ANAHTAR KELİMELERBurkitt Lenfoma, Dişeti büyümesi

ÖZET

KEYWORDSBurkitt’s Lymphoma, Gingival enlargement

ABSTRACT

Burkit lenfoma (BL) farklı epidemiyolojik, klinik, patolojik, immünolojik ve moleküler sitogenetik özelliklere sahip ekstranodal bir malignensidir. Geçmişte, Burkitt lenfoma ortalama 11 aylık sur-vival 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 sunulmak-tadır.

Burkitt’s lymphoma (BL) is an extranodal maling-nancy with distinct epidemiological, clinical, patho-logical, 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 inten-sive, 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.

Hacettepe Dişhekimliği Fakültesi DergisiCilt: 29, Sayı: 2, Sayfa: 29-33, 2005

African Type Burkitt’s Lymphoma: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.

OLGU RAPORU (A Case Report)

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IntrODUCtIOn

Burkitt’s lymphoma (BL) is an extranodal malingnancy with distinct epidemiological, clini-cal, pathological, immunological, and molecular cytogenetic characteristics.1 Burkitt’s lymphoma is a malignancy of B-lymphocyte origin that rep-resents an undifferentiated lymphoma. During the 1950s, Dennis Burkitt described rapidly growing jaw and abdominal lymphoid tumors in East African children.2 The Tumor, Burkitt’s lymphoma, is the human cancer most closely lin-ked with a virus. Ebstein-Barr virus is associated with 90% of African patients with BL, but this percentage is considerably lower for BL seen in other parts of the world. The reason for the as-sociation between BL and Ebstein-Barr virus re-mains unknown.1-3 The peak age of the African 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 characteri-zed by rapid progression of symptoms with fre-quent multifocal extranodular involvement, inc-luding 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 African form of BL most frequently manifests itself as rapidly gro-wing, extranodal jaw tumors in young children, but it also may be first detected as an abdomi-nal involving the kidneys or ovaries. The growth of the tumor mass may produced facial swelling and propitosis. Pain, tenderness, and paresthe-sia 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 undif-ferantiated, small, noncleaved B-cell lymphoma. The lesional tissue invades as broad sheets of tumor cells and exhibits round nuclei with seve-ral 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 countri-es where the neoplasms usually first detected as an abdominal mass.1,2

BL lesions have a dramatic response to che-motheraphy, particularly cyclophosphamide. The tumor also has been shown to be sensitive to methotrexate, vincristine, and cytarabine. Com-binations of drugs have achieved remissions in more than 90% of patients. Unfortunately, most experience recurrences and ultimately die of the-ir 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 proto-cols 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, tre-ated 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 gin-giva 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-

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tal and histopathological examination was repor-ted as “chronic inflammatory process”.

The patient admitted to our hospital with the same complaints in November 2002. Oral cli-nical 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-lin-gual 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 ede-ma and also swelling of the cheeks extending to the zygomatic arcus on the left. Two lymph no-des 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 re-vealed alveolar bone resorption (Fig 3). A new

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/

mm3, platelets: 315.000/mm3 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 1-2

Intraoral appereance of patient. Note the swellings and severely inflamed gingivae

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

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was detected (Fig 5). A thoracic and abdominal computerized tomography showed bilateral kid-ney lesions. The other bones except the facial ones were normal on plain X-rays. The cyto-logical 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 inc-luded vincristine, cyclophosphamide, predniso-lone, adriamycine, high dose methotrexate with leucovorine rescue. The patient had a comple-te 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 radi-ographic 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 disor-ders more commonly found within the jaws. Be-cause 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 as-sure early diagnosis and a better prognosis of the disease. Clinical differential diagnosis of Burkitt’s lymphoma should include: acute dentoalveolar abscess8, osteomyelitis, rhabdomyosarcoma, pe-riapical lesions, ameloblastoma, eosinophilic gra-nuloma, 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 al9 reported a case with a lo-wer lip paresthesia. BL is one of the most rapidly growing tumors, doubling in size every 24 ho-urs.10 Because of its extremely rapid growth rate, prompt diagnosis before the initiation of specific treatment is imperative for a favorable prognosis.

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

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Alveolar bone resorption and lamina dura loss has been seen in radiological examination. In the literature, radiographic findings of the invol-ved 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 si-milar. 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.

REFERENCES

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15. Hupp JR, Collins FJV, Ross A, Myall RWT. A review of Burkitt’s lymphoma: importance of radiographic diagnosis. J Maxillofacial Surg. 1982; 10: 240-245.

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17. Anavi Y, Kaplinsky C, Calderon S, Zaizov R. Head, neck, and maxillofacial childhood Burkitt’s lymphoma: a retrospective analysis of 31 patients. J Oral Maxillofac Surg. 1990; 48: 708-713.

18. Hanazawa T, Yukinori K, Sakamaki H, Yamaguchi A, Nagumo M, Okano T. Burkitt’s lymphoma involving the mandible. Oral Surg Oral Med Oral Pathol. 1998; 85: (2): 216-220.

CORRESPONDING ADRESS

Murat AKKOCAOĞLU, DDS, PhDHacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery

Tel: 90 312 305 22 76 Fax: 90 310 44 40 E-mail: makkocao@hacettepe.edu.tr