RESUMEN
Purpose To study the clinicopathological features of congenital solitary intestinal fibromatosis (SIF).Methods Clinical,radiological and pathological features of 2 cases of SIF were analyzed along with a literature review.Results Two cases of SIF with one girl and one boy.Both presented with vomiting and abdominal distention at the birth day.A plain abdominal radiograph revealed small bowl obstruction and pneumoperitoneum.Jejunum atresia and perforation were detected at surgery.Microscopically,spindle tumor cells diffusely infiltrated in the whole intestinal wall,tumor cells were monomorphic with bland looking and rare mitosis was reviewed.Immunohistochemically,vimentin was positive while muscular markers as SMA,desmin and Myogenin were all negative.And S-100,CD117,CD34,ALK and β-catenin were not expressed.ETV6 split gene was not detected in one of the patients by FISH.Two patients of SIF had no tumor recurrence with follow-up till now.Conclusion Congenital SIF is a rare and unique intestinal tumor in neonate,and it should be differentiated from congenital fibrosarcoma and myofibroma.SIF appears a very good prognosis after segmental resection.