Burkitt's lymphoma

doi:10.1016/S0165-5876(86)80060-X

International Journal of Pediatric Otorhinolaryngology

Volume 12, Issue 1, November 1986, Pages 73-84

https://doi.org/10.1016/S0165-5876(86)80060-X Get rights and content

Summary

Cancer is the second leading cause of death in children and, among cancers, lymphoma is the third most frequently diagnosed type. The majority of these are non-Hodgkin's lymphomas (NHL). Although NHL have been grouped in a variety of confusing ways, recent research in cytogenetics and immunology, coupled with a better understanding of the normal pathways of lymphoid differentiation, has greatly lessened this confusion. Burkitt's lymphoma (BL) is a type of B-cell NHL which is sub-classified as either endemic (African) or non-endemic (American). Kinetic studies have demonstrated a doubling time of only 24 h. As a result of this extremely rapid growth, symptomatic complaints occur early in the disease course. Structures contiguous to the primary become compressed and metabolic derangements occur. Signs of chronic debilitation such as cachexia, diarrhea, generalized lymphadenopathy, and weight loss usually are not found. Thirteen cases of American BL have been treated since 1980 at the Texas Children's Hospital and form the basis of this review. Ten patients had obstructive abdominal symptoms including constipation and pain, and 3 had masses in the neck, maxilla, or tonsil. Systemic manifestations included ascites, jaundice, fever, and hepatosplenomegaly. Two patients ultimately developed central facial nerve paralysis.

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Cited by (19)

Burkitt lymphoma presenting as facial nerve paresis
2016, International Journal of Pediatric Otorhinolaryngology Extra
Citation Excerpt :
Sporadic BL usually arises in the head and neck or abdomen, but may involve bone marrow or central nervous system in up to 25% of patients [9]. Waldeyer’s ring and the terminal ileum are common sites of involvement [10], with the classic disease presentation for sporadic disease being an abdominal mass that is often in the ileocecal region [12,13]. Here we report two unusual cases of pediatric BL presenting with facial nerve paralysis.
Acute onset unilateral facial palsy can be seen in children with otitis media. However, when symptoms fail to improve with antibiotics a broader differential should be considered. Here we present two cases of patients who were initially treated for otitis media, but developed facial paresis and were subsequently diagnosed with Burkitt lymphoma involving the temporal bone. In both instances facial nerve function improved with chemotherapy initiation.
Malignant cervical masses in children
2015, Otolaryngologic Clinics of North America
Citation Excerpt :
Almost all patients with endemic BL show high antibody titers to EBV determinant antigens, and 80% to 90% of their tumor cells contain copies of the EBV DNA genome; in contrast, only 15% to 20% of patients with the sporadic form of BL show this EBV association.42 BL almost exclusively affects children, with a predilection for boys.40,41 However, the clinical characteristics of endemic BL differ greatly from those of the sporadic form.
Proof for EBV's sustaining role in Burkitt's lymphomas
2009, Seminars in Cancer Biology
Citation Excerpt :
As the tumor develops, a hostile microenvironment may also stimulate apoptotic cell death. Burkitt's lymphomas grow extremely rapidly [55], possibly making nutrients and oxygen limiting for cell growth and survival. Lastly, once clinical treatment for the tumor has begun, the chemotherapeutic drugs commonly used are themselves potent inducers of apoptosis [37].
We have found that not all Epstein–Barr viral (EBV) plasmids are duplicated each cell cycle. This inefficiency is intrinsic to EBV's mechanism of DNA synthesis in latently infected cells and necessarily leads to a loss of EBV plasmids from proliferating cells. If EBV provides its host cells advantages that allow those cells that retain EBV to outgrow those that lose it, then such proliferating populations will be EBV-positive. EBV-associated human tumors are EBV-positive. Thus, the presence of EBV plasmids in most cells of a tumor demonstrates that EBV sustains these tumors in vivo. The virus can provide multiple selective advantages to tumor cells, including promoting cell proliferation and inhibiting cell death. In the case of Burkitt's lymphomas (BL), most current evidence indicates that the tumor requires the virus minimally to block apoptosis.
Sporadic Burkitt's lymphoma of the head and neck in the pediatric population
2003, International Journal of Pediatric Otorhinolaryngology
Burkitt's lymphoma (BL) is one of the fastest growing malignancies in the pediatric population in the United States. BL is a high-grade B-cell Non-Hodgkin's lymphoma (NHL) which exists in endemic, sporadic, and human immunodeficiency-associated subtypes. The African, or endemic, variant usually involves the maxilla and other facial bones while head and neck manifestations in non-endemic BL are rare. We present three unusual present ations of sporadic BL stemming from Waldeyer's ring and the orbit. The clinical and pathologic features of BL are reviewed.
Dorsal tongue mass
2000, Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics
Citation Excerpt :
AMBL often presents as an abdominal mass, usually involving the distal ileum, cecum, or mesentery. Presentations in the head and neck are uncommon, with involvement of the facial bones, jaw, and other extranodal sites reported in less than 10% of cases.13-16 Specific sites of head and neck involvement by AMBL include the nasopharynx, tonsil, ethmoid sinus, orbit, mandible, maxilla, cheek, hard palate, mastoid, and parapharyngeal space.
Burkitt's lymphoma of the oral cavity in Israel
1999, Journal of Cranio-Maxillofacial Surgery
The clinical presentation of Burkitt's lymphoma in the maxillofacial area is variable. The objective of this study is to review and analyse all cases of Burkitt's lymphoma with oral or maxillofacial involvement diagnosed in our department. A retrospective review of patients with Burkitt's lymphoma in the facial area between the years 1978 and 1997 was undertaken. The patients, 8 male and 5 female, were from 7 to 50 years old (mean 15.3 years). Six patients were Stage I and 7 were Stage II. Five had abdominal involvement and 2 lymph node involvement besides the maxillofacial presentation. Three tumours were in the mandible, 3 in both mandible and maxilla, 2 in the palate, and 5 in the maxilla alone. In 5 patients the tumour presented as a facial swelling, in 3 as an exophytic mass, in 2 as an ulcer, 1 case presented as a hyperplastic lesion, and 2 were periapical lesions. Complaints included pain (7), swelling (5), and sensory disturbance (2). EBV titres were positive in 4 patients. Abdominal involvement was only seen in patients under 12 years old. All patients were treated with chemotherapy, while adjuvant radiotherapy was indicated in 3 cases. Follow-up of 1–20 years revealed a 2-year survival rate of 61.5%. It seems that the Israeli disease is between that of the African and American types, when considering age distribution.

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PaperBurkitt's lymphoma

Summary

Am. J. Med.

Lancet

Blood

Int. J. Pediat. Otorhinolaryngol.

Med. Clin. Am.

Malignant Disease of Infancy, Childhood and Adolescents

American Burkitt's lymphoma

Am. J. Med.

Tumors of the Head and Neck

A sarcoma involving the jaws in African children

Brit. J. Surg.

A tumour safari in east and central Africa

Brit. J. Cancer