| Summary: | A huge abdominal cystic lesion with ascites was detected in a male neonate at 31 weeks of gestation. Increasing ascites and the appearance of subcutaneous edema were detected, which caused fetal hydrops. The patient was delivered by emergency cesarean section at 33 weeks of gestation. The birth weight was 2,407 g, and the Apgar score was 8/9 points (1-/5-min values). Breathing at birth was stable, but the patient presented with remarkable abdominal distention due to the ascites. Later, the patient presented with tachypnea, and breathing gradually worsened, so an emergency operation was performed. There were no intraoperative findings within the small intestine, but there was a large amount of ascites and a cystic mass arising from the liver. The patient’s breathing and circulation dynamics could only be stabilized by ascites removal, so only a tumor biopsy was performed. The pathological findings led to the diagnosis of an inflammatory myofibroblastic tumor, and steroids were administered early after surgery for the purpose of an anti-inflammatory effect and tumor shrinkage. The abdominal distention was alleviated, and blood examinations showed a reduced inflammatory response. There was no apparent shrinkage of the tumor, however; thus, radical surgical treatment was performed on day 24. The postoperative course was uneventful, so the patient was discharged on day 36. Seven years after the operation there has been no recurrence or distant metastasis.
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