Orbital And Peri-Orbital Tumours

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Orbital And Peri-Orbital Tumours
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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.

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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

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Lymphoma
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Lymphoma

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

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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
• gt60yrs
• Insidious painless mass / proptosis
• Predilection for anterosuperior orbit and
  lacrimal gland
• Look for smooth salmon patch in superior fornix
  (BRLH often nodular)

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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

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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.

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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.

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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 syndromePlatelet 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 !

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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

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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.

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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.

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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.

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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

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CAROTICO-CAVERNOUS FISTULA
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CAROTICO-CAVERNOUS FISTULA
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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
  53,4 The average age of presentation is 7.8
  years.

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RHABDOMYOSARCOMA

• The commonest primary malignant orbital tumour in
  children
• MgtF, 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

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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

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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.

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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.

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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)
• MF
• 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

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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

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PSEUDOTUMOUR
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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.

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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.

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BASAL CELL CARCINOMA ( BCC )
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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.

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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.

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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.

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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.

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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