How does the clinical presentation of Burkitt lymphoma (BL) differ by variant?

Updated: Dec 20, 2019
  • Author: Ali H Kanbar, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

The 3 different clinical variants of Burkitt lymphoma (BL) described (endemic, sporadic, and immunodeficiency related) may have varied clinical presentations. [2, 6, 38]

The endemic form of Burkitt lymphoma (eBL) is most commonly seen in patients in equatorial Africa, with jaw and facial bone (orbit) involvement occurring in more than 50% of cases. Patients most often present with swelling of the affected jaw or other facial bones, loosening of the teeth, and swelling of the lymph nodes, which are nontender and rapidly growing, in the neck or below the jaw. Other clinical presentations including abdominal masses (ileal, cecal, etc), as well as ovarian, gonadal, skeletal, and breast involvement have also been documented.

The sporadic forms (sBL) most often present with abdominal tumors with bone marrow involvement. Patients most commonly present with abdominal tumors that cause swelling and pain in the affected area. Some patients present with symptoms of bowel obstruction secondary to an ileal-cecal intussusception caused by tumor growth. However, generalized lymphadenopathy is rare. Approximately 90% of patients with sBL and 50% of patients with eBL have abdominal masses upon presentation.

Involvement of the Waldeyer ring (palatine and lingual tonsils, nasopharyngeal adenoid) and nodes is common in sBL, and may result in dysphagia, dyspnea, wheezing, and airway obstruction. [39] Jaw tumors are very rare in sBL.

Like eBL, sBL can involve the ovaries, kidneys, and breasts. Cases of Burkitt lymphoma presenting as acute pancreatitis, cardiac masses, bilateral ovarian masses, and even skin lesions have been reported. [40, 41, 42, 43]

Immunodeficiency-related Burkitt lymphoma usually presents as nodal involvement. Bone marrow involvement occurs frequently. Waldeyer ring and mediastinal nodes are rarely involved.


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