Limb body wall complex is a spectrum of multiple severe anomalies. The etiopathogenesis and clinical classification are still under discussion. In our article, while reviewing previous etiopathogenetical hypothesis, we propose a new clinical classification regarding embryological theories and pheneotypical features. According to the Van Allen diagnostic criteria, the findings of 6 affected fetuses are presented. Prenatal diagnosis was performed in 5 of 6 cases. Craniofacial malformations were present in only 1 case. Thoracic defect and abdominoschisis (either infraumbilical or supraumbilical) associated with visceral eventration, placental-umbilical cord anomalies, and limb defects were detected in the other 5 cases. Aberrant development of each of the 4 embryonic folds (cephalic, 2 lateral abdominal, and caudal) associated with faulty umbilical ring development and placental formation were considered responsible for development of various malformations. In previous clinical classifications, existence or absence of the craniofacial malformation was utilized as an unique discriminating criterion while multiple anomalies exist. In this report, we propose a new clinical classification concerning almost all anomalies caused by defective placental attachment and maldevelopment of the 4 folds.

We report a case of a rare, unusual benign tumor, the cotyledonoid leiomyoma or Sternberg tumor, detected incidentally in a 67-year-old woman who underwent total hysterectomy and bilateral salpingo-oophorectomy because of a persisting left ovarian cyst of 5 cm. This is the 14th case of cotyledonoid leiomyoma and 2nd case which had no intrauterine portion but had extrauterine extensions. At laparotomy, it was detected that a deep red nodular papillary tumor had been dissecting the posterior leaf of the left broad ligament, projecting into the abdominal cavity just near the left cornu of the uterus, and extending in downward direction. The remaining portion was lying between the layers of the left broad ligament and arose from the subserosa of the left side of the uterine isthmus. Although the bizarre and unusual shape was in favor of malignity, the frozen-section examination revealed a benign histology. Although the cotyledonoid leiomyoma is a benign entity, it may suggest a malignant disease owing to its unusual sarcomatoid appearance and its rarity. Therefore, awareness of obstetricians and gynecologists regarding this rare entity will prevent unnecessary anxiety and interventions.