CASE REPORT Sporadic Burkitt’s lymphoma presenting with multiple cranial neuropathies Jamie D. Sisk, MD, and Richard O. Wein, MD, Jackson, Mississippi B urkitt’s lymphoma most commonly presents as a mandible-based malignancy that affects African children; however, this not the only type of presentation of this tumor. Although uncommon, an extranodal variant of Burkitt’s lymphoma can present with head and neck manifestations similar to more common upper aerodiges- tive tract malignancies. We present a case of Burkitt’s lymphoma manifesting multiple cranial neuropathies at the time of diagnosis. A 54-year-old African-American male presented with a history of progressive fatigue, dysphagia, dysarthria, and the recent onset of rapidly enlarging left-sided neck mass. In addition, he had also noted the development of a bulky right axillary mass. Physical examination revealed a left hypoglossal nerve paralysis and decreased oropharyngeal sensation with significant pooling of secretions. No gag reflex could be elicited of the left side. Left shoulder weakness suggestive of potential CN XI involvement was also noted. Inspection of the neck revealed an 8 cm 6 cm firm, nontender mass spanning level II and III on the left side. The right axillary mass was 4 cm 5 cm and firm to palpation. Flexible laryngoscopy revealed an ulcer- ative mass of the left nasopharynx, while the hypophar- ynx and larynx were normal. CT examination of the neck showed mucosal thicken- ing of the posterior nasopharynx extending inferiorly into the parapharyngeal space (Fig 1) as well as matted lymphadenopathy in level I through IV on the left (Fig 2). CT examination of the abdomen and pelvis illustrated marked enlargement of the liver and spleen as well as enlarged celiac lymph nodes. A complete blood count revealed a white blood cell count (WBC) of 111 10 9 /liter. HIV testing was negative. Fine-needle aspiration of the neck mass was per- formed. Histology and immunohistochemical staining were consistent with Burkitt’s lymphoma. The patient was initially treated with CHOP-based chemotherapy regimen (cyclophosphamide, doxorubicin, vincristine, and prednisolone) and experienced normalization of his WBC and a marked improvement in his neck and axillary lymphadenopathy. Despite continued outpatient chemo- therapy, the patient died approximately 3 months after diagnosis from pneumonia associated with neutropenia. DISCUSSION Burkitt’s lymphoma comprises a heterogeneous group of B cell malignancies with a rate of cell division among the highest of any human tumor. It is invariably associated with translocations of chromosome 8 and affects expres- sion of c-myc, a gene centrally involved in regulation of cell-cycle progression and apoptosis. 1 The tumor cells are intermediately sized B cells that diffusely infiltrate both nodal and extranodal tissues and express B cell– specific surface markers including CD19 and CD20. The histologic hallmark of Burkitt’s lymphoma is the “starry sky” appearance resulting from apoptotic cells inter- spersed with pale phagocytic macrophages. Burkitt’s lymphoma occurs in one of three clinical settings: en- From the Department of Otolaryngology and Communicative Services, University of Mississippi Medical Center. Reprint requests: Richard O. Wein, MD, Assistant Professor, Depart- ment of Otolaryngology and Communicative Services, University of Mis- sissippi Medical Center, 2500 North State Street, Jackson, MS 39216- 4505. E-mail address: Rwein@ent.umsmed.edu. Otolaryngology–Head and Neck Surgery (2006) 135, 640-642 0194-5998/$32.00 © 2006 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2005.05.041