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Burkitt's lymphoma of the base of the tongue: a case report and review of the literature.

Abstract

Burkitt's lymphoma is a highly aggressive, mature B cell non-Hodgkin's lymphoma that is rare outside Africa. We report a case of Burkitt's lymphoma presenting as a rapidly expanding tongue-base mass that caused airway obstruction in an 80-year-old Palestinian man living in California. According to our review of the literature, this is only the third reported case of Burkitt's lymphoma arising in the base of the tongue. We also discuss the incidence, epidemiology, genetics, prognosis, and treatment of this malignancy. Because Burkitt's lymphoma is one of the fastest-growing tumors in humans, rapid diagnosis and treatment are important. Treatment involves brief-duration, high-intensity chemotherapy and central nervous system prophylaxis. It is important for the otolaryngologist to recognize this disease and to understand the steps necessary to treat this aggressive tumor.

Introduction

Burkitt's lymphoma is a highly aggressive, mature B cell non-Hodgkin's lymphoma. It appears in three variant forms: endemic, sporadic, and immunodeficiency-associated. In all three variants, the c-myc oncogene is activated via a specific chromosomal translocation that results in disordered cell proliferation.

The tongue base is an extremely rare location for this tumor. To the best of our knowledge, only 2 cases have been previously reported in the English-language literature. (1,2) In this article, we report the third such case.

Case report

An 80-year-old Palestinian man presented to our clinic with a 3-month history of a muffled voice, odynophagia, cough, and snoring. Physical examination revealed that alarge mass had arisen from the right side of the base of the tongue; the mass crossed the midline and extended to the vallecula. On computed tomography (CT) of the tongue base, the mass measured 4.3 x 2.4 x 2.8 cm (figure 1, A).

[FIGURE 1 OMITTED]

The patient underwent local tracheotomy and endoscopy (figure 2) with biopsies. Pathologic examination of the biopsy specimens revealed a diffuse monomorphous population of small and medium-size lymphocytes with round to oval nuclei, prominent basophilic nucleoli, coarse chromatin, and a thick nuclear membrane; abundant mitotic figures and tingible-body macrophages were easily identified, giving rise to the classic "starry-sky" appearance (figure 3). A moderate amount of amphophilic cytoplasm was identifiable. Flow cytometry demonstrated expression of CD10, CD19, and CD20 antigens with monoclonal restriction of the lambda light chain. Tumor staging was performed on the basis of findings on lumbar puncture, bone marrow biopsy, liver function tests, chest x-ray, positron-emission tomography (PET), and CT of the head, chest, abdomen, and pelvis. All staging workups were negative (including a normal lactate dehydrogenase level), and imaging confirmed that the lymphoma was confined to the area of the tongue base. The patient was found to have microcytic anemia, and further workup revealed a beta-thalassemia trait.

[FIGURES 2-3 OMITTED]

The patient was treated with a modification of the Stanford regimen (3) plus rituximab for a total of 3 cycles of chemotherapy. Involved-field radiation was planned. (4) The first cycle of chemotherapy involved the administration of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) in addition to both intravenous and intrathecal methotrexate. Despite growth-factor support, the patient developed febrile neutropenia and was admitted. The second cycle of therapy consisted of R-CHOP and intrathecal methotrexate, and the patient again experienced febrile neutropenia despite growth-factor support. The third cycle consisted of R-CHOP in lower doses; the intrathecal methotrexate was omitted. Again, despite the dose reduction and the omission of intrathecal chemotherapy, the patient experienced febrile neutropenia.

Following the third cycle, PET and CT (figure 1, B) of the neck demonstrated that the tumor had resolved completely. Therefore, the decision was made to cancel the involved-field radiation.

At the 2 1/2-year follow-up, the patient exhibited no signs of residual lymphoma.

Discussion

Burkitt's lymphoma is the fastest-dividing lymphoma, with 100% of cells being in a cell cycle at any given time; it has been considered one of the most rapidly growing tumors in humans. (5) The tumor was first described as a neoplasm arising in the jaws of African schoolchildren. (6) Subsequently, a neoplasm of identical histology was identified in the United States. (7)

In 1994, the International Lymphoma Study Group published the revised European-American lymphoma (REAL) classification system for lymphoid neoplasms. (8) In this system, lymphomas were classified according to morphologic appearance, immunophenotype, and genetic features. In 2001, the REAL classification system was updated by the World Health Organization, which classified Burkitt's lymphoma together with Burkitt's leukemia as a mature (peripheral) B cell neoplasm. (9) Previous classification systems had also included "Burkitt's-like lymphoma," but this entity was subsequently considered to be the same as Burkitt's lymphoma. (10)

Morphologically, Burkitt's lymphoma cells have round nuclei with clumped nucleoli and an abundant basophilic cytoplasm that contains lipid vacuoles. The tumor has a diffuse pattern of infiltration. The starry-sky appearance under low magnification is caused by the presence of macrophages that contain ingested apoptotic tumor cells. Burkitt's lymphoma cells express surface IgM, bcl-6, CD10, CD19, CD20, CD22, and CD79a; they lack CD5, CD23, and TdT. (11)

Burkitt's lymphoma arises from activation of the c-myc oncogene, most often as a consequence of a chromosomal translocation between chromosome 8 and chromosome 14, 2, or 22; in 80% of cases, it is translocation (8;14). (12) In the endemic and sporadic variants, the location of this translocation is different. (12,13) Epstein-Barr virus (EBV) is strongly associated with the endemic variant, being present in 80 to 90% of tumor cells. (14,15) EBV is less strongly associated with the sporadic variant, being present in only 15 to 20% of tumor cells. (15) It is not known what role EBV plays in the pathogenesis of Burkitt's lymphoma. (16) EBV nuclear antigen, a viral protein consistently expressed in Burkitt's lymphoma cells, has been shown to be required for the survival of tumor cells. (17)

Endemic Burkitt's lymphoma is commonly seen in equatorial Africa, where it accounts for 50 to 70% of all pediatric malignancies. (18) The mean age at presentation is 7 years. (18) The disease frequently involves the jaw and kidney; jaw involvement occurs in approximately 50% of cases. (18) The ileum, cecum, ovary, and breast may also be involved, and invasion of the cerebrospinal fluid is common. (11)

Nonendemic Burkitt's lymphoma accounts for 1 to 2% of all adult lymphomas in Western Europe and the United States. (9) The mean age at presentation is 11 years. (19) Most of these tumors (56%) arise in the abdomen. (19) Involvement of extranodal sites in the head and neck occurs in fewer than 25% of cases. (20) Jaw involvement occurs in only 7% of cases. (19) Bone marrow and central nervous system (CNS) involvement occurs in 30 to 38% and 13 to 17% of adult cases, respectively. (11)

Only 2 cases of Burkitt's lymphoma of the tongue base have been previously reported in the English-language literature. (1,2) Other sites of involvement in the head and neck include the parapharyngeal space, tonsil, mandible, maxilla, skull, maxillary sinus, nasopharynx, mastoid, orbit, and nasal cavity. (21-28) In a study (22) of 80 children with Burkitt's lymphoma, 31 (38.8%) had involvement of the head and neck region, including 10 (12.5%) who had primary jaw tumors, a finding that is consistent with other reports of sporadic Burkitt's lymphoma.

Immunodeficiency-associated Burkitt's lymphoma generally occurs in patients with human immunodeficiency virus (HIV) infection. In a series of 399 patients with acquired immunodeficiency syndrome who presented with head and neck manifestations, 8% had Burkitt's lymphoma as a rapidly enlarging neck mass. (29) Unlike other HIV-associated lymphomas, however, Burkitt's lymphoma tends to occur in patients whose CD4 counts exceed 200 cells/ [mm.sup.3]. These patients--like those with sporadic Burkitt's lymphoma--typically present with extranodal disease that most commonly involves the abdomen. (30)

The Murphy/St. Jude's staging system (31) is commonly used to stage non-Hodgkin's lymphomas in children (figure 4). In adults, the Ann Arbor staging system, (32) which was originally developed for Hodgkin's lymphomas, is often used. The usefulness of the Ann Arbor system is limited by the fact that Hodgkin's lymphoma and non-Hodgkin's lymphoma have different patterns of spread. (33) For this reason, the Murphy/St. Jude's system is sometimes used in adult studies of Burkitt's lymphoma. (11)

In the past, Burkitt's lymphoma was treated with a prolonged regimen of high-intensity chemotherapy--including induction, consolidation, and maintenance phases--similar to those used for patients with acute lymphoblastic leukemia. But this regimen was ineffective, and it gave way to a regimen of high-intensity chemotherapy over a short duration. The chemotherapeutic agents include cyclophosphamide, doxorubicin, vincristine, methotrexate, and cytarabine. Current therapy does not involve routine debulking surgery because the chemotherapy itself is effective and because early surgery is associated with an increase in complications. (11)

In children with advanced-stage Burkitt's lymphoma, 2-year disease-free survivalranges from 75 to 89%. (11) In adults, who have a higher incidence of CNS and bone marrow involvement, treatment also includes intrathecal chemotherapy. With this treatment, 65 to 100% of adults have achieved a complete response, and 47 to 86% of patients remained in remission after treatment. (11) Many of the chemotherapeutic regimens currently used in adults are based on principles previously developed for the treatment of childhood Burkitt's lymphoma. (11) The addition of rituximab (an anti-CD20 monoclonal antibody) to chemotherapy has greatly improved the outcome of treatment for non-Hodgkin's lymphoma. (34) Similarly, our patient was treated with 3 cycles of R-CHOP, with and without intrathecal methotrexate, and he experienced a complete response to therapy.

Lymphoma is two to four times more common in patients of Middle Eastern descent than others, and extranodal manifestations are more common, as well. (22) Several series have examined the incidence of Burkitt's lymphoma in the Middle East. (22,35,36) In these series, patients' ages ranged from 2 to 50 years, and median ages ranged from 7 to 15 years. In the Middle East, Burkitt's lymphoma has shown characteristics intermediate between the American and African types in both the age at presentation and the site of involvement. (22,35.36) Our patient was an adult of Palestinian descent, and his tumor was negative for EBV, which made his tumor more similar to the endemic variant.

References

(1.) Manolopoulos L, Nikolopoulos TP, Yiotakis J, et al. Burkitt's lymphoma in the base of the tongue: Differential diagnosis and management. ORL J Otorhinolaryngol Relat Spec 2003;65:226-9.

(2.) Yoskovitch A, Hier MP, Begin LR, et al. Dorsal tongue mass. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:5-8.

(3.) Bernstein JI, Coleman CN, Strickler JG, et al. Combined modality therapy for adults with small noncleaved cell lymphoma (Burkitt's and non-Burkitt's types). J Clin Oncol 1986;4:847-58.

(4.) Miller TP, Dahlberg S, Cassady JR, et al. Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate- and high-grade non-Hodgkin's lymphoma. N Engl J Med 1998;339:21-6.

(5.) Lister TA, Armitage JO. Non-Hodgkin's lymphomas. In: Abeloff MD, Armitage JO, Litchter AS, Niederhuber JE, eds. Clinical Oncology. New York: Churchill Livingstone; 2000:2690-1.

(6.) Burkitt D. A sarcoma involving the jaws in African children. Br J Surg 1958;46:218-23.

(7.) Oyetunji NM, Ladapo AA. Burkitt's lymphoma of the mastoid temporal bone. J Laryngol Otol 1981 ;95:1063-7.

(8.) Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: A proposal from the International Lymphoma Study Group. Blood 1994;84:1361-92.

(9.) Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2001.

(10.) Harris NL, Jaffe ES, Diebold J, et al. The World Health Organization Classification of neoplasms of the hematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee meeting--Airlie House, Virginia, November 1997. Hematol J 2000;1:53-66.

(11.) Blum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and lymphoma. Blood 2004; 104:3009-20.

(12.) Hecht JL, Aster JC. Molecular biology of Burkitt's lymphoma. J Clin Oncol 2000; 18:3707-21.

(13.)Knudson A Jr. Genetics of tumors of the head and neck. Arch Otolaryngol Head Neck Surg 1993;119:735-7.

(14.) Epstein MA, Achong BG, Barr YM. Virus particles in cultured lymphoblasts from Burkitt's lymphoma. Lancet 1964; 15:702-3.

(15.) Ziegler JL. Burkitt's lymphoma. N Engl J Med 1981;305:735-45.

(16.) Thorley-Lawson DA, Gross A. Persistence of the Epstein-Ban virus and the origins of associated lymphomas. N Engl J Med 2004; 350:1328-37.

(17.) Kennedy G, Komano J, Sugden B. Epstein-Barr virus provides a survival factor to Burkitt's lymphomas. Proc Natl Acad Sci USA 2003; 100:14269-74.

(18.) Burkitt D. Malignant lymphomata involving the jaws in Africa. J Laryngol Otol 1965;79:929-39.

(19.) Levine PH, Kamaraju LS, Connelly RR, et al. The American Burkitt's Lymphoma Registry: Eight years' experience. Cancer 1982;49:1016-22.

(20.) Wang MB, Strasnick B, Zimmerman MC. Extranodal American Burkitt's lymphoma of the head and neck. Arch Otolaryngol Head Neck Surg 1992;118:193-9.

(21.) Bauer GP, Volk MS, Siddiqui SY, et al. Burkitt's lymphoma of the parapharyngeal space. Arch Otolaryngol Head Neck Surg 1993; 119:117-20.

(22.) Anavi Y, Kaplinsky C, Calderon S, Zaizov R. Head, neck, and maxillofacial childhood Burkitt's lymphoma: A retrospective analysis of 31 patients. J Oral Maxillofac Surg 1990;48:708-13.

(23.) Banthia V, Jen A, Kacker A. Sporadic Burkitt's lymphoma of the head and neck in the pediatric population. Int J Pediatr Otorhinolaryngol 2003;67:59-65.

(24.) Welling DB, McCabe BE American Burkitt's lymphoma of the mastoid. Laryngoscope 1987;97:1038-42.

(25.) Standefer JA Jr., Mattox DE, Aufdemorte TB, et al. Midfacial Burkitt's lymphoma. Otolaryngol Head Neck Surg 1985;93:262-7.

(26.) Vinayak BC, Reddy KT, Fulton J, Milford CA. Pediatric nonendemic Burkitt's lymphoma of the head and neck. Ann Otol Rhinol Laryngol 1994;103:238-40.

(27.) David SS. Burkitt's lymphoma of the nasopharynx. J Laryngol Otol 1972;86:387-93.

(28.) Abaza NA, Iczkovitz ML, Henefer EP. American Burkitt's lymphoma manifested in a solitary submandibular lymph node. Oral Surg Oral Med Oral Pathol 1981;51:121-7.

(29.) Marcusen DC, Sooy CD. Otolaryngologic and head and neck manifestations of acquired immunodeficiency syndrome (AIDS). Laryngoscope 1985;95:401-5.

(30.) Davi F, Delecluse HJ, Guiet P, et al. Burkitt-like lymphomas in-AIDS patients: Characterization within a series of 103 human immunodeficiency virus-associated non-Hodgkin's lymphomas. Burkitt's Lymphoma Study Group. J Clin Oncol 1998;16:3788-95.

(31.) Murphy SB. Childhood non-Hodgkin's lymphoma. N Engl J Med 1978;299:1446-8.

(32.) Carbone PP, Kaplan HS, Musshoff K, et al. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res 1971;31:1860-1.

(33.) Shipp MA. Prognostic factors in aggressive non-Hodgkin's lymphoma: Who has "high risk" disease? Blood 1994;83:1165-73.

(34.) Coiffier B, Lepage E, Briere J, et al. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med 2002;346:235-42.

(35.) Anaissie E, Geha S, Allam C, et al. Burkitt's lymphoma in the Middle East. A study of 34 cases. Cancer 1985;56:2539-43.

(36.) Ardekian L, Rachmiel A, Rosen D, et al. Burkitt's lymphoma of the oral cavity in Israel. J Craniomaxillofac Surg 1999;27:294-7.

From the Department of Otolaryngology--Head and Neck Surgery (Dr. Feinberg and Dr. Shibuya), the Department of Medicine (Dr. Ou), and the Department of Pathology (Dr. Gu), University of California-Irvine College of Medicine, Orange, Calif.

Reprint requests: Steven M. Feinberg, MD, Department of Otolaryngology-Head and Neck Surgery, University of California-Irvine College of Medicine, 101 City Dr., South Bldg. 56, Suite 500, Orange, CA 92868. Phone: (714) 456-5753; fax: (714) 456-5747; e-mail: sfeinber@uci.edu

The information in this article was originally presented as a poster at the Western Section meeting of the Triological Society; Feb. 3-5, 2005; Summerlin, Nev.
Figure 4. Murphy/St. Jude's staging system. (32)

Stage Criteria for extent of disease

I * A single tumor (extranodal) or single anatomic area (nodal)
 with the exclusion of the mediastinum and abdomen

II * A single tumor (extranodal) with regional node involvement
 * Two or more nodal areas on the same side of the diaphragm
 * Two single (extranodal) tumors with or without regional node
 involvement on the same side of the diaphragm
 * A primary gastrointestinal tumor, usually in the ileocecal
 area, with or without involvement of associated mesenteric
 lymph nodes only, grossly completely resected

III * Two single tumors (extranodal) on opposite sides of the
 diaphragm
 * Two or more nodal areas above and below the diaphragm
 * All the primary intrathoracic tumors (mediastinal, pleural,
 thymic)
 * All extensive primary intra-abdominal disease, unresectable
 * All paraspinal or epidural tumors, regardless of other tumor
 site(s)

IV * Any of the above with initial CNS and/or bone marrow
 involvement
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Author:Shibuya, Terry Y.
Publication:Ear, Nose and Throat Journal
Date:Jun 1, 2007
Words:2739
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