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Journal of Clinical Oncology, Vol 24, No 27 (September 20), 2006: pp. 4521-4522 © 2006 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.06.2315
Primary Burkitt's Lymphoma of Frontal Bone: A Rare PresentationDepartments of Radiation Oncology, Pathology, and Pediatric Oncology, Tata Memorial Hospital, Mumbai, India A 13-year-old boy presented with a forehead swelling of 2 months duration, protrusion of the right eye for 1 month, and bilateral neck swelling for 15 days. On thorough medical evaluation, he was observed to be in good general condition with a Karnofsky performance score of 90. Local examination revealed a 9 x 9 cm bony swelling involving the forehead; the swelling was not tender and was associated with proptosis of the right eye (Fig 1A). He also had bilateral rubbery, mobile, nontender neck nodes. The rest of the systemic examination was unremarkable. Computed tomography scan of the skull revealed an enhancing soft tissue swelling arising from the right squamotemporal bone, with intracranial and extradural extension associated with erosion and spiculation of the bone (Fig 1B). Incisional biopsy of the forehead swelling showed the typical "Starry Sky" appearance suggestive of high-grade B-cell Burkitts type non-Hodgkins lymphoma (NHL; Fig 2). Immunohistochemistry revealed CD10, CD20 positivity and MiB-1 was expressed by all cells (Fig 3). The diagnosis of Burkitts lymphoma (BL) was confirmed. Standard staging investigations for lymphoma were unremarkable. Final stage of the disease was St Judes stage II. He was treated with the standard protocol of multiagent chemotherapy, resulting in complete radiologic regression of the frontal mass at the end of induction chemotherapy.
Lymphomas are the third most common malignancy in children and constitute approximately 10% of childhood malignancies.1 NHL accounts for almost two thirds of childhood lymphomas.2 BL accounts for 40% to 50% of all NHL.3 Dennis Burkitt first described the BL in 1958. BL presents in two forms, the most common being the African endemic type, and the less common being the sporadic type. Incidence of the endemic type of BL is 100 instances per million children. The endemic type is almost always associated with Epstein Bar virus and involves the jaw and other facial bones. The incidence of the sporadic type of BL is 1 to 2 instances per million children.4 The most frequently involved site in the sporadic form is the abdomen, followed by the head and neck region. The less common presenting sites include an epidural mass, skin nodule, and bone. We present this rare case of sporadic BL involving frontal bone. The literature on BL revealed a number of unusual sites of presentation. However, there are only three reported cases of BL involving skull bones; one described a case of a 12-year-old boy who had a palpable painless occipital skull mass of BL type,5 another reported a patient with recurrent BL involving the left frontal bone with intracranial and extradural extension,6 but our patients case is the first of its kind of primary BL involving frontal bone as the primary site. The third case is of a 6-year-old boy with BL of mastoid bone.7 Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES
1. Sandlund JT, Downing JR, Crist WM: Non Hodgkins lymphoma in childhood. N Engl J Med 334:1238-1248, 1996 2. Ziegler JL: Burkitt's lymphoma. N Engl J Med 305:735-745, 1981[Medline] 3. Burkitt D: A sarcoma involving the jaws in African children. Br J Surg 46:218-223, 1958[Medline] 4. DeVita VT Jr, Hellman S, Rosenberg S: Cancer: Principles and Practice of Oncology (ed 7). Philadelphia, PA, Lippincott, Williams, and Wilkins, pp 1951 5. Aslan Y, Okten A, Demirci A: Primary Burkitt's lymphoma of the cranial vault in a child. Pediatr Radiol 25:S232-S233, 1995 (suppl 1)[Medline] 6. Gawish HH: Primary Burkitt's lymphoma of the frontal bone: Case report. J Neurosurg 45:712-715, 1976[Medline] 7. Welling DB, McCabe BF: American Burkitt's lymphoma of the mastoid. Laryngoscope 97:1038-1042, 1987[Medline]
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Copyright © 2006 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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