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