A case of Kaposi's sarcoma in a HIV negative child

A 3-year old child was admitted in our hospital because of bilateral swelling of cervical lymphnodes, cough, coryza, fever and sweating. The child was sick looking and pale.

The Chest X-Ray showed a mediastinic enlargement on both sides of the trachea with lobulated soft tissue masses. The radiologist was suggesting the diagnosis of lymphoma. The abdominal U/S was negative and did not find any sign of abdominal lymphoma. The U/S of the neck confirmed bilateral bunches of much enlarged lymphnodes. We have done needle biopsy on the cervical lymphnodes. Meanwhile the child has developed periorbital swelling, facial oedema, and the impression has been of rapid increase of the size of the lymphnodes. The HIV test is negative. The biopsy report indicates Kaposi’s sarcoma (KS). Kaposi’s sarcoma is not always associated with HIV infection. Above all in Africa we have cases of the disease in immunocompetent patients. Available data suggests that abnormal activation of the immune system may play a role in the pathogenesis of Kaposi’s sarcoma.

In the case of the non-HIV related Kaposi’s (the so called endemic African Kaposi’s sarcoma) multiple parasitic infestations, including malaria and bacterial or viral infections, could be the source of continuous activation. Infection by herpes simplex virus has been identified in some studies as the putative agent of KS.

While the sporadic (classic) form of KS is normally seen in elderly, the endemic African form is seen very often in paediatric age. KS is for instance the commonest childhood tumour in some African countries, notably Zimbabwe.

Dr Giuseppe Gaido