diagnose and management of orbital lymphoma ophthalmology departement 2014

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Diagnose & Management
of Orbital Lymphoma
Ardizal Rahman
OphthalmologyDepartment
Faculty of Medicine Andalas University
DR. M. Djamil Hospital
2014

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MalignantLymphoma
Hodgkin Lymphoma
Non-Hodgkin Lymphoma
Orbital Lymphoma

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LYMPHATIC SYSTEM
 Lymph vessel
 Lymph
 Lymph nodes
 Other part of lymphatic system

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PREDILECTION
 Conjunctiva
 Lacrimal gland
 Nasolacrimalduct
 Eyelid
 Extraocular muscles

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RISK FACTOR
 Age
 Infection
 Immunocompromise
 Exposure to toxic chemicals
 Genetics

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STAGING
 Ann-Arbor staging system:
 Stage I : confined to the orbit
 Stage II : involvement of adjacent structures
 Stage III : nodal abdominal disease below the diaphragm
 Stadium IV : disseminated involvement of one or more extra-nodal
sites

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SYMPTOMSOF LYMPH NODES ENLARGEMENT
 Respiratory distress
 Loss of appetite
 Abdominal pain
 Weight loss
 Swelling of extremities
 Pleural effusion
 Skin thickening
 Anemia
 Susceptible from bacterial
infection

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DIAGNOSIS
Accurate
staging &
treatment
planning
Historytaking
& physical
examination
Laboratory
examination
ChestX-ray, CT
scan & bone
marrowbiopsy

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CT SCAN
 Well-defined lesion, homogeneous & distinct margin border
 Unifocal,isodensity until slight hiperdensity
 Mold to adjacent structure without infiltrates it

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DIFFERENTIAL DIAGNOSIS
Abscess
Pseudotumor
Thyroid
Orbitopathy
LacrimalGland
Tumor
Optic Nerve
Glioma
Metastases

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MANAGEMENT
 Mayor criteria in deciding treatment :
Histopathologic subtype of lymphoma, according to WHO
classification
Disease dissemination, inside & outside periocular area
Prognostic factors thatrelated to the disease & patient
Orbital lymphoma impact on eyes & visualfunction

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MANAGEMENT
 Treatment modalities:
• Radiotherapy
• Chemotherapy
• Surgical
• Immunotherapy
• Antibiotictherapy

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RADIOTHERAPY
 Result in a very high control rate from 86% until100%, & local
recurrence rate between 0% and 15%
 Radiotherapy  30 Gy in low degree lymphoma & 40 Gy in
intermediate degree lymphoma
 Late complication  cataract,xerophthalmia, glaucoma & ischemic
retinopathy

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CHEMOTHERAPY
 Chlorambucil or fludarabine  low degree lymphoma
 Combination chemotherapy : cyclophosphamide, doxorubicin,
vincristine, & prednison (CHOP)  high degree lymphoma
 Elderly patients combination of immunotherapy rituximab
(monoclonal antibody anti-CD20) with chemotherapy
cyclophosphamide, adriamycin, vincristine & prednisolon (R-CHOP)

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SURGICAL EXCISION
 Local recurrence more commonly after simple surgical excision rather
than after radiotherapy
 IFN & Rituximab
 Blasiet al complete respond obtained from allof 5 patients with
conjunctival MALT lymphoma that treated with 1.500.000 IU IFN
 Raderer et al Rituximab induced complete respond in 42%
patients with relapsed MALT lymphoma
IMMUNOTHERAPY

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Anti–C psittaci Antibiotic Therapy
 Ferreri et al  therapy with doxycycline in patients with marginal
zone B-celllymphoma which C psittacipositive  complete
remission in 2 from 9 patients
 Grunberger et al no therapeutic effect in 11 patients with MALT
lymphoma
 Antichlamydia antibiotic therapy cannot be considered as standard
therapy for orbital lymphoma

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PROGNOSIS
 Commonly was good(overall 5 years survival rate  50% - 94%)
 Nonconjunctival primary site, advanceddisease stage, lymph nodes involvement,
age > 60 y.o., presence ofB symptoms, & elevated of serum LDH level  negative
prognostic factor
 Metastases risk :
 Conjunctival primary site  lowest risk (20%)
 Orbital primary site  intermediate risk (35%)
 Eyelid primary site  highest risk (65%)

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