Journal of Indian Society of Pedodontics and Preventive Dentistry
Journal of Indian Society of Pedodontics and Preventive Dentistry
                                                   Official journal of the Indian Society of Pedodontics and Preventive Dentistry                           
Year : 2007  |  Volume : 25  |  Issue : 4  |  Page : 194--199

Burkitt's lymphoma in an Indian girl: A case report

K Patil1, VG Mahima1, BS Jayanth2, L Ambika1,  
1 Department of Oral Medicine and Radiology, J.S.S. Dental College and Hospital, Mysore - 570 015, India
2 Department of Oral and Maxillofacial Surgery, J.S.S. Dental College and Hospital, Mysore - 570 015, India

Correspondence Address:
K Patil
Department of Oral Medicine and Radiology, J.S.S. Dental College and Hospital, S.S. Nagar, Mysore-570 015


Burkitt«SQ»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 mandible in a 9-year-old girl of Indian origin is reported.

How to cite this article:
Patil K, Mahima V G, Jayanth B S, Ambika L. Burkitt's lymphoma in an Indian girl: A case report.J Indian Soc Pedod Prev Dent 2007;25:194-199

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Patil K, Mahima V G, Jayanth B S, Ambika L. Burkitt's lymphoma in an Indian girl: A case report. J Indian Soc Pedod Prev Dent [serial online] 2007 [cited 2020 Oct 29 ];25:194-199
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Lymphomas are a group of malignant tumors involving cells of the lymphoreticular or immune system such as B-lymphocytes, T-lymphocytes, and monocytes. 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 immunodeficiency virus (HIV) also appear to be at risk for developing BL. [2]

 Case Report

A 9-year-old girl reported to the Department of Oral Medicine and Radiology, J.S.S Dental College and Hospital with the chief complaint of swelling in the lower jaw on the left side since 15 days. The patient gave a history of painful decayed lower left posterior tooth 20 days back, which was extracted. A swelling developed in the same region following the extraction. The swelling was asymptomatic and had rapidly increased over a period of 15 days to attain the present size. Patient gave history of difficulty in chewing and bleeding from the gums of lower left teeth on brushing. There was no history of paresthesia. Past medical, surgical, and drug histories were unremarkable. The patient's family history was not significant.

General physical examination revealed a moderately built and nourished child. Bilateral submandibular lymph nodes were enlarged, palpable and tender, one on each side, measuring about 1 cm × 1.5 cm in size and firm in consistency. The left submandibular lymph node was fixed to the underlying structures, whereas the right lymph node was mobile in both anteroposterior and mediolateral planes. Lymph nodes of other regions were not palpable.

Extraoral examination showed a solitary, diffuse swelling measuring approximately 6 cm × 5 cm in size on the left side of the mandible extending from the level of ala of the nose to the lower border of mandible. Mediolaterally, the swelling extended from angle of mouth to the angle of mandible on left side [Figure 1],[Figure 2]. The surface of the swelling appeared smooth, stretched, and erythematous. There was no evidence of secondary changes over the swelling. On palpation, there was local rise in temperature and the swelling was moderately tender. It was firm in consistency, nonfluctuant, noncompressible, nonreducible, and no discharge was noted. The swelling was fixed to the underlying structures.

Intraoral hard tissue examination showed mixed dentition. Grade II mobility was noted in 75, 36, 84, 85, and 46. Soft tissue examination revealed a solitary, well-defined, lobulated, erythematous, mass measuring approximately 5 cm × 6 cm in the mandibular left posterior region [Figure 3]. The mass extended anterioposteriorly from the mandibular deciduous first molar to the permanent first molar on the left side. It also extended to the buccal and lingual vestibules. The surface of the swelling appeared irregular with pressure indentation of teeth and petechiae. On palpation, the mass was nontender, firm in consistency, nonfluctuant, noncompressible, and nonreducible. The patient's oral hygiene was fair. There was generalized gingival bleeding on gentle probing.

History and clinical examination were suggestive of a malignant neoplasm arising from the mandible. A differential diagnosis of non-Hodgkin's lymphoma, Ewing's sarcoma, BL, osteosarcoma, chondrosarcoma, neurosarcoma, and fibrosarcoma was made. Osteomyelitis of the mandible was also considered.

Hematological investigations were carried out. Increased white blood cell counts (12,200 cells mm -3 ) and raised erythrocyte sedimentation rate (ESR; 16 mm after 1 h) were evident. HIV test was performed using Abbot to determine Immunoassay and was found to be negative. Serochemical values of alkaline phosphatase and lactic dehydrogenase were raised and were found to be 133 U/l and 2584 U/l, respectively.

Intraoral periapical radiograph showed complete loss of lamina dura in relation to 75 and 36 with no evidence of periodontal ligament space [Figure 4]. The bone level could not be appreciated suggesting that the teeth were just suspended in the soft tissue. Mandibular cross-sectional occlusal view showed buccal and lingual cortical plate expansion [Figure 5].

Panoramic radiograph showed soft tissue shadow of the tumor mass on the left side of the mandible. The left body of the mandible showed an ill-defined radiolucency with destruction of trabeculae giving a "moth-eaten" appearance. The mandibular left and right first permanent molars were displaced by the radiolucent tumor giving characteristic appearance of "teeth-floating in air" [Figure 6]. Chest X-ray and ultrasound of the abdomen were normal.

Incisional biopsy of the intraoral mass was peformed. Histopathological sections showed stratified squamous epithelium with a subepithelial tumor composed of uniform dysplastic cells arranged in sheets with scanty stroma [Figure 7]. Lymphocytes were large and round with scanty cytoplasm and increased nuclear to cytoplasmic ratio. Vesicular nuclei with prominent nuclei were seen. Many abnormal and normal forms of mitoses were evident. Numerous macrophages within the tumor tissue gave a characteristic "starry-sky appearance" consistent with the classical picture of BL [Figure 8],[Figure 9].

Immunohistochemical investigation was performed. CD-20 protein tumor marker was diffusely positive in neoplastic cells and substantiated the diagnosis of BL [Figure 9]. Bone marrow aspiration was performed which suggested acute lymphoblastic leukemia-L3. For cytochemical analysis, the samples were subjected to specific stains, namely Myeloperoxidase and Periodic acid Schiff stain (PAS). Myeloperoxidase staining was carried out to rule out myeloid from lymphoid series of cells and was found to be negative. PAS stained positively to lymphoblasts. Chromosomal aberration t(8-14) was observed during the cytogenetical analysis further confirming the diagnosis of BL.

The patient was referred to a pediatrician for systemic evaluation and on examination the patient was found to be free of other systemic manifestations of the disease. A final diagnosis of BL was considered based on the clinical, hematologic, radiographic, histopathologic, immunohistochemical, and cytochemical investigations.

The patient was referred to a regional oncology center where prompt chemotherapy was instituted. After first cycle of MCP (methotrexate and cyclophosphamide) protocol, the swelling reduced to one fourth of its original size. Subsequently, seven more cycles of chemotherapy were instituted. At the end of eight cycles of chemotherapy, the swelling showed complete regression [Figure 10],[Figure 11]. Milky white scrapable plaques typical of pseudomembranous candidiasis were observed on the left buccal mucosa of the patient following chemotherapy [Figure 12], which subsided on oral fluconazole therapy.

Post-treatment radiographs showed no evidence of soft tissue shadow of the tumor mass and bony destruction as was seen in the previous appointment. Bone regeneration and normal trabecular pattern were regained following chemotherapy [Figure 13],[Figure 14],[Figure 15]. The patient is under observation and is asked to report every 2 months for evaluation.


Burkitt's lymphoma is a high-grade aggressive subgroup of non-Hodgkin's lymphoma and is composed of small, noncleaved, diffuse, undifferentiated malignant cells of lymphoid origin. [3] It is the fastest growing human tumor with a doubling time of less than 24 h. [3],[4] Dennis Burkitt first described this entity in 1956 in equatorial Africa. [5]

In Africa, this tumor accounts for approximately 50% of all childhood cancers. Outside Africa, it accounts for less than 2% of all cases of non-Hodgkin's lymphoma. In 1987, Choudary et al. conducted a retrospective analysis of BLs occurring in the Indian population. The results showed involvement of the jaws in 27 out of 52 published cases. This observation suggested a higher incidence of BL involving jaws as compared to the BL occurring in other nonendemic areas of the world. [2]

In an Indian series of solid malignant tumors in children, Pramanik et al. , in 1997, studied 263 cases over a 10-year period and found only two cases (0.76%) of BL. [6] Many etiologic theories have been espoused. The role of EBV in BL is not well understood. The virus may be a prime etiologic agent, a co-carcinogen, or just an innocent passenger. This virus preferentially infects B cells via the C3d complement receptor, CD 21 . [3] Other co-factors may include chromosomal abnormalities, immune defects, and protein energy deficits. [3]

Burkitt's lymphoma cells contain a reciprocal chromosomal translocation, the most frequent of which is an 8q24; 14q32 translocation. [3] Three subtypes of BL have been identified: [3]

African ( endemic ) Burkitt's lymphoma ( eBL ): It has a peak incidence between 3-8 years of age and the male to female ratio is 2 : 1. The commonest site of disease presentation in eBL is the face with multiple facial bone involvement. It usually involves the maxilla and mandible. [3],[7],[8]

American ( sporadic ) Burkitt's lymphoma ( sBL ): The sporadic form affects older individuals, with a mean age of 11 years and has no gender predilection. [7] It is more likely to have leukemic or bone marrow involvement and less likely to have jaw involvement. [6] In sBL, the most common site of presentation is the abdomen. [7],[8]

HIV-associated Burkitt's lymphoma : Burkitt's lymphoma is also known to be associated with HIV infection. Most patients are adults with marked immunosuppression. There is tumor presentation both in lymph nodes and at extranodal sites particularly in CNS, bone marrow and gastrointestinal tract. [9]

Earliest sign of BL is loosening of the teeth. Pain and paresthesia are occasionally present. [7],[8] As the tumor grows, the teeth are displaced out of their sockets. When permanent teeth are affected they erupt prematurely, which is often an overlooked early sign of the tumor of the jaw. BL is also unique in which it is perhaps the only malignancy that infiltrates the dental tissues - the dental pulp, developing tooth follicle, and the periodontal ligament. [8],[10] Facial deformity may be evident in the early stage of disease. [10],[11]

Cervical lymphadenopathy is very rare in endemic form but more common in sporadic form of BL. [1] Asymptomatic enlargement of the cervical lymph node chain is a common early sign. Suspicion of lymphoma should increase when lymphadenopathy appears without sign of infection or there is involvement of more than one lymph node chain or a lymph node 1 cm or greater in diameter persisting for more than 1 month. [1]

Further growth leads to expansion of the jaw, displacement of the teeth, derangement of the arch and occlusion, and a large mass protruding into the mouth. In spite of such gross displacement of developing teeth, their exfoliation is a late feature in the disease process. [10]

Burkitt's lymphoma in India is intermediate between the sporadic and endemic types in its clinical presentation. Its association with EBV varies from 25% to 80%. [6] The current case too could be classified as intermediate, exhibiting features of both endemic and sporadic forms. The features in favor of endemic BL are age of the patient, site of involvement, and presence of mild pain.

The features in favor of sporadic BL are the Indian origin of the child, enlarged submandibular group of lymph nodes, and its association with acute lymphoblastic leukemia.

A separate staging system for BL has been developed by Ziegler (1981), whereas Levine et al. , (1982) classified the cases of the American BL as follows: [12]

Stage I: single tumor mass (extra-abdominal 1A or abdominal 2A).

Stage II: two separate tumor masses on the same side of the diaphragm.

Stage III: involvement of more than two separate masses or disease on both the sides of the diaphragm.

Stage IV: pleural effusion, ascitis or involvement of the central nervous system (malignant cells in the cerebrospinal fluid) or bone marrow.

On the basis of the staging proposed by Levine et al. , the present case can be grouped as Stage IV due to the bone marrow involvement.

Jaw tumors are commonly confused with lesions such as an acute dental abscess, osteomyelitis, ossifying fibroma, embryonal rhabdomyosarcoma, osteosarcoma, Langerhan's disease, histiocytoma, dentigerous cyst, mucoepidermoid carcinoma, eosinophilic granuloma, neuroblastoma and fibrous dysplasia. [3],[10],[13]

Radiologic features of this osteolytic lesion aid in the diagnosis. The lesions may begin as multiple, ill- defined, non-corticated radiolucencies, which later coalesce into larger, ill-defined radiolucencies with an expansile periphery. [3]

Erupted teeth in the area of Burkitt's tumors are grossly displaced, as are developing tooth crypts. Tumor cells within the crypt may displace the developing tooth bud to one side of its crypt. A tumor that is located apical to a developing tooth may cause it to be displaced such that it appears to erupt with little, if any, root formation. [2] Lamina dura of the involved teeth is destroyed [14] and the cortical boundaries such as inferior borders of the mandible are thinned and later destroyed. [3] Our case showed similar radiological features but there was no thinning of the inferior border of the mandible on the left side.

Burkitt's lymphoma can be distinguished histologically and cytologically from other forms of malignant lymphomas. Histologic sections show an undifferentiated type of B-cell lymphoma. Immature cells, 10-25 mm in diameter proliferate and have several prominent nucleoli within rounded nuclei. The sheets of tumor cells are interspersed with large pale macrophages, providing the "starry-sky" appearance, which is typical of but not unique to BL. [9],[14]

Immunohistochemical stains Ki-67, CD-19, CD-20, CD-22, CD-79a protein may be useful in diagnosis. [6],[11] In the present case, CD-20 protein marker was used, which was diffusely positive in neoplastic cells.

Several laboratory findings are consistently abnormal in BL patients. Serum lactate dehydrogenase (LDH) is elevated to a level corresponding to the extent of the tumor dissemination. Increased activity of alanine aminotransferase, serum alkaline phosphatase, [3],[15] and immunoglobulins has been reported. [14] Anemia and leukocytosis are common. In addition, the ESR and blood urea nitrogen (BUN) may be elevated. [2] This patient showed increased levels of LDH, serum alkaline phosphatase, ESR, and leucocytosis.

The distortion of the jaws and face caused by the tumor may be bizarre and dramatic. The result of chemotherapy and immunotherapy are excellent. [5] Surgical debulking of large localized jaw or abdominal tumors is beneficial prior to chemotherapy. [1] 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. [10],[16] A recent report suggests that a combination of cyclophosphamide, vincristine, and methotrexate give better results than any single drug. [10]

Radiotherapy produces dramatic results but because of the complexities of immune system, it is probably contraindicated. [5] It has been found that prognosis improves with younger age at diagnosis, minimal tumor burden and rapid initiation of chemotherapy, demonstrating the importance of early diagnosis and treatment. [3] Dramatic remissions occur in more than 90% of patients with high-dose alkylating agent therapy as seen in our case. Relapse occurs in two thirds of cases, most often in patients with advanced stages. With combination chemotherapy, the overall 2-year survival rate is 55% with a range of 80% for low-stage disease and 40% for the advanced stage disease. [7]


Burkitt'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 fitting example of the importance of timely diagnosis and prompt treatment which proved to be life saving for the child.


The authors wish to thank Dr. Sunila, Professor and Head, Department of General Pathology, J.S.S Medical College and Hospital, Mysore; Dr. Lakshman, Consultant Pathologist, Kannan Diagnostic Center, Mysore; and Dr. Madhuri Wadhwa, Chief Pathologist, Vikram Diagnostics, Mysore.


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