African Type Burkitt's Lymphoma - dishekdergi.hacettepe.edu.tr

OLGU RAPORU (A Case Report)
ABSTRACT
Burkitt’s lymphoma (BL) is an extranodal malingnancy
with distinct epidemiological, clinical, pathological,
immunological, and molecular cytogenetic
characteristics. The prognosis for Burkitt’s lymphoma
in the past was poor, with a median survival time of
only 11 months. More recent trials with more intensive,
multiagent chemotherapeutic protocols show a
68% remission rate after 38 months follow-up.
In this case report we present of a 10-year-old male
patient with Burkitt’s lenfoma diagnosed clinically
and histopathologically, treated by chemotherapy and
followed up eighteen months post chemotherapy.
KEYWORDS
Burkitt’s <strong>Lymphoma</strong>, Gingival enlargement
Hacettepe Dişhekimliği Fakültesi Dergisi
Cilt: 29, Sayı: 2, Sayfa: 29-33, 2005
<strong>African</strong> <strong>Type</strong> Burkitt’s <strong>Lymphoma</strong>:
A Case Report
Afrika Tipi Burkit Lenfoma: Bir Olgu Sunumu
*Murat Akkocaoğlu, DDS, PhD, **Nalan Yazıcı, MD,PhD, *Oğuzcan Kasaboğlu, DDS, PhD,
***Bilgehan Yalçın, MD, PhD, ****Nuray Er, DDS, PhD,
*Hacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery.
**Hacettepe University, İhsan Doğramacı Children Hospital, Department of Pediatric Oncology
*Hacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery.
***Hacettepe University, İhsan Doğramacı Children Hospital, Deparment of Pediatric Oncology
****Hacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery.
ÖZET
Burkit lenfoma (BL) farklı epidemiyolojik, klinik,
patolojik, immünolojik ve moleküler sitogenetik
özelliklere sahip ekstranodal bir malignensidir.
Geçmişte, Burkitt lenfoma ortalama 11 aylık survival
zamanı ile kötü bir prognoza sahipken, yakın
zamanlardaki girişimsel, multiajan kemoterapötik
uygulamalar ile gerçekleştirilen çalışmalar sonucu
38 ay izlemde %68 iyileşme oranına ulaşılmıştır.
Bu olgu sunumunda, klinik ve histopatolojik olarak
teşhis konulmuş ve kemoterapi ile tedavi edilmiş 10
yaşındaki erkek hastanın 18 aylık takibi sunulmaktadır.
ANAHTAR KELİMELER
Burkitt Lenfoma, Dişeti büyümesi

30
IntrODUCtIOn
Burkitt’s lymphoma (BL) is an extranodal
malingnancy with distinct epidemiological, clinical,
pathological, immunological, and molecular
cytogenetic characteristics. 1 Burkitt’s lymphoma
is a malignancy of B-lymphocyte origin that represents
an undifferentiated lymphoma. During
the 1950s, Dennis Burkitt described rapidly
growing jaw and abdominal lymphoid tumors
in East <strong>African</strong> children. 2 The Tumor, Burkitt’s
lymphoma, is the human cancer most closely linked
with a virus. Ebstein-Barr virus is associated
with 90% of <strong>African</strong> patients with BL, but this
percentage is considerably lower for BL seen in
other parts of the world. The reason for the association
between BL and Ebstein-Barr virus remains
unknown. 1-3 The peak age of the <strong>African</strong>
BL is between 5 and 7 years. The male to female
ratio ranges between 2:1 and 6,5:1 and is much
higher in patients under 13 with an incidence of
0,1 to 0,3/100,000. 4
The clinical presentation of BL is characterized
by rapid progression of symptoms with frequent
multifocal extranodular involvement, including
central nervous system involvement. With
in the oral cavity, this tumour can progress very
fast and appears as a facial swelling or exophytic
mass involving the jaws. 5 Involvement of facial
bones and oral cavity occurs in less than 30% of
cases in most series. 6-7 The <strong>African</strong> form of BL
most frequently manifests itself as rapidly growing,
extranodal jaw tumors in young children,
but it also may be first detected as an abdominal
involving the kidneys or ovaries. The growth
of the tumor mass may produced facial swelling
and propitosis. Pain, tenderness, and paresthesia
are usually minimal, although marked tooth
mobility may be present because of aggressive
destruction of the alveoler bone. 2
The radiographic features are consistent with
a malignant process and include a radiolucent
destruction of the bone with ragged, ill-defined
margins. This process may begin as several
smaller sites, which eventually enlarge and coa-
lesence. Patchy loss of the lamina dura has been
mentioned as an early sign of BL.
BL histopathologically represents an undifferantiated,
small, noncleaved B-cell lymphoma.
The lesional tissue invades as broad sheets of
tumor cells and exhibits round nuclei with several
prominent nucleoli and numerous mitoses.
A classic starry-sky pattern is associated with
lesional tissue, aphenomenon that is caused by
the presence of macrophages within the tumor
tissue. Tumors with a similar histomorphology,
commonly referred to as American Burkitt’s
lymphoma, have been observed in other countries
where the neoplasms usually first detected as
an abdominal mass. 1,2
BL lesions have a dramatic response to chemotheraphy,
particularly cyclophosphamide.
The tumor also has been shown to be sensitive to
methotrexate, vincristine, and cytarabine. Combinations
of drugs have achieved remissions in
more than 90% of patients. Unfortunately, most
experience recurrences and ultimately die of their
disease. The prognosis for Burkitt’s lymphoma
in the past was poor, with a median survival time
of only 11 months. More recent trials with more
intensive, multiagent chemotherapeutic protocols
show a 68% remission rate after 38 months
follow-up.
In this case report we present of a 10-year-old
male patient with Burkitt’s lenfoma from Turkey
diagnosed by gingival overgrowth, alveoler bone
loss, tooth mobility and histopathologically, treated
by chemotherapy and followed up eighteen
months post chemotherapy.
Case rePOrt
A ten year old boy presented with a six week
history of painful and progressive swelling of the
jaw bilaterally. It was considered as dental abcess
at the local hospital and antibiotic therapy was
considered for two weeks.The lesions of the gingiva
and the swelling of the jaw did not resolve.
He had also headache, fever and weight loss. A
gingival biopsy was performed at the local hospi-

tal and histopathological examination was reported
as “chronic inflammatory process”.
The patient admitted to our hospital with the
same complaints in November 2002. Oral clinical
examination revealed gingival overgrowth
in both maxilla and mandible and marked tooth
mobility. The marginal and attached gingivae in
almost all teeth, on both buccal-labial palatal-lingual
aspect, were severely inflamed, bright red in
appearence, and hyperplastic (Figs 1 and 2).
Initial physical examination showed a marked
swelling of the mandibula bilaterally, facial edema
and also swelling of the cheeks extending to
the zygomatic arcus on the left. Two lymph nodes
are palpable, each smaller than 0.5 cm in
the right anterior cervical region. The rest of the
physical examination was unremarkable.
The orthopantomographic examination revealed
alveolar bone resorption (Fig 3). A new
FIGURE 1-2
Intraoral appereance of patient. Note the swellings and
severely inflamed gingivae
31
biopsy was performed from the right lower gin-
giva. Both the new biopsy and the previous one
were reported as “ Burkitt’s lymphoma” (Fig 4).
The initial laboratory examinations of the pa-
tient were as follows: Hb:9.1 g/dl, WBC: 7800/
mm 3 , platelets: 315.000/mm 3 with a normal dif-
ferential; serum biochemistry was normal except
a LDH of 1737 IU. Bone marrow examination
revealed diffuse involvement by lymphoblasts;
computerized tomography of the head showed
lytic lesions on the right frontoparietal region
with an epidural soft tissue component and skin
involvement; also a left sphenoidal lytic lesion
with a soft tissue mass extending to the anterior
of the temporal lobe and the orbit extraaxially
FIGURE 3
Panoramic radiograph demostrating bone defects and lytic
areas on both jaws
FIGURE 4
Low magnification shows “starry sky” appearance due to
interspersed macrophages with abundant cytoplasm and
hyperchromatic neoplastic lymphoid cells

32
FIGURE 5
CT showing a left sphenoidal lytic lesion with a soft tissue mass
extending to the anterior of the temporal lobe and the orbit
extraaxially
FIGURE 6
Intraoral view after 1 year demostrating gingival healing and
tooth eruption
FIGURE 7
Postchemotherapy panoramic radiograph showing the healing
of bone lesions
was detected (Fig 5). A thoracic and abdominal
computerized tomography showed bilateral kidney
lesions. The other bones except the facial
ones were normal on plain X-rays. The cytological
examination of cerebrospinal fluid was
negative.
With the diagnosis of stage IV Burkitt’s type
non-Hodgkin’s lymphoma; we treated the patient
with LMB-B chemotherapy regimen which included
vincristine, cyclophosphamide, prednisolone,
adriamycine, high dose methotrexate with
leucovorine rescue. The patient had a complete
response to chemotherapy. In April 2003,
chemotherapy was ended when the patient was
in complete remission. He is still under regular
follow-up with no evidence of disease eighteen
months after cessation of treatment. Fig 6 and 7
shows the post chemotherapy clinical and radiographic
picture of normal tooth development.
DIsCUssIOn
Diagnosis of Burkitt’s lymphoma, especially
when the sole presentation is in the maxillofacial
region, is very difficult. The clinical presentation
of the disease may mimic a wide variety of disorders
more commonly found within the jaws. Because
primary presentation of the disease is often
in the mouth and jaws, a high index of suspicion
is required from the medical staff in order to assure
early diagnosis and a better prognosis of the
disease. Clinical differential diagnosis of Burkitt’s
lymphoma should include: acute dentoalveolar
abscess 8 , osteomyelitis, rhabdomyosarcoma, periapical
lesions, ameloblastoma, eosinophilic granuloma,
multiple myeloma, leukemia, and other
fibro-osseous lesions. The signs and symptoms
of oral Burkitt’s lymphoma include mobile teeth,
toothache, oral masses, gingival enlargement,
pain, and jaw expansion. 5 Additional to all these
findings Ugboko et al 9 reported a case with a lower
lip paresthesia. BL is one of the most rapidly
growing tumors, doubling in size every 24 hours.
10 Because of its extremely rapid growth rate,
prompt diagnosis before the initiation of specific
treatment is imperative for a favorable prognosis.

Alveolar bone resorption and lamina dura loss
has been seen in radiological examination. In
the literature, radiographic findings of the involved
jaw have been well described by Burkitt, 11
Adatia, 12-14 Hupp et al., 15 Wood et al., 16 Anavi et
al., 17 and Hanazawa et al. 18 Our findings were similar.
According to our experience, dentists and
maxillofacial surgeons should evaluate oral and
radiologic findings as facial swelling and gingival
enlargement, alveoler bone loss with a suspect of
BL considering the rapid growth rate and poor
diagnosis.
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33
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Hacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery
Tel: 90 312 305 22 76 Fax: 90 310 44 40 E-mail: makkocao@<strong>hacettepe</strong>.<strong>edu</strong>.tr