During January 2015 to December 2020 there were 17 cases of orbital lymphoma who went to M. Djamil Hospital Padang, majority of male patients, with a mean range of age 60 years. The most clinical manifestations of orbital lymphoma were proptosis (58.82%) followed by a palpebral mass (41.18%) and most cases were unilateral. All patients were performed orbital CT scan and histopathological examination. Most of patients were non-Hodgin lymphoma with small lymphocytic type which is a low grade lymphoma. There was one patient with a mismatch between clinical manifestations and histopathological results so the histopathological examination was reviewed again. The management performed in this orbital lymphoma patient was chemotherapy in 16 patients and 1 patient refused chemotherapy and performed an anterior orbitotomy.

1. MEIRONI WAIMIR
ONCOLOGY SUBDIVISION
DEPARTMENT OF OPHTHALMOLOGY
MEDICAL FACULTY OF ANDALAS UNIVERSITY
DR. M . DJAMIL HOSPITAL PADANG
2021
Research
THEPROFILEOFORBITALLYMPHOMA
IN M. DJAMIL HOSPITAL PADANG
2015-2020

2. INTRODUCTION
Lymphoma
Neoplastic transformation of cells that reside predominantly within lymphoid tissues.
Orbital lymphoma
A type of lymphoproliferative tumor originating from the ocular adnexa which has a
broad spectrum.

3. Orbital lymphoma Involvement the
periocular sites
Orbital lymphoma
• 50-60% of ocular adnexal
lymphoma.
• 90% of orbital lymphoma being
Non Hodgkin's lymphoma.
• 6-8% of all orbital
tumors.
• 10-15% of adnexal
tumors.
Conjunctiva (20-33%),
orbits (46-74%), and
eyelids (5-20%).
3
INTRODUCTION

4. RESEARCH PURPOSES
Special
Purpose
Characteristics
 age and
gender
Clinical
manifestations
and
lateralization
Histopathological
Management
General purpose:
Knowing the profile of orbital lymphoma patients in M. Djamil Hospital Padang from January 2015
to December 2020.

5. Provide information and
an overview
The profile of orbital lymphoma
patients in M. Djamil Hospital
Padang for the period 2015 to
2020.
Basic data for further
research
Provide information
for future researchers
Can be used as a
comparison material for
further research.
5
BENEFITS OF RESEARCH

6. ETIOLOGY AND PATHOGENESIS
 Lymphoma  lymphoid malignancy originating from the clonal proliferation of B lymphocytes, T
lymphocytes, or natural killer (NK) cells.
 Lymphoma  Hodgkin lymphoma, which originates from B lymphocytes, and Non Hodgkin's
lymphoma originating from different clones.
 The cause of cell mutation in lymphoma is not certain
 Monoclonal theory  neoplastic changes initially affect one cell which then multiplies and gives rise
to neoplasms.
 Environmental theory  related to carcinogenic agents  changes at various stages in the growth
of normal blood cells  neoplastic clones  cause swelling of the lymph nodes.

7. • Late onset and slow progression  over
many years to show clinical symptoms.
• Usually unilateral but can also bilateral.
• 50-70 year age group.
• Affects both genders with a slightly more
predilection for women
CLINICAL MANIFESTATIONS
• The clinical manifestations  non-
specific, depending on the location of
the lymphoma.
• Visual acuity  usually undisturbed,
because infiltration to optic nerve is
rare

8. Proptosis
CLINICAL MANIFESTATIONS
Ptosis Salmon-patch appearance

9. CLASSIFICATION OF LYMPHOMA
In general lymphoma is classified into  Hodgkin's lymphoma and Non-Hodgkin's lymphoma.
 Classification according to Rappaport
• First introduced in 1956 and then modified in
1978.
• Classified based on cytology and histology.
 Working Formulation
• Compiled by the National Cancer Institute in
1982.
Classification Characteristics
Low
- Small lymphocytic
- Follicular,predominantlysmallcleaved cell
- Follicular,mixed,smallcleavedcellandlarge cell
Intermediate
- Follicular,predominantlylarge cell
- Diffuse,mixed,smalldanlarge cell
- Diffuse,largecell(cleavedand noncleaved)
High
- Diffuse large cell, immunoblastic
- Lymphoblastic (convoluted and non-
convoluted)
- Small noncleaved cell

10. CLASSIFICATION OF LYMPHOMA
 The Revised European American Classification of Lymphoid Neoplasma (REAL) and WHO
• Based on clinical morphology, immunophenotype, genotype, and clinical manifestations of lymphoma.
MALT lymphoma
(Mucosa-Associated Lymphoid Tissue)
Chronic Lymphocytic Lymphoma (CLL)
Follicular Center Lymphoma
High Grade Lymphomas
• Low grade lesion, 40-60% of orbital lymphoma.
• Spontaneous remission in 5-20% of cases, but can also undergo a
histological transformation become more aggressive.
• Low grade lesion of lymphocytes that appear mature
• Low grade lesion with a follicular center.
• Including large cell lymphoma, lymphoblastic lymphoma and
Burkitt lymphoma.

11. GRADING AND STAGING OF LYMPHOMA
Grading  Describes the progression of the disease
Staging  The extent of organ involvement in lymphoma
The Ann Arbor Staging System is the most commonly used staging system for orbital lymphoma.
Low Grade
• Indolent or slow growth rate.
Intermediate Grade
• Moderate growth rate.
High Grade
• Aggressive or rapid growth rate.
Stage I
• Confined to
the orbit.
Stage II
• Involvement
of adjacent
structures
such as the
sinuses,
tonsil, and/or
nose.
Stage III
• Abdominal
nodal disease
below the
diaphragm.
Stage IV
• Disseminated
involvement
of one or
more
extranodal
sites, such as
liver or bone.

12. DIAGNOSIS
 The diagnosis of orbital lymphoma  History and physical examination, complete blood count,
imaging (CT scan or orbital MRI), and biopsy.
 Systemic examination to determine the involvement of other organs  very important in orbital
lymphoma  Collaboration with an internist.
CT scan
 homogeneous and isodense texture to muscle, showing mild contrast-enhanced improvement.
Lymphoma usually does not cause bone destruction.

13. DIAGNOSIS
MRI
Isointense or hypointense mass on T1-weighted, but appears isointense to hyperintense on T2-
weighted.
MRI of a large cell lymphoma patient with multiple intraorbital
masses infiltrating the lacrimal gland and extraocular muscles.

14. DIAGNOSIS
Histopathological examination
 Important diagnostic step in the management of orbital lymphoma.
Open biopsy or fine-needle aspiration biopsy (FNAB)
Diagnosis of lymphoma is confirmed when diffuse immature cells are found and lymphocytes
that are actively mitotic.

15. DIAGNOSIS

16. DIFFERENTIAL DIAGNOSIS
The differential diagnosis of orbital lymphoma is extensive, due to the lack of specific
features for orbital lymphoma.
 The differential diagnosis for orbital lymphoma includes:
• Benign lymphoproliferative lesions,
• Orbital tumor metastases,
• Pseudotumor,
• Lacrimal adenoma,
• Cavernous hemangioma, and
• Diffuse lymphangioma.

17. Observation Surgical
Therapy
Radiotherapy Chemotherapy Immunotherapy
17
MANAGEMENT
Some of the main criteria for the choice of optimal therapy and treatment are:
lymphoma histopathological subtypes, the extent of the disease inside and outside the periocular area, prognostic
factors associated with the patient and disease, and the impact of orbital lymphoma on visual function.

18. MANAGEMENT
 Observation
 Controversial
 May be used in frail elderly patients with asymptomatic disease or in severe comorbid that
preclude aggressive therapeutic approaches.
 Surgical Therapy
• Diagnostic and therapeutic.
Diagnostic  Biopsy
Surgical resection  Capsulated tumors, such as tumors of the lacrimal gland and
conjunctiva.
• Microinfiltration of the tumor into the surrounding tissue  the risk of recurrence.
• Surgery as a primary therapy is not recommended.

19. MANAGEMENT
 Radiotherapy
 Therapeutic option in patients with early stage and localized orbital lymphoma.
 The dose  depends on the stage of orbital lymphoma.
 Radiotherapy doses of 25-35 Gy are recommended in low grade lymphoma.
 Chemotherapy
 For more aggressive histological subtype orbital lymphoma with potential for systemic
involvement and distant metastases such as large diffuse B-cell lymphoma.
 Usually done after surgical therapy, radiotherapy or in patients with an advanced stage.
 Indolent lymphoma is very sensitive to single agent and combination chemotherapy.

20. MANAGEMENT
 Monotherapy
 Low grade lymphoma
 Chlorambucil or fludarabine and
cyclophosphamide
 In MALT lymphoma, the average total
dose is 600 mg.
 Combination chemotherapy
 Intermediate - high grade lymphoma
 Combination regimen cyclophosphamide,
doxorubicin, vincristine, and prednisone
(CHOP) or cyclophosphamide, vincristine,
doxorubicin, and dexamethasone (CVAD).
 Chemotherapy
 Immunotherapy
 Anti-lymphocyte antibodies  New form of lymphoma treatment.
 The most commonly used  Anti-CD-20 antibody, rituximab  destruction of B cells using
complement and antibody-mediated destruction mechanisms and induction of apoptosis.

21. RESEARCH METHODS
Place and Time of
Research
• At the Medical Records
Division of M. Djamil
Hospital Padang in
November and December
2020.
Types of Research
• Retrospective descriptive
study
• Data were taken from
specific status of tumor
subdivision and medical
records  grouped
according to general data
and tumor data.
Population and
Sample Research
• The population was all
orbital lymphoma patients
who were treated at the
Ophthalmology
Department of M. Djamil
Hospital Padang from
January 2015 to December
2020.
• All populations were
sampled.

22. RESEARCH METHODS
Inclusion Criteria
• Orbital lymphoma patients who have been diagnosed based on histopathology in Oncology
Subdivision Department of Ophthalmology, M. Djamil Hospital Padang from January 2015
to December 2020.
• Orbital lymphoma patients who have complete medical records.
Exclusion Criteria
• Patients with suspected orbital lymphoma, but has not been confirmed by histopathological
examination.
 Sample Criteria

23. RESEARCH METHODS
Orbital lymphoma
• A malignancy of the lymph nodes or extranodal lymphoid tissue characterized by the
proliferation or accumulation of lymphoid tissue cells and may appear on the conjunctiva,
lacrimal glands, palpebral soft tissue, and extraocular muscles, which are usually extraconal.
Orbital lymphoma patients
• Patients who have been diagnosed with orbital lymphoma based on the results of the
anatomical pathology examination after the biopsy.
 Operational Definition

24. RESEARCH RESULT
Characteristics Amount (n) %
Gender
Male
Women
13
4
76.47
23.53
Age (years)
40-50
51-60
61-70
>71
5
5
4
3
29.41
29.41
23.53
17.65
Characteristics of Lymphoma Patients According Age and Gender

25. RESEARCH RESULT
Clinical Symptoms Amount %
Proptosis
Mass on the palpebra
10
7
58.82
41.18
Total 17 100
Lateralization Amount %
Unilateral
Bilateral
14
3
82.35
17.65
Total 17 100
Distribution Acoording to Clinical Manifestations Distribution according to lateralization

26. RESEARCH RESULT
Histopathology
Category Amount %
Small lymphocytic type
Large cell type
15
2
88.24
11.76
Total 17 100
Therapy Amount %
Surgical
Chemotherapy
1
16
5.88
94.12
Total 17 100
Distribution According to Histopathological
Examination
Distribution According to therapy

27. DISCUSSION
17 patients with orbital lymphoma who were treated at M. Djamil Hospital Padang in the period
January 2015 to December 2020  13 male (76.47%) and 4 female (23.53%).
Sjo LD (2009)
• Majority of
orbital
lymphomas were
found in male.
Olsen et al
(2019)
• More cases in
men: 52% of men
and 48% of cases
in women.
Ahmed et al
(2020)
• More cases in
women 53.3%
and 46.7% male.
There are variations in gender in cases of orbital lymphoma

28. DISCUSSION
Sharma et al
(2015)
• Orbital
lymphoma can
appear in the
age range of 15 -
70 y.o  mostly
in old age.
Olsen et al
(2019)
• The average age
of patients with
orbital
lymphoma who
came to
treatment was
73% over 50 y.o.
Ahmed et al
(2020)
• The average age
of orbital
lymphoma
patients in the
United States is
66 years.
In this study  The average age of patients was 60 years. The youngest age was 44 years
and the oldest age was 84 years

29. DISCUSSION
The most manifestation of orbital lymphoma was proptosis (58.82%) and mass in the
palpebra (41.18%). Almost all manifestations were unilateral (82.35%).
The majority of orbital lymphoma presents unilateral manifestations,
especially in B cell lymphoma, up to 90%.
 Salmon patch appearance
 On the medical records  were not found, because almost all
patients came with chief complaints of protusion accompanied
by chemosis  The appearance of the salmon patch is difficult to
judge. Mr. N, 65 y.o was diagnosed with orbital
lymphoma with clinical manifestations of a mass
in the superior palpebrae on the RE.

30. DISCUSSION
All patients with suspected orbital lymphoma  CT scan and biopsy.
CT scan  All performed as an orbital mass and showed the impression of a lymphoma.
The histopathological examination  Non-Hodgkin's lymphoma with small lymphocytic type in
88.24% and large cell type in 11.76%.
An estimated 85-90% of orbital lymphomas are low grade, with small, monoclonal and diffuse
proliferation of B-cell lymphocytes. Whereas follicular or nodular features are found only 10-15%
of cases

31. DISCUSSION
 One patient  has two
histopathological examination results.
 The first biopsy  reactive
hyperplasia of the lymph nodes.
 The second histopathological result 
a small cell type non-Hodgkin's
lymphoma.
 All patients  consulted to the
Internal Medicine Department to find
out any accompanying systemic
abnormalities.
 All patients had systemic
abnormalities.

32. Surgical therapy alone is not recommended
 high risk of relapse after surgery.
Radiotherapy as initial therapy has been
reported to be very effective in orbital
lymphoma, especially in MALT.
DISCUSSION
 The management  Chemotherapy in 16 patients (94.12%) and 1 patient (5.88%)
anterior orbitotomy surgery and refusing chemotherapy.
 Chemotherapy is performed by internists using the CHOP regimen

33. DISCUSSION
 Systemic chemotherapy  effective therapies for orbital lymphoma.
 Mainly given in the form of a combination post surgery and radiotherapy
or given alone in stage III and IV lymphoma.
 In more aggressive lymphoma, chemotherapy is the more appropriate
option.
 In this study  Chemotherapy was recommended. The regimen used was
cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP).

34. CONCLUSION
During January 2015 to December 2020 there were 17 cases of orbital
lymphoma who went to M. Djamil Hospital Padang, majority of male
patients, with a mean range of age 60 years.
The most clinical manifestations of orbital lymphoma were proptosis
(58.82%) followed by a palpebral mass (41.18%) and most cases were
unilateral.

35. CONCLUSION
All patients were performed orbital CT scan and histopathological
examination. Most of patients were non-Hodgin lymphoma with small
lymphocytic type which is a low grade lymphoma.
There was one patient with a mismatch between clinical manifestations
and histopathological results so the histopathological examination was
reviewed again.
The management performed in this orbital lymphoma patient was
chemotherapy in 16 patients and 1 patient refused chemotherapy and
performed an anterior orbitotomy.

36. THANK YOU
Kerinci Mountain 3805 mdpl

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45. ECOG/WHO/Zubrod score
• 0 – Asymptomatic (Fully active, able to carry on all predisease activities without
restriction)
• 1 – Symptomatic but completely ambulatory (Restricted in physically strenuous activity
but ambulatory and able to carry out work of a light or sedentary nature. For example,
light housework, office work)
• 2 – Symptomatic, <50% in bed during the day (Ambulatory and capable of all self care
but unable to carry out any work activities. Up and about more than 50% of waking
hours)
• 3 – Symptomatic, >50% in bed, but not bedbound (Capable of only limited self-care,
confined to bed or chair 50% or more of waking hours)
• 4 – Bedbound (Completely disabled. Cannot carry on any self-care. Totally confined to
bed or chair)
• 5 – Death

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Persebaran nodus limfa di tubuh manusia

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