Burkitt lymphoma (BL) is a highly aggressive, B cell non-Hodgkin lymphoma (NHL) that is rare outside Africa. We report a case of HIV-associated BL originating in the right maxillary sinus in a 31-year-old Hispanic male living in Washington, USA. We also highlight the diagnostic challenges in arriving at the diagnosis when the patient initially presented with a painless right facial swelling. Rapid diagnosis and treatment are critical because BL is one of the fastest-growing tumors. Treatment involves brief duration of high intensity chemotherapy and central nervous system prophylaxis. It is important for oral and maxillofacial surgeons to recognize this disease and understand the necessary steps to treat this aggressive tumor.
Burkitt lymphoma (BL) is a highly aggressive, B-cell non-Hodgkin lymphoma that is very rare outside Africa
A patient with midfacial swelling was misdiagnosed with dental abscess but later found to have BL in the maxillary sinus
We need to broaden the differential diagnosis in HIV positive patients presenting with atypical facial swelling
Burkitt lymphoma (BL) is a highly aggressive, B cell non-Hodgkin lymphoma (NHL), characterized by the translocation and deregulation of the MYC gene on chromosome 8q14. Three distinct clinical forms of BL have been described; these include endemic, non-endemic (sporadic), and immunodeficiency-associated [ ]. The endemic form commonly occurs in children residing in equatorial Africa and New Guinea. The jaw or facial bones are most commonly involved, in approximately 50–60% of cases. Non-endemic BL occurs in the United States and Western Europe, accounting for <1% of adult NHL cases [ , ]. Non-endemic BL most commonly involves the abdominal organs, including the ileum, cecum and stomach amongst other sites. Lastly immunodeficiency-associated BL, which is most commonly due to human immunodeficiency virus (HIV), has a heterogeneous clinical presentation. Sites most commonly involved include the bone marrow, central nervous system and lymph nodes.
Anatomically, BL involving the head and neck region, most frequently involves cervical nodes, while extra-nodal site involvement in the head and neck region occur in less than 25% of reported cases [ ]. We present a case of HIV-associated BL originating in the right maxillary sinus, highlighting the diagnostic challenges in arriving at the diagnosis in a patient presenting with painless right facial swelling.
Report of a case
A 31-year-old Hispanic male was referred to the Oral & Maxillofacial (OMS) outpatient clinic at University of Washington (UW) for evaluation of persistent right mid-facial swelling. A telephonic or in-person Spanish interpreter was utilized during all the encounters. Three months prior to his presentation, he developed progressive swelling and altered sensation of the right cheek, which was attributed to pressure effect due to sleep position. His symptoms progressed in the following weeks to involve trismus and painful mastication with solid foods. He first underwent an evaluation by his dentist, who extracted the patient’s right wisdom teeth (ADA #1 and 32) due to concerns of dental infection. Despite this intervention, symptoms of midfacial swelling persisted. At a 2-week dental follow up visit, an incision and drainage of the right posterior maxillary buccal space and extraction of tooth #2 were performed. A course of clindamycin was also prescribed. Despite these interventions, the midfacial swelling appeared minimally improved with persistence of right facial paresthesia.
The patient was then referred to the University of Washington Oral and Maxillofacial Surgery (UW OMS) outpatient clinic. Upon presentation, he reported persistent paresthesia over his right check but minimal pain. He denied fever, night sweats, dyspnea, dysphonia, changes in vision, or malaise. He endorsed unintentional weight loss of an unspecified quantity over the past 2 weeks, the latter largely attributed to the inability to eat solid food due to trismus. His past medical history was significant for HIV, diagnosed 2 years prior to presentation and managed with antiretroviral therapy (abacavir, dolutegravir and lamivudine). HIV RNA was undetectable in the most recent viral load assay, and the CD4 count was 509 cells/μL. He had allergies to penicillin. Socially, he reported smoking tobacco, approximately half pack per day for 14 years, and he drank 1–2 alcoholic drinks weekly.
Physical examination revealed an alert and oriented Hispanic male in no acute distress. His vital signs were normal. There was hypoesthesia with 3 out of 5 directional sensation at the right infraorbital nerve distribution. He had obvious right midfacial induration without overlying erythema. His extraocular movements were intact. The inferior border of the mandible was palpable. There was a palpable and tender cervical lymphadenopathy along the right sternocleidomastoid muscle levels I and II ( Fig. 1 ).
Intraorally, a non-fluctuant induration of about 4 cm in size was palpable from the right posterior maxilla extending to the buccal area of tooth #3. The previous incision and drainage site was well-approximated with chromic gut sutures. There was no purulence on palpation. Tooth #3 had a grade 3 mobility. The tongue, floor of mouth, and remainder of the oral cavity and pharynx were normal. His mouth opening was about 30 mm. Evaluation of the panoramic radiograph showed evidence of recent extraction socket #32; however, extraction sockets #1 and 2, as well as the floor of right maxillary sinus, were not well-visualized due to overlying soft tissue shadowing ( Fig. 2 ).
Given his persistent swelling in the setting of paresthesia and normal vital signs, concerns for a non-odontogenic pathology were considered. An urgent computer tomography (CT) scan maxillofacial and neck with contrast was obtained. The CT scan showed a soft tissue mass involving the right maxillary sinus and masticator space with erosive changes of the pterygoid plate and maxillary sinus extending into the pterygopalatine fossa, the Vidian canal, and into the foramen rotundum. There was extension to the orbital floor with abutment of the inferior rectus ( Figs. 3–5 ). A right level IIb necrotic lymph node, of about 3 cm, was also noted ( Fig. 6 ).