ABSTRACT
Resumen: La fibromatosis mesentérica es un subtipo profundo de tumor desmoide (TD), un tumor benigno de origen fibroblástico localmente agresivo por su tendencia a infiltrar los tejidos adyacentes. Son raros, esporádicos y pueden asociarse con el síndrome de Gardner. El tratamiento de elección es la resección completa, evitando la recurrencia local. Comunicamos el caso clínico de una paciente con fibromatosis intrabdominal mesentérica única, bien circunscripta, que simulaba por la imagenología una masa de origen pelviano.
Summary: Mesenteric fibromatosis is a deep sub-type of desmoid tumors consisting of a benign tumor of fibroblastic origin which is locally aggressive given its tendency to infiltrate adjacent tissues. They are unusual and sporadic, and may be associated to Gardner's Syndrome. Complete resection is the treatment of choice, avoiding local recurrence. The study reports the clinical case of a patient with intra-abdominal sporadic mesenteric fibromatosis, well circumscribed that appeared to be a pelvic mass in MR imaging.
Resumo: A fibromatose mesentérica é um subtipo profundo de tumor desmóide (DT); é um tumor benigno de origem fibroblástica que é localmente agressivo devido à sua tendência a infiltrar tecidos adjacentes. São raros, esporádicos e podem estar associados à síndrome de Gardner. O tratamento de escolha é a ressecção completa, evitando recidiva local. Relatamos o caso clínico de uma paciente com fibromatose mesentérica intra-abdominal única e bem circunscrita que simulava uma massa de origem pélvica na imagem.
Subject(s)
Fibromatosis, Abdominal , Pelvic NeoplasmsABSTRACT
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.