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Clinical Characteristics

The disease is generalized and almost always multifocal when the patient is first seen, whatever the presenting symptoms or site. It is preferable to consider it as "multifocal" rather than "metastatic", because the original tumor site can seldom be recognized and there is often extensive bone marrow involvement. A careful search must therefore be made to establish the extent of the disease.

Jaws and Facial Bones

The most frequent, visible and characteristic clinical feature is unilateral or bilateral enlargement of the maxilla and mandible, either together or separately (Figs. 41.2, 41.3). In Ethiopia, jaw tumors are slightly less common than tumors at abdominal sites. The facial and jaw tumors grow rapidly and are usually painless until bone involvement becomes severe (Fig. 41.4). Ulceration is rare, but may occur in the jaws; the teeth are displaced and eventually lost. Tumors in the facial bones may cause proptosis, but the eyes may also be displaced by tumors in the lacrimal glands without bone involvement.

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Fig. 41.2A,B. (A) A West African mask (wood), probably Ibo, Nigeria, with a strong resemblance to a child with Burkitt's lymphoma. It is unusual for African masks to depict illness or deformity. (B) An 11 year-old Nigerian boy with a tumor in the left maxilla. Both the boy and the mask show facial scarification.

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Fig. 41.3A-E. West and East African children with Burkitt's lymphoma. (A) A 3 year-old girl with a tumor in the right mandible. (B) An 8 year-old boy with a tumor in the left mandible. (C) A 13 year-old boy with tumors in the right maxilla and mandible. (D) A 12 year-old boy with a tumor in the right maxilla spreading outwards and also into the mouth, displacing the teeth. (E) A small child in Kenya with a large Burkitt's tumor.

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Fig. 41.4 A-D. Burkitt's lymphoma. (A) Two tumors in the mandible of a child. AFIP 219932-253. (B) Distortion of the teeth by bilateral tumors in the mandible of another child. The larger tumor is very hemorrhagic. AFIP 219932-254. (C) A lateral section through a maxilla with a large lymphoma spreading up and around a retro-orbital area, into the ethmoid and sphenoid sinuses and the palate. (D) The classical microscopic appearance of Burkitt's lymphoma. There are large histiocytes with eccentric nuclei interspersed with lymphocytic tumor cells to give the "starry sky" appearance. Although this is not specific for Burkitt's tumor, the cellular uniformity is seldom found in other lymphomas. AFIP 219932-255.

The distortion of the jaws and face may be bizarre and dramatic; the results of satisfactory treatment are often equally dramatic, and the extent to which the face may return to normal is astonishing. Because the tumor grows rapidly and children are reluctant to come to hospital, the disease may be far advanced when first seen, but this should not discourage an enthusiastic approach to treatment. The results of chemotherapy and immunotherapy are excellent; surgery is seldom indicated. Radiotherapy produces dramatic results but, because of the complexities of the immune system, is probably contraindicated. Radiation facilities are not always readily available in many parts of tropical Africa and New Guinea and this may be an advantage; personal experience suggests that although tumors respond rapidly to a relatively low radiation dosage, recurrence occurs either at the same site or elsewhere, and radiotherapy should probably be used only where there is local bone pain or severe bone destruction, as occurs in the vertebrae.

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