Congenital-infantile fibrosarcoma: A clinicopathological study of five patients entered on the Prague Children s Tumor Registry
Abstract
Five children with congenital-infantile fibrosarcoma are analyzed. The tumor was found at birth in four children: in one patient it was recognized at the age of 7 months.
In three children the tumor affected the lower extremity. In one patient the inguinal region was the primary site, in another the abdominal wall.
The morphology was that of a highly cellular spindle cell sarcoma with cells arranged in a fascicular pattern. Variations of this common pattern such as a cartwheel arrangement, and foci of small oval cells were observed.
The immunohistochemistry revealed positivity of vimentin in four investigated tumors and muscle specific actin in three. Desmin, sarcomeric actin and myoglobin were all negative.
There were scattered cells positive with KP1 (CD68), MAC 387, and in one case, with factor XIIIa antibodies which were considered to be reactive rather than tumor cells. The flow cytometry study showed DNA content in three tumors within diploid range; one tumor was hyperdiploid with the DNA index 1.2.
Three children are disease-free from nine to 21 years after the diagnosis. One of them had the tumor preoperatively irradiated, and the subsequent histological examination revealed an almost complete tumor necrosis.
In one patient there were six recurrences (treated by surgery only), and the child is well 25 months after the last recurrence. In one child the disease had an unusually aggressive course, and the patient died of widespread metastases to the lungs, lymph nodes and bones.