PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA IN 21 PATIENTS, INCLUDING 3 WITH PARTIAL SPONTANEOUS REGRESSION

This investigation studied the general conditions and prognostic facto rs of pulmonary epithelioid haemangioendothelioma (PEH), which is a ra re disease, Twenty-one patients were collected throughout Asia by a qu estionnaire, Age at the detection or onset of symptoms of PEH was 14-6 4 yrs (mean 44 yrs), Males were more likely to be detected by symptoms (4/8, 50%) than were females (1/13, 8%), Fifteen showed bilateral mul tiple nodular opacities. Partial spontaneous regression occurred in th ree asymptomatic patients tone male and two females, all with bilatera l multiple nodular opacities) 5, 13 and 15 yrs after detection, Two of the three patients with pleural effusion died within 1 yr, while the 16 patients with no effusion were alive more than 1 yr later (p<0,05), Histologically, two patients with fibrinofibrons pleuritis and extrap leural proliferation of tumour cells died within 2 yrs, while only one of 14 patients lacking such manifestations died within the same perio d (p<0,05), All three patients without spindle tumour cells survived f or 12 yrs after the diagnosis, while all four patients with such cells died during the same period (p<0,05), In conclusion, 21 patients with pulmonary epithelioid haemangioendothelioma were reported, of whom th ree demonstrated partial spontaneous regression, and adverse prognosti c features were identified.