A case of Kaposi's haemangiosarcoma.

In 1872 Kaposi described a dermatological condition which he called 'idiopathic multiple pignent sarcoma '. At the present time it is often known as Kaposi's haemangiosarcoma. Kaposi described it as affecting the skin, and it certainly affects this part most often, but for years it has been known to involve other organs. In 1940, Choisser and Ramsey reviewed the literature and mentioned a total of 600 cases since 1872. Stats (1946) lists the sites which, after the skin, may be affected most often. He mentions the glans penis, the submucosa of the gastro-intestinal tract, the respiratory tract and the lymph nodes. Less common sites are the spleen, liver, heart, diaphragm and bladder. Seagrave (1948) has described a case in which the submucosa of the gastro-intestinal tract and the skin were involved. In such cases blood is often found in the stools. The disease is said to be rare in negroes, but Kaminer and Murray (1950) record 38 cases in the South African Bantu occurring in the period 1942-49. It is certainly very uncommon in children and appears to occur mostly in adults over the age of 40. MacKee and Cipollaro (1936) mention an incidence of less than 1 under the age of 10 years. T1he sex incidence is predominantly male. Denzer and Leopold (1936) recorded the clinical aspects of the disease in a male child of 44 years; the necropsy findings were subsequently described by Stats (1946). The case recorded here is that of a boy, and it illustrates the very uncommon form of the disease without skin manifestations. Choisser and Ramsey (1940) described two cases of Kaposi's tumour of the heart without skin lesions; in these cases the right auricle was involved. Cardiac nodules have also been described in cases with cutaneous involvement. Tedeschi, Folsom and Carnicelli (1947) mention three cases affecting viscera without skin manifestations. These authors consider the disease to be systemic in nature affecting various parts at the same time. Aegerter and Peale (1942) consider the condition to be a vascular cancer and regard the visceral lesions as true metastases. However, lymph node involvement is often not regional to any affected organ, and supports the view that the disease is a systemic affection like a reticulo-endotheliosis, although the disease is generally considered to be neoplastic. The case recorded here illustrates this point.