Burkitt Lymphoma and Burkitt-like Lymphoma Clinical Presentation

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

History and Physical Examination

Burkitt lymphoma (BL) is a highly aggressive B-cell lymphoma. All the symptoms are caused by rapid turnover of the mature B lymphocytes and the involvement of extranodal sites and invasion of contiguous organs.

Because of the rapid growth of the Burkitt tumor, patients may quickly manifest significant metabolic derangement and renal function impairment. Less common presentations of Burkitt lymphoma (BL) include an epidural mass, skin nodules, central nervous system (CNS) symptoms, and bone marrow involvement. [5]

Burkitt lymphoma (BL) can also present as acute lymphocytic leukemia (L3-ALL), with fever, anemia, bleeding, and adenopathy.

eBL, sBL, and immunodeficiency-associated BL

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.

Burkitt-like lymphoma

Patients with Burkitt-like lymphoma (BLL) may have a variable presentation compared with those with classic Burkitt lymphoma (BL). Patients with BLL usually present in their fourth or fifth decade of life. More than 50% of affected patients present with widespread (nodal and extranodal) disease with frequent bone marrow and peripheral blood involvement. Some patients have a leukemic presentation.

After reviewing Southwest Oncology Group (SWOG) data, Braziel et al reported the following clinical features of BLL [44] : Median age was reported as 47 years (range, 28-69 y), and 70% of the patients presented with bulky advanced disease (stage II, III, or IV), with 60% of patients having two or more extranodal sites of lymphoma involvement.

Signs

Major signs of Burkitt lymphoma include a soft-tissue mass associated with the involvement of the jaw or other facial bones, rapidly enlarged cervical lymph nodes, abdominal masses, and ascites. The physical examination findings depend on the sites of extranodal involvement and may include the following:

  • Abdominal tenderness

  • Ascites

  • Abdominal mass

  • Hepatosplenomegaly

  • Palpable tumor of the mandibulomaxillary region

  • Ecchymosis and/or petechiae (as a result of thrombocytopenia)

  • Meningeal signs (from CNS disease)

  • Painless lymphadenopathy

Summary of common clinical manifestations

Common findings and symptoms in patients with Burkitt lymphoma are summarized below.

Abdominal masses, which can cause abdominal pain and distention andascites

Mandibular or maxillary mass

  • Most common presentation in eBL

  • Maxillary tumors are more common (may involve the orbit)

  • Jaw involvement occurs much less frequently (15-20% of sBL cases)

CNS involvement

  • Meningeal infiltration, with or without cranial nerve (CN) involvement (frequently, CN III and CN VII); this is the most common mode of presentation with CNS disease

  • Headaches, visual impairment, and paraplegia from spinal involvement; these may be the initial presenting features in some cases

"B" systemic symptoms

  • B systemic symptoms are uncommon but may be associated with other presenting symptoms, such as fever, weight loss, night sweats, fatigue.

Other

  • Bone marrow involvement is common in Burkitt lymphoma

  • Lymphadenopathy is more common in affected adults than in children

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Staging

Patients with more than 25% bone marrow involvement are usually referred to as having Burkitt leukemia (BL). However, most patients will present with nodal or extranodal masses. Different staging systems, such as the National Cancer Institute (NCI) and Ann Arbor and St. Jude/Murphy staging systems, as well as a risk-adapted approach, have been proposed for these patients and are summarized below.

NCI staging system

See the list below:

  • A - Single solitary extra-abdominal site

  • AR - Intra-abdominal, more than 90% of tumor resected

  • B - Multiple extra-abdominal tumors

  • C - Intra-abdominal tumor

  • D - Intra-abdominal plus 1 or more extra-abdominal sites

Ann Arbor system and St. Jude/Murphy staging (commonly used)

Stage I

  • Single tumor (extranodal)

  • Single anatomic area (nodal)

Stage II

  • Single tumor (extranodal) with regional node involvement

  • Primary gastrointestinal tumor

  • Lymphoma involving nodal areas on the same side of the diaphragm

Stage IIR

  • Completely resected intra-abdominal disease

Stage III

  • Lymphoma involving sites on opposite sides of the diaphragm

  • All primary intrathoracic tumors

  • All paraspinal or epidural tumors

  • Extensive intra-abdominal disease

Stage IV

  • Any of the above, with central nervous system (CNS) or bone marrow involvement (< 25%) at presentation

Around 30% of patients present with limited-stage disease (stage I or II), and 70% present with more extensive disease (stage III or IV). Bone marrow involvement is found in 30-38% of patients, and the CNS is involved in 13-17% of adult patients.

Risk-adapted approach

Currently, most patients are treated following a risk-adapted approach. In this classification, patients are broadly divided into two groups: low risk and high risk.

Low-risk patients are those who have nonbulky disease (< 10 cm), early stage (I or II) disease, good performance status, and a normal lactate dehydrogenase (LDH) level. High-risk patients include all other patients.

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