Infantile myofibromatosis presenting as a bilobed abdominal and anterior mediastinal mass: Resection via clam-shell incision

Abstract

Infantile Myofibromatosis (IM) is a rare benign proliferative fibrous tumor of infancy arising from abnormal development of myofibroblastic and fibroblastic cell [1,2]. In this case report we present a 3-year-old boy who visited our emergency department with a history of abdominal mass started at birth as a small lump in the epigastric area massively progressed in size over the last 3 months associated with postprandial non-bilious vomiting, loss of appetite, loss of weight and decrease activity. Physical examination revealed dehydrated, cachectic child in a mild respiratory distress with decreased air entry to the right side. A well-circumscribed huge mass extending from the xiphisternum to the umbilicus was palpable in the abdomen. Laboratory workup including tumor markers were within normal range. Computed tomography scan (CT) showed evidence of a huge lobulated heterogeneous soft tissue mass straddling between the anterior mediastinum and abdominal cavity with no evidence of calcification. He was hospitalized for further assessment and complete management. In conclusion unusual presentation and diagnosis in some of pediatric tumors need an extra ordinary diagnostic approaches and surgical technique with multidisplanary team management.