Primary tumors of the great vessels (aorta, pulmonal artery, and inferior v
ena cava) are rare and represent in most cases vascular leiomyosarcomas. Fu
rthermore, there also exists a group of sarcomas arising from the intima, k
nown as intimal sarcomas, associated with early metastasis and a very poor
prognosis. Osteopontin (OPN) is an extracellular matrix protein that binds
to av integrins, thereby promoting cell attachment, chemotaxis, and signal
transduction. The reported association of OPN with malignancy and metastasi
s prompted us to examine the expression of this protein in seven sarcomas o
f the pulmonary artery. Strong OPN-specific staining could be detected in t
umor cells and the adjacent extracellular matrix. Using a double labeling p
rocedure, proliferating cells showed a strong positive reaction with antibo
dies against OPN. In addition, this protein could be demonstrated in the cy
toplasm of macrophages. CD44, a putative receptor of OPN, was expressed on
the cellular surface of tumor-associated lymphocytes. The expression of OPN
in macrophages and tumor cells indicates that this molecule could possibly
mediate cellular adhesion of both cell types in pulmonary sarcomas. The de
tection in the extracellular matrix shows that OPN is actively secreted and
may interact with the corresponding receptor, CD44, on the surface of lymp
hocytes. Although the function of OPN is not yet fully understood, our data
indicate that strong expression of this molecule in poorly differentiated
sarcomas could play a role in the progression of malignancy and metastasis
as described previously for carcinomas.