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Thyroid and Salivary Glands: Lymph Nodes

There may be tumors in the thyroid or any of the salivary glands and these are often multifocal. Extranodal tumors occur, but generalized peripheral lymphadenopathy is uncommon, although cervical, axillary and supraclavicular nodes occasionally become enlarged. In North American and European patients, the tonsillar and nasopharyngeal ring is often involved and peripheral lymphadenopathy is not uncommon (20%), but these are not the usual presentations in Africa or other parts of the tropics.

Abdomen

Burkitt's tumor may present as a swelling of the abdomen. The retroperitoneal tissues, kidneys, liver, spleen, gonads and endocrine organs are frequently involved; even the pancreas does not escape. Bilateral ovarian tumors at any age are particularly characteristic, especially in nontropical countries. Clinical examination of the abdomen may be misleading in that it is often difficult to identify which organ or structure is involved. A hard irregular mass may be palpated, but cannot always be localized successfully. In nontropical countries and in older patients, the abdomen is the most common site of presentation (Figs. 41.8 thru 41.12).

Chest

Tumors in the mediastinum can cause pressure symptoms, and nodular deposits in the epicardium and myocardium may cause cardiac arrhythmias or other symptoms. At least one adult has presented with right heart failure and rapid deterioration from intracardiac lymphomas in both atria and the right ventricle. Patients may present with pleural effusions, but clinical features of lung involvement are rare.

Massive bilateral breast tumors have been reported in women of child-bearing age.

Skull and Central Nervous System

In the CNS, tumors are frequent and may present clinically as cranial nerve palsy or paraplegia. Paralysis is usually sudden, may follow minor trauma, and may be preceded by backache or nerve root pain. The paralysis is usually flaccid. Secondary changes are inconstant. Lumbar puncture will reveal a raised protein, but the CSF is otherwise normal. Cord compression is usually due to extradural deposits, which may be multiple and at different levels.

There may be tumors within the skull: most are dural or extradural. Those arising in the bones of the cranial vault follow the same pattern as in any other bone and present clinically as a soft tissue mass rather than with pain. Avascular deposits within the brain have been recorded, but are uncommon. The symptoms are those of a space-occupying lesion. In children, papilledema is rare because suture diastasis is possible.

Skeleton

Any bone may be involved: clinically there is usually a painless swelling or swellings. Individual long bones may suffer pathological fracture. Pain is seldom the first symptom, unless there is a fracture or massive bone involvement. Tumors of vertebrae may cause paraplegia, but extradural deposits are the more frequent cause of paralysis (see above)(Figs. 41.14 thru 41.16). (Facial and skull tumors have already been discussed).

Nontropical Burkitt's tumor

There is no intrinsic difference between "tropical" and "nontropical" Burkitt's lymphoma. Patients in nontropical regions are often older, and jaw tumors are less common. They may present with abdominal masses or intrathoracic tumors, pleural effusions, peripheral lymphadenopathy or with paraplegia. Regardless of the presenting site, paraplegia and CNS involvement are almost inevitable if the disease progresses. Diffuse involvement of bone marrow is common in children, but rare in adults and, when it does occur, has a poor prognosis.

In nontropical and therefore in nonendemic (sporadic) areas, survival has been linked to the site of the tumors when the patient is first seen. When a single tumor is found, long-term survival is more likely. If there is extensive dissemination, particularly with abdominal and pelvic tumors and marrow involvement, or when there are tumors within the central nervous system, survival is shorter. All those who die from the disease, of either the tropical or the nontropical variety, are likely to have widespread multisystem involvement.

In summary, these tumors can occur anywhere. Multiple soft, yellowish-white tumors, sometimes with areas of necrosis and small hemorrhages scattered throughout many organs, should suggest Burkitt's tumor. The nodules may appear clinically to be separated from the surrounding tissue, but this is a macroscopic appearance not confirmed microscopically.

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Copyright: Palmer and Reeder