Burkitt ’ s Lymphoma in a 5 year old Bengali Girl : A Case Report

Burkitt's lymphoma (BL) is a rare monoclonal proliferation of B-lymphocytes and is classified as a poorly differentiated lymphocytic lymphoma. This tumor was first noted in Africans. The cause of this tumor is debatable, but strong evidence implicates Epstein-Barr virus in its development. This tumor predominantly affects children and is probably the fastest growing tumor in humans, with exuberant proliferation. It is a very rare malignancy accounting for only 0.76% of solid malignant tumors among Indian children. A case of BL of the right hemimandible in a 5-year-old Bengali girl is reported. Key words: Burkitt's lymphoma; Immunohistochemistry; Chemotherapy. DOI: 10.3126/jnps.v31i1.3903 J Nep Paedtr Soc 2010;31(1):61-63


Introduction
L ymphomas are a group of malignant tumors involving cells of the lymphoreticular or immune system such as.Burkitt's lymphoma (BL) is the eponym given to a malignant tumor of the hematopoietic system, characterized by undifferentiated lymphocytes.It is a high-grade aggressive subgroup of non-Hodgkin's lymphoma and is composed of small, noncleaved, diffuse, undifferentiated, malignant cells of B lymphoid origin 1 .
African (endemic) BL (eBL) occurs as a pediatric disease and is almost always associated with Epstein-Barr virus (EBV) exposure.American (sporadic) BL (sBL) occurs in children and adults elsewhere and is less likely to be related to EBV.Patients with human immunodefi ciency virus (HIV) also appear to be at risk for developing BL 2 .

Case Report
A 5 year old girl was referred to us from a peripheral hospital with chief complaints of progressively increasing swelling over right lower jaw with diffi culty in degluttition for about a month.She had a history of tooth extraction in the recent past.After that the swelling was increasing in size & involved whole of the right hemimandible.Teeth became loose and the swelling became prone to bleed.The child also had diffi culty in talking and was having occasional dyspnoea.
Patient was alert, conscious & cooperative with anxious look.She was anaemic but afebrile.Neck veins not engorged, neck glands were not palpable.Respiratory rate was 26/ min.There was a 16cm x 10 cm oval shaped mass involving the right hemimandible with venous prominence and shiny skin over it with raised temperature.Intraorally the swelling had irregular ulcerated surface which bled on touching.Tongue was deviated to left with dislocation of few molar and premolar teeth.Liver and spleen were nonpalpable.Lymph nodes at other stations were also impalpable.
Clinically it was thought that this was a case of osteosarcoma of right half of mandible.Other differential diagnoses were fi brous dysplasia of jaw and adamentinoma.
In the mean time there was rapid increase in the size of the swelling with episodic alarming bleeding from the intraoral part.Patient underwent tracheostomy followed by bilateral external carotid artery ligation and punch biopsy from the intraoral part of the lesion.Ryle's tube was inserted for enteral feeding.
The SOL responded dramatically following two cycles of chemotherapy and she was discharged after closure of tracheostoma.At present she is on regular follow up and is doing well.
Relevant investigations at the time of admission.

Discussion
The sporadic (North American) form of Burkitt lymphoma more commonly has an abdominal presentation (typically pain), whereas the endemic (African) form frequently presents with tumors of the jaw 3 .Endemic Burkitt's lymphoma refers to those cases occurring in African children, usually 4-7 years old, with a male: female ratio of 2:1, involving the bones of the jaw and other facialbones, as well as kidneys, gastrointestinal tract, ovaries, breast, and other extranodal sites 4 .Childhood lymphomas with the morphology of Burkitt's lymphoma appear to be uniform with respect to immunophenotype and cytogenetics; this is not true of such lymphomas in adults 5 .Although the immunophenotype of bona fi de examples of Burkitt's lymphoma is uniform from case to case, the features of diffuse large B-cell lymphoma are heterogeneous, and a subset of them has an immunophenotype that is the same as that seen in Burkitt's lymphoma 6 .BL cells are the neoplastic counterparts of a subset of normal activated germinal center B-cells 7 .BL cells express surface IgM and B-cell-associated antigens   (CD19, CD20, CD22, CD79a), as well as CD10, HLA-DR, and CD43 8 .They lack CD5, B cell leukemia/lymphoma 2 (bcl-2), and typically lack CD23 9 .They show nuclear staining for B cell lymphoma 6 (BCL-6) protein, which is independent of bcl-6 gene rearrangement 10 .
Treatment includes dose-adjusted EPOCH with rituximab 11 .The result of chemotherapy and immunotherapy are excellent.Surgical debulking of large localized jaw or abdominal tumors is benefi cial prior to chemotherapy.Cyclophosphamide 40 mg/kg in a single intravenous administration and repeated about 2 weeks later has given good results.Vincristine and methotrexate are also successful in some cases.A recent report suggests a combination of Cyclophosphamide, vincristine and methotrexate give better results than any single drug.

Conclusion
B urkitt's lymphoma is a rare, rapidly progressing malignant tumor of childhood with varied clinical features.Its infrequent occurrence among the Indian population should not deter the clinician from including it as a part of differential diagnosis.The importance of good clinical acumen in the early diagnosis of the disease cannot be overemphasized.The present case is a fi tting example of the importance of timely diagnosis and prompt treatment which proved to be life saving for the child.

Fig 2 :
Fig 2: Digital OPG X Ray showing cyst like SOL in the right side of the mandible with displacement of teeth without any thinning or destruction of cortical boundary.

Fig 1 :
Fig 1: Patient at the time of presentation.