Immunohistochemical and ultrastructural studies of a primary aortic intimal sarcoma.

An intimal sarcoma of the abdominal aorta in a 63-year-old woman is reported. The clinical symptoms consisted of chronic arterial hypertension, vomiting and epigastric pain. Treatment was operative, but the patient died 20 hours after surgery. The studies were performed on a surgical specimen and on autopsy material. The aortic tumour consisted of pleomorphic spindle-shaped and giant cells. In the vertebral metastases a storiform pattern of the tumour cells was found. No specific features characteristic for leiomyogenic, lipogenic or an endothelial nature of the tumour giant cells was disclosed in electron microscopy and the picture rather indicated their histiocytic character. Of the 18 cellular markers studied, the immunostainings for vimentin and alpha-1-antichymotrypsin were evidently positive. The tumour was classified as a pleomorphic intimal aortic sarcoma probably a malignant fibrous histiocytoma (MFH). The literature on 26 previously published aortal tumours is reviewed with emphasis on their topographical distribution and histological classification. In only 4 previous cases was the final diagnosis supported by electron microscopical or immunopathological findings. The role of marker studies in the classification of aortal tumours is discussed.

[Low incidence of non-Hodgkin lymphoma but high seroprevalence of HTLV-I in Curaçao]. / Lage incidentie van non-Hodgkin-lymfoom, maar hoge seroprevalentie van HTLV-I op Curaçao.

OBJECTIVE: To determine whether the low incidence of non-Hodgkin's lymphoma (NHL) in Curaçao has changed in comparison with the increase in incidence in many western countries, and to investigate the role of the HTLV-I infection that is endemic in the Caribbean area. DESIGN: Retrospective. SETTING: Curaçao, Netherlands Antilles. METHOD: Retrospective file analysis in the only hospital in Curaçao. RESULTS: During the period 1987-1992, 31 patients had a histologically confirmed diagnosis of NHL resulting in an annual incidence rate of 4.9/100,000 adults. There was a strong age-related increase in NHL incidence rate (0.5 for patients < 30 years to 17.8 for patients > or = 70 years), with a male to female ratio of I. (In the western world the incidence is 12-14, in the seventies it was 4.5 in Curaçao.) Seven of 12 patients (58%) tested were seropositive for HTLV-I. Median survival was 6 months, despite conventional therapy. CONCLUSION: While HTLV-I infection can often be demonstrated in NHL patients in Curaçao, NHL incidence has remained low over the past 25 years.