W. Rhomberg et al., PROGNOSIS AND EPIDEMIOLOGY OF MALIGNANT H EMANGIOENDOTHELIOMA OF THE THYROID, Schweizerische medizinische Wochenschrift, 123(35), 1993, pp. 1640-1644
Five new cases of malignant hemangioendothelioma of the thyroid are re
ported. This rare malignant tumor is described predominantly in region
s with endemic goiter such as the Alpine zone. The estimated incidence
of the disease is between 0.15 und 0.25 per 100,000 inhabitants per y
ear for Western Austria. Two of the five patients were heavily exposed
to vinyl chloride occupationally for 9 and 30 years respectively. Sur
gically, a R-0 resection was performed in two patients, R-1 in two, an
d R-2 in one patient. Postoperative radiotherapy was given in four cas
es. The tumor doses were between 58 and 65 Gy. Three patients received
the sensitizer razoxane in addition. Local tumor control was achieved
in 4 of 5 patients. The survival times of the 5 patients were 78, 68, 38+, 7.5 and 3 months. Two patients are still alive after 68 and 38
months. This small series may indicate that the outcome of the disease
may not be uniformly deleterious and the resistance to radiotherapy r
eported in the literature [3, 10] is debatable.