This document presents a case of orbital Burkitt lymphoma in a 14-year-old male from Uganda who initially presented with abdominal symptoms and was diagnosed with Burkitt lymphoma. He received chemotherapy but later developed right eye swelling and proptosis. Examination found a large orbital mass and biopsy confirmed recurrent Burkitt lymphoma. The key points discussed include the clinical features of Burkitt lymphoma, the diagnostic approach involving imaging and biopsy, the characteristic pathology findings, and management with chemotherapy and possible radiation therapy.

1. ORBITAL BURKITTS LYMPHOMA
Dr. Mugabi Barnabas
International oculoplastics fellow teaching 13/07/2023

2. Case presentation
• 14-year-old male
• Referred in April 2023 with 1 month history of right eye
swelling and proptosis

3. September 2022
• h/o an abdominal mass in Sept. 2022 associated with pain,
abdominal distension with ascites, and jaundice.
• Pyrexia, excessive sweating, weight loss.
• Anaemia, and generalized lymphadenopathy.
• No h/o of TB contact, clay like stool with dark-colored urine

4. Investigations
• FNAC
• Immunohistochemistry
• Flow cytometry
• Bone marrow aspirate
• CBCs

6. History continued
• He was diagnosed of Burkitt lymphoma
• Started on chemotherapy
• cyclophosphamide, vincristine, methotrexate, and prednisone
• He had satisfactory response and was discharged in March 2023

7. A month later…

8. • Referred by hematology department
• One month of rapidly progressive right
eye proptosis and lower eyelid swelling.
• Symptoms preceded by headache
associated with reduced vision and pain

9. Past ocular history
• Unremarkable, no previous trauma or surgery, but was
on eyedrops of unknown prescription
General medical history
• Treated Burkitt lymphoma – completed chemotherapy
• Previous blood transfusions
• Acute liver injury
• Malnutrition
• GCSF
• No other chronic illnesses such as HTN, DM, HIV, sickle cell disease

10. General surgical history
Unremarkable
Drug history
• Prednisolone
• Methotrexate
• Omeprazole
• Ondarsetron
• Metroclopramide
Family history
No h/o sickle cell disease or trait
Social history
Neither drinks nor smokes

11. General examination
Young child, wasted, Temp: 36.7, (JACOLD) unremarkable
Respiratory system: not in distress, equal air entry bilaterally, normal
breath sounds. 15rpm
CVS: chest symmetry ,H1and 2 normal, BP; 101/64mmHg,PR; 60bpm
Abdomen: normal fullness, no palpable organs

12. • Visual acuity: OD 6/24 OS 6/6
• Right proptosis
• Superomedial globe displacement
• Inferior scleral show (3-4mm)
• Firm, non-tender, non-mobile palpable
mass mainly along the inferior orbital rim,
measuring 4x6cm, with no pulsatility,
warmness or discolouration.

13. Ocular examination on 17 April 2023
PARAMETERS OD OS
LIDS Retracted with ptosis Normal
Conjunctiva hyperaemic normal
Cornea Clear Clear
A/C Not assessed because of pain Deep and quiet
Iris Not appreciated Normal
Pupil Dilated and reacting to light
No obvious RAPD
CRRL
No RAPD
Lens Not well appreciated clear
EOM Partial ophthalmoplegia Full
Fundus Not done WNL
CDR O.3

14. Ocular motility
No lagophthalmos

15. No lesions in the buccal cavity
No palpable lymph nodes

16. • Recurrent Burkitt lymphoma with orbital mets
• Neuroblastoma
• Rhabdomyosarcoma
• Orbital cellulitis
Differential diagnosis

18. HIV testing was negative

19. Incisional biopsy was performed and the patient went
back to paediatric hematology department and was
restarted on chemotherapy for presumed disease relapse.

20. 10 days later…
Markedly increased proptosis
Hertel
Right >30mm – base 118 – left 18mm
Right acuity – NPL
Left acuity – 6/9

21. SLE
PARAMETERS OD OS
LIDS Retracted, lagophthalmos
Negative bell’s phenomenon
Normal
Conjunctiva Hyperaemic and chemotic normal
Cornea Clear Clear
A/C Not assessed because of pain Deep and quiet
Intraocular pressure 16 mmHg 18 mmHg
Iris Not appreciated Normal
Pupil Dilated and poorly reacting to light CRRL
No RAPD
Lens Not well appreciated clear
EOM severe ophthalmoplegia Full
Fundus Not done WNL
CDR O.3

22. Biopsy result confirmed Burkitt lymphoma
of the orbit (African type)
Improved on chemotherapy

24. Discussion
• General considerations
• Clinical features
• Diagnostic approach
• Pathology
• Management

25. General considerations
• Burkitt lymphoma is a non-Hodgkin B-cell
lymphoma that is being recognised more in
the orbit
• Rapidly progressive, solid lymphoma with
a predilection for the jaw and abdomen
• 50% of childhood malignant tumors in East
Africa

26. • Three distinct forms of Burkitt lymphoma
 African type
 Non-African (American) type
 Acquired immunodeficiency syndrome
(AIDS) type
• Involves the orbital soft tissue or bone
and can extend into the globe.

27. Clinical features
• In the African form, orbital involvement is commonly secondary to
invasion from the maxillary bone.
• The American form typically involves lymph nodes, bone marrow, and
viscera.
• The AIDS-related form is more aggressive and mainly affects the
central nervous system in patients with AIDS

28. Diagnostic approaches
• An abdominal and orbital mass with
proptosis and hyperglobus (unilaterally or
bilaterally) in an African child is highly
suggestive of diagnosis.
• Orbital CT and MRI demonstrate a maxillary
mass with secondary orbital involvement.

29. Diagnostic approaches
• The AIDS-related form can involve
both orbital soft tissue and bone
• A patient with suspected Burkitt
lymphoma should have a systemic
evaluation for lymphoma and HIV
infection.

30. Pathology
• Burkitt lymphoma is a proliferation
of closely packed B lymphocytes
• Interspersed histiocytes containing
phagocytosed debris cause the classic
“starry sky” appearance
• Evidence of the EBV
• Chromosomal abnormalities, particularly a translocation of chromosome 8
to the long arm chromosome 14, occur in many cases.

32. Management
• Management parallels that for the systemic disease
• In general, a biopsy should be done combined with attempted debulking of the
lesion.
• This tumor is extremely sensitive to chemotherapeutic agents using
cyclophosphamide, vincristine, methotrexate, and prednisone.
• External irradiation (30 Gy to the affected area) can be used in some cases that
appear resistant to chemotherapy
• The prognosis has improved greatly in recent years.

33. References
• 1.Jaffe ES. The 2008 WHO classification of lymphomas: implications for clinical practice and
translational research. Hematology 2009;523–531.
• 2.Burkitt D. A sarcoma involving the jaws in African children. Br J Surg 1958;46:218–223.
• 3. Burkitt D, O’Conor GT. Malignant lymphoma in African children. I. A clinical syndrome. Cancer
1961;14:258–269
• 4.Templeton AC. Orbital tumours in African children. Br J Ophthalmol 1971;55:254–261.
• 5.Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating
lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111: 997– 1008.
• 6.Reifler DM, Warzynski MJ, Blount WR, et al. Orbital lymphoma associated with acquired
immune deficiency syndrome (AIDS). Surv Ophthalmol 1994;38:371–380.