lymphoma
  59. 59. Mucosa associated lymphoid tissue (MALT)  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 large type.
  60. 60. Clinical Presentation  Gradually progressive, painless mass  Located anteriorly in the orbit or beneath the conjunctiva ; show salmon- patch appearance  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.
  61. 61. Diagnosis  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 either monoclonal/polyclonal  Specific monoclonal Ab directed against surface light chain (κ or λ) are used to study for determination of cells that represent monoclonal (malignant) proliferations.
  62. 62. Diagnosis  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 occurring elsewhere.  B/L periocular involvement : increases risk of systemic disease but not definitive.
  63. 63. Management  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 systemic spread.  D/t invasive nature : surgical cure cant be achieved.  Aggressive lymphomas : radiation, aggressive chemotherapy or both .1/3rd of lesion can be cured.
  64. 64. Plasma Cell Tumors
  65. 65. Introduction  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 plasmacytic elements.  Composed of lymphocytes or lymphoplasmocytes.  Shows similar spectrum but are less common.
  66. 66. Histiocytic Disorders  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
  67. 67. Histiocytic Disorders - Presentation  Lytic defect  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.
  68. 68. Xanthogranuloma  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)
  69. 69. 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.
  70. 70. Adult-onset asthma with periocular xanthogranuloma (AAPOX)  Periocular xanthogranuloma, asthma, lymphadenopathy, and often increased IgG levels.
  71. 71. Erdheim Chester Disease (ECD)  Dense, progressive, recalcitrant fibrosclerosis of the orbit & internal organs, including mediastinum , pericardium ; and the pleural, perinephric, and the retroperitoneal spaces.  Xanthogranuloma of ECD is diffuse , may lead to visual loss.  Bone involvement & death is higher.
  72. 72. 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.
  73. 73. Lacrimal Gland Tumors
  74. 74. Introduction  Most common : dacryoadenitis  Other LG tumefactions without any inflammatory signs & symptoms  Majority : Lymphoproliferative disorders , 50 % develop in lacrimal fossa  Imaging is helpful  Inflammatory & lymphoid proliferation : expand diffusely & appear elongated, mold around the globe  Epithelial neoplasms : isolated globular masses , indent the globe, bony remodelling occurs
  75. 75. Epithelial tumors of the LG
  76. 76. Pleomorphic adenomas  Most common epithelial tumor of LG.  Occurs during 4th – 5th decade.  M>F  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.
  77. 77. Pleomorphic adenomas  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.
  78. 78. 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 encapsulation  Histologically, made of benign appearing cells that grow in tubules , solid nests,, or cribriform Swiss –Cheese pattern.  Basaloid has worst prognosis than cribriform.
  79. 79. 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 IC extension.
  80. 80. 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 syndrome)  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.
  81. 81. Secondary Orbital Tumors
  82. 82. Globe & Eyelid Origin  Within the eye – Choroidal melanomas , Retinoblastoma  Eyelid – Sebaceous gland carcinoma, SCC, BCC
  83. 83. Sinus Origin  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.
  84. 84. Metastatic tumors
  85. 85. Metastatic Tumors in Children
  86. 86. Neuroblastoma  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 sphenoid marrow  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.
  87. 87. Leukemia  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 myelogenous leukemia.  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.
  88. 88. Metastatic Tumors in Adults
  89. 89.  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.
  90. 90. Breast carcinoma  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 motility.  Some responds well to hormonal therapy.  Fresh tissue for estrogen- receptor assay if metastatic breast cancer is found on exploration.  Hormone therapy responds well for receptor positive.
  91. 91. Bronchogenic carcinoma  Most frequent origin in men.  Primary lesion may be small, CT lung should be performed if in suspicion.
  92. 92. Management of orbital metastases.  Palliative treatment  Local radiation therapy.  Wide excision of orbital lesion : carcinoids & RCC : better survival.

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