The association of cancer and connective tissue disease is well known,
the most frequent being certainly with dermatomyositis. The associati
on cancer and PSS is more controversial. The incidence of neoplasia in
that group seems to be comparable with the general population but the
proportion of certain types of cancer is different, and the temporal
relationship with the apparition of symptoms of PSS is stunning. The h
ypothesis actually in favor is an imbalance of the immune system, whic
h cause the diminution of the immune surveillance and the apparition o
f cancer and a concomitant dysregulation of the system, causing the fi
brosis of the PSS. We describe a 75 year-old white female who develope
d a colic adenocarcinoma; she also had, concomitantly, a systemic scle
roderma, with sclerodactyly and pulmonary fibrosis. The patient corres
ponded to the criteria of the American Rheumatism Association for prog
ressive systemic scleroderma (PSS). The prognosis of patients with PSS
depends on their systemic involvement but also, we believe, in the mo
re aged group, on the apparition of a neoplasia.